The Study of Pediatric Infectious Diseases
Why is there a need to know pediatric infectious diseases? We live in a germ filled world. Microbes of infinite variety and complexity colonize our body surfaces and orifices.
Infectious diseases will continue to plague human beings as long as they live in the world we know.
Infectious problems cut across every discipline and subdiscipline of medicine.
What is the responsibility of a physician in relation to treating infectious diseases? The task of the physician is to meet these challenges with confidence borne of increased understanding of the principles of microbiology and immunology and of how these principles are essential to rational decision-making in the clinical areas. Causes of 105 million deaths among children
The Diagnostic Process
It is important to make the anatomic syndrome diagnosis before trying to determine the specific etiologic agent
Clinical diagnosis is an intellectual process in analyzing a patients disease
o A judgment that begins when the patient is first seen; observed signs and symptoms and ends when the diagnosis cannot be refined further
Steps in Diagnosis
History and physical examination o The experienced clinician forms a hypothesis or
tentative diagnosis from the given history o The physical examination can be completed in a
brief period relative to the disease suspected
Working or presumptive diagnosis o Basis for laboratory exams and therapy
Approach to Diagnosis by Symptoms and Signs
Fever of unknown origin
Fever and seizures
Fever and lymphadenopathy
Fever and abdominal pain
Fever and diarrhea
Fever and hepatosplenomegaly
Fever and limp
Fever and neutropenia
Fever and petechiae General Approach to the Child with Possible Infection
A. Detailed history and PE is essential a. History:
Present illness, significant medical history, travel and animal contact, medications, unusual food ingestions and risk of infection with HIV (e.g., IVDU, remote history of blood transfusion, unprotected sexual intercourse)
b. Comprehensive physical examination: Special attention to general appearances,
rashes, skin manifestations of endocarditis, hepatosplenomegaly, evidence of joint effusion, lymph node enlargement
B. Use of the laboratory: Pathogens identified through:
a. Direct methods Cultures of bacteria and viruses Microbiologic stains, including:
Gram stain
Ziehl-Neelsen stain (AFB)
Silver stain (fungal elements)
Wright stain (stool WBCs) Fluorescent antibody-antigen staining for
HSV1 & 2, VZV, RSV, Influenza A and B, parainfluenza
Direct observation including wet mount for fungal elements, dark-field microscopy for T. pallidum
PCR b. Indirect methods
Intradermal skin testing for Mycobacterium tuberculosis and Coccidioides immitis
Antibody testing for viruses: EBV, CMV, VZV, HIV; Toxoplasma gondii, Bartonella henselae and Mycoplasma pneumoniae
c. Nonspecific laboratory indications of infection typically include elevation of acute-phase reactants: CRP and ESR
Fever and Fever of Unknown Origin
Fever
Elevation of body temperature to above normal (37.4C orally or 38C rectally)
Challenge o To establish the causative agent distinguish viral
from bacterial disease o Identify the site of a localized infection
Temperature Variability
Individual to individuals
Diurnal variation in temperature o Circadian rhythm
Oral, axillary or rectal temperatures
Responses among children and adults
Physiological and pathologic states causing fever Physiologic Fever States
Digestion
Exercise
Ovulation
Pregnancy
Warm environment
Emotion Pathologic Causes
Infection
Inflammation (e.g., connective tissue disease)
Neoplasms
Vaccines
Dehydration Common Causes of Fever
Minor Illnesses Major Illnesses
URTI Viral exanthema Gastroenteritis
Bacterial meningitis UTI Pneumonia Malaria
Mechanisms of a Protective Effect of Fever
Enhanced neutrophil migration
Increased production of antibacterial substances by neutrophils
Increased production of interferon
Increased antiviral and antitumor activity of interferon
Increased T-cell proliferation
Decreased growth of microorganisms in iron-poor environment
Extreme Hyperpyrexia (>41C) and Hypothermia
Extreme Pyrexia Hypothermia
Central fevers (neoplastic, trauma, or infection)
Drug fever Heat stroke HIV Malignant hyperthermia Malignant neuroleptic syndrome
Elderly and the newborn (poikilotherms)
Cold exposure Hypothyroidism Overwhelming infection Sepsis in CRF Overzealous treatment
with antipyretics
Pathogenesis of Fever
2 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Fever Patterns
Intermittent exaggerated circadian rhythm that includes a period of normal temperature on most days
o High spikes with return to normal
Remitting persistent and varies by more than 0.5C for >24 hours (like intermittent but never returns to normal)
Sustained persistent and does not vary more than 0.5C for >24 hours (like remittent but with less marked swings of temperature)
Relapsing febrile periods that are separated by intervals of normal temperature
o Tertian 1st and 3rd days (P. vivax) o Quartan 1st and 4th days (P. malariae)
Spectrum of Fever Syndromes
Fever without localizing signs
Fever of short duration associated with infection
Recurrent and prolonged fever associated with infection
Fever with a rash
Fever in association with a chronic disease
Fever associated with a collagen diseases
Fever associated with malignancy
Drug fever
Factitious fever
Hospital-acquired Classic Working Definition
Continuous fever of at least 3 weeks duration with daily temperature elevation above 38.3C and remaining undiagnosed after 1 week of intensive study in the hospital
Working Definition (Pediatric)
Fever >38C persisting as a predominant feature for more than 7-10 days in a child in whom a careful, thorough history and physical examination and preliminary laboratory data fail to establish a diagnosis
Fever of Unknown Origin in:
Infectious Causes Noninfectious Causes
Common Mastoiditis (chronic); Sinusitis Salmonellosis Subdiaphragmatic abscess Cytomegalovirus, EBV, Hepatitis viruses Tuberculosis Malaria VLM
Common Leukemia Lymphoma JRA SLE Periodic fever
Uncommon Perinephric abscess Pyelonephritis Psittacosis
Uncommon Polyarteritis nodosa Neuroblastoma Serum sickness
Rare Disseminated Histoplasmosis Toxoplasmosis SBE Leptospirosis
Rare Pancreatitis Drug fever
Etiology
1. Infectious causes 40-50% 2. Collagen-vascular 10-20%
Juvenile rheumatoid arthritis 10% SLE 3% HSP 1% Vasculitis 1%
3. Malignancies/Neoplasms 5-10% Occult solid tumors (neuroblastoma,
Wilms tumor, retinoblastoma) Hematologic tumors
(leukemia, lymphoma, Hodgkins) 4. Miscellaneous 10-15%
Factitious fever Metabolic (drug fever, milk allergy,
dehydrated diabetes insipidus) Genetic (familial Mediterranean fever,
Caffeys disease, hypothalamic dysfunction, hemoglobinopathy crisis)
5. Unknown/undiagnosed 20-30%
What is the Eventual Etiology of FUO in Children?
No. of Cases % of Cases
Infection Respiratory Others
198 102 96
44.4 22.9 21.7
Collagen disease 57 12.8
Inflammatory bowel disease 7 1.6
Neoplasm 25 5.6
No diagnosis 48 10.7
Resolved 56 12.6
Miscellaneous 54 12.1
Infectious
Most frequent o Typhoid fever, malaria, disseminated/miliary TB,
UTI, hidden (cryptic) abscesses, septicemia
Typhoid fever o Stepladder pattern (sustained, progressive) o Fever will not be shorter than five days to a week o Faget sign pulse-fever disproportion
Differential Diagnosis Salmonella Infection (Typhoid Fever)
Seriously and obviously ill with no apparent cause
Abdominal tenderness
Shock
Confusion
Child with sickle-cell disease
Osteomyelitis/arthritis infant
Anemia associated with malaria Malaria
Residence in an endemic area
Blood film positive
Severe anemia
Enlarged spleen
Jaundice Miliary (Disseminated) Tuberculosis
Weight loss
Anorexia, night sweats
Systemic upset
Enlarged liver and/or spleen
Cough
Tuberculin test positive or negative
Sterile pyuria
X-ray shows very widespread small discrete shadows in both lung fields
Sequel of hematogenous dissemination of the organism, which may develop in the post-primary phase or in patients with long-standing latent infection
Also encountered in patients with immunosuppression Pyelonephritis
CVA or suprapubic tenderness
Crying on passing urine
Passing urine more frequently than usual
Incontinence in previously continent child
White blood cells and/or bacteria in urine on microscopy Abscess
Fever with no obvious focus of infection (deep abscesses)
Local tenderness or pain
Fluctuant mass
Specific signs depend on site subphrenic, psoas, lung, renal, retroperitoneal
Follows surgical operations or manipulation/ umbilical catheterization
Septicemia
Seriously and obviously ill, highly-febrile child with no focus of infection
Purpura, petechiae
Shock or hypothermia in a young infant
(+) blood cultures Adage FUO is more likely to be caused by an unusual presentation of a common disorder than a common manifestation of a rare disorder. 3 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Evaluation History
Thorough history essential
Careful description of the fever pattern
Attention to associated findings at the time of fever, including tachycardia, skin warmth, and diaphoresis
Exposure to ill contacts
Animal exposure
Travel history (recent and remote)
Dietary history (raw meat/fish ingestion; unpasteurized milk; pica)
Family history (genetic-ethnic background) History of Exposures
Ill persons TB, salmonellosis, viral
Animals Zoonosis, bartonellosis (cat scratch disease), leptospirosis, toxoplasmosis
Travel Malaria, fungi, others
Food Trichinosis
Soil VLM, toxoplasmosis
Drugs Drug fever (antibiotics)
Insects Rickettsia, malaria, others
Clues in the History that may Localize the Infection
Sore throat Strep tonsillitis
Cough, rusty sputum Lobar pneumonia
Severe joint pain/swelling Pyogenic arthritis
Severe pain in head and back of the neck with stiffness
Meningitis
Severe pain in a bone Osteomyelitis
Tender liver Amebic liver abscess Viral hepatitis
Ill-defined subcutaneous inflammation Cellulitis, pyomyositis
Bloody diarrhea Shigella, Campylobacter
Chronic Diseases Complications
Congenital heart diseases Bacterial endocarditis
Cyanotic heart disease Cerebral abscess
Rheumatic fever Bacterial endocarditis Recurrence of RF
Shunted hydrocephalus Shunt infection Ventriculitis, septicemia
Chronic renal disease Urinary tract infection
Congenital or acquired immunodeficiency
Opportunistic organisms, fungi, parasites
Recent surgery Concealed abscesses
Physical Examination
Complete, detailed, thorough and diligent
Repeated frequently. Constant reassessment for specific focal signs can pick up growing tumors/masses/abscesses, changing murmurs or early jaundice
Look for a focus of infection or findings to suggest an occult infectious process; findings suggesting a non-infectious disease
Temperature Chart
Document genuine fever
Clues often overlooked or their significance not appreciated o Review by someone without prior knowledge of
previous finding simportant
Discern fever pattern Approach to the Diagnosis
Exclude factitious fever
Observation of fever in the hospital for 48 hours without antipyretics and observing the pattern
Laboratory studies should be organized towards the most likely diagnosis after complete history and physical examination
Laboratory
The most broadly-based, highest-yield, and most cost-effective testing is done first, and the lowest-yield, most esoteric testing is done last if no diagnosis is made despite persistence of symptoms
Directed toward the most likely diagnostic studies Laboratory Data and Ancillary Procedures
Preliminary o CBC with differential count, platelet count o ESR o Aerobic and anaerobic blood cultures o Urinalysis and urine culture o Chest x-ray film o Tuberculin test o Serologic testing useful in salmonellosis,
brucellosis, tularemia, rickettsial infections o Any other tests indicated by the history or physical
findings
Analyze all data How helpful are CT scans and nuclear medicine studies to determine diagnosis?
Minimally o In prospective study of 109 patients with FUO,
scanning procedures have very low utility in the absence of clinical findings that suggested a localized process
o Bone marrow examination had little value when hematologic abnormalities were lacking in the peripheral blood
Treatment
Stop all medications
Therapeutic (empirical) trials o Suspected disseminated TB o Systemic JRA o Malaria o Rheumatic fever o Culture-negative endocarditis
Diagnosed conditions treat accordingly
Pallor
Jaundice
Rashes/purpura
Mucocutaneous manifestations of systemic diseases
Murmurs or changing murmurs
Abdominal masses or tenderness
Hepatomegaly
Splenomegaly
Lymphadenopathy
Scalp
Eyes
Fundi
Ear drums
Nose
Throat
Gums
Teeth
Sinuses
Bones
Joints
Muscles
External genitalia, pelvic organs
Urine
4 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Gram-Positive Organisms Causing Pyogenic and other Infections
1. Staphylococcus species
a. S. aureus b. S. epidermidis
2. Streptococcus a. S. pneumonia (Pneumococcus) b. Group A Streptococcus (S. pyogenes)
Staphylocccus species
Aerobic cocci that grow in pair or clusters
Either coagulase-positive (S. aureus) or coagulase-negative (S. epidermidis, S. saprophyticus, S. haemolyticus)
Part of the normal flora of humans Pathogenesis
Disease may result from tissue invasion or injury caused by various toxins and enzymes produced by the organisms
Virulent factors o Loose polysaccharide capsule (slime layer) o Coagulase (clumping factor) o Protein A o Catalase, Panton-Valentine leukocidin (PVL),
hemolysins o Penicillinase or -lactamase o Exotoxins ( Exfoliatin A and B) o Enterotoxins (A, B, C1 , C 2 , D, E) o Toxic shock syndrome toxin-1 (TSST-1)
Staphylococcus aureus
The most common cause of pyogenic infections of the skin and soft tissue
o Impetigo, furuncle (boils), cellulitis, abscess, lymphadenitis, paronychia, omphalitis, and wound infection
o Bacteremia osteomyelitis, suppurative arthritis, deep abscesses, pneumonia, empyema, endocarditis, pyomyositis and pericarditis
Toxin-mediated diseases o Food poisoning o Staphylococcal scarlet fever o Staphylococcal skin scalded syndrome (SSSS) o Toxic shock syndrome (TSS)
Clinical Manifestations
The signs and symptoms vary with the location of the infection, which is most commonly the skin but may be any tissue
Skin infections are more prevalent among person living in low socioeconomic circumstances and particularly those in tropical climates
Staphylococcus epidermidis (Coagulase-Negative Staphylococci) (CONS)
Cause infections in patients with indwelling foreign devices intravenous catheters, hemodialysis shunts and grafts, CSF shunts, peritoneal dialysis catheters, prosthetic cardiac valves and prosthetic joints.
Common cause of nosocomial neonatal infection Direct Invasion: Infections Originating from Hair Follicles
Folliculitis Furunculosis
Carbuncle Furunculosis
Impetigo contagiosa
Honey-colored crusts Impetigo Contagiosa and Impetigo Bullosa
Impetigo contagiosa o The classic lesion begin as erythematous papules in
traumatized areas. They quickly evolve into honey-colored crusted plaques with surrounding erythema
o GAS and S. aureus are the chief causative agents
Impetigo bullosa o Exclusively staphylococcal in origin o The characteristic lesion are caused by
epidermolytic toxin
Impetigo contagiosa Impetigo bullosa
Other Staphylococcal Localized Skin Infections
Abscess Cellulitis
Staphylococcal Pneumonia
A. 5 year old child with S. aureus pneumonia initially demonstrated consolidation of the right middle and lower zone.
B. Seven days later, multiple lucent areas are noted as pneumatoceles develop.
C. Two weeks later, significant resolution is evident, with a rather thick-walled pneumatocele persisting in the right midzone associated with significant residual pleural thickening.
5 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Toxin Mediated Disease: Staphylococcal Scalded Skin Syndrome (Ritter disease)
Occurs predominantly in infants and children < 5yr of age
The onset of the rash may be preceded by a prodrome of malaise, fever, and irritability associated with exquisite tenderness of the skin or the appearance of generalized erythema may be abrupt without preceding symptoms.
The erythematous skin may rapidly acquire wrinkled appearance and sterile, flaccid blisters and erosions develop
Circumoral erythema or radial crusting and fissuring around the eyes, mouth, and nose (sunburst pattern)
Areas of epidermis may separate in response to gentle shear force (Nikolsky sign)
Caused by phage group 2 staphylococci, strains 71 and 55
Sunburst pattern
Management of Staphylococcal Infection
Appropriate antibiotics o Oral cloxacillin o Parenteral oxacillin, Cefazolin, vancomycin
[MRSA]+ clindamycin
Incision and drainage
Other supportive measures (toxin-mediated) o fluid replacement o Application of emollient
Streptococccus pneumoniae (Pneumococcus)
Gram positive, lancet-shaped polysaccharide encapsulated diplococcus
Encapsulated strains cause most serious disease in humans (otitis media, life threatening pneumonia, bacteremia, and meningitis)
Capsular polysaccharides impede phagocytosis
Virulence is related in part to capsular size
Invasive strains 4, 6B, 9V,14, 18C, 19F & 23F
Most healthy individuals carry various S. pneumoniae serotypes in their upper respiratory tract (>90% of children 6 mo and 5 yr)
Children 100 serotypes
Types 1, 12, 28, 4, 3, and 2 the most common causes of uncomplicated streptococcal pharyngitis
M types associated with pharyngitis rarely cause skin infections
M types associated with skin infections rarely cause pharyngitis
Pharyngeal strains (M type 12) is associated with glomerulonephritis, while others are associated with rheumatic fever
More of the skin strains (M types 49, 55, 57 and 60) nephritogenic
Produces a large variety of enzymes and toxins (Streptococcal pyrogenic exotoxins A, B, and C)
Common cause of infections of the upper respiratory tract (pharyngitis) and the skin ( impetigo, pyoderma) in children
Also cause distinct clinical entities (scarlet fever and erysipelas), as well as toxic shock syndrome and necrotizing fasciitis
Causes potentially serious nonsuppurative complications: rheumatic fever and acute glomerulonephritis
Pathogenesis: Direct invasion and toxin mediation
Diagnosis: Throat swab culture or rapid antigen detection test
Non-specific pharyngotonsillitis
Highly suggestive of group A -streptococcal infection: palatal petechiae 6 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Scarlet Fever
Toxin-mediated disease
Rash appears within 24-48 hours after onset of symptoms
Begins around the neck and spread over the trunk and extremities
It is a diffuse, finely papular, erythematous eruption producing a bright red discoloration of the skin, which blanches on pressure.
More intense along the creases of the elbows, axillae, and groin
The skin has a goose-pimple appearance and feels rough
The face is usually spared, although the cheeks may be erythematous with circumoral pallor
After 3-4 days, the rash begins to fade and is followed by desquamation
The tongue is usually coated and the papillae are swollen (strawberry tongue)
White strawberry tongue Red strawberry tongue Treatment of Streptococcal Infection
Non-invasive: Penicillin
Invasive: Penicillin + Clindamycin Debridement
An Overview of Mucocutaneous Symptom Complex
An exanthem is a skin eruption occurring as an integral part of an infectious disease. The corresponding changes in the mucous membranes is an enanthem. Accurate diagnosis is not always possible on preliminary examination judgment should be deferred until the rash develops. Classification
Maculopapular eruption
Vesiculobullous or vesiculopustular
Petechial or purpuric eruption Measles
Conjunctivitis with photophobia
Kopliks spots graying white dots usually as small as grains of sand, with slight, reddish areola
Distribution of rash o Starts behind the ears along the hairline, face the
spreads downward over the body o More confluent on the upper part, discrete on the
lower part
Brownish discoloration and branny desquamation Rubella (German Measles)
Distribution of rashes are similar to measles but they are discrete and not associated with desquamation
o Rash starts from the trunk and spreads to the neck, face and proximal extremities
Forchheimer spots red spots are often seen on the palate Congenital Rubella Syndrome
Congenital defects occur in >80% of infants infected in the first trimester and virtually no defects are identified in infants infected after the first 16 weeks of gestation
Infants infected in the first trimester are more likely to have multiple congenital defects, whereas those infants infected after 11-12 weeks of gestation are more likely to have only deafness or retinopathy
Clinical Findings in Congenital Rubella Syndrome
General: IUGR, hepatosplenomegaly, chemical evidence of hepatitis
CNS: Mental retardation, behavioral disorders, hypotonia, seizures, CSF protein
Cardiac: PDA, peripheral and valvular pulmonary stenosis, aortic stenosis, VSD
Ocular: Cataracts, salt and pepper retinopathy, corneal clouding, glaucoma
Orthopedic: Radiolucencies in long bones
Hematologic: Transient thrombocytopenia with purpura
Dermatologic: Blueberry muffin spots, dermatoglyphic
Endocrine: Diabetes in 2nd or 3rd decase Prevention
Active immunization (15 months, 4-6 years)
Passive immunization for exposed pregnant woman o Gamma-globulin (.55 mL/kg)
Enteroviral Exanthem Infectious Mononucleosis
Exudative pharyngitis
Cervical lymphadenopathy
Hoaglands sign lid edema Typical Serologic Findings Related to the Stage of EBV Infection
Stage of Infection Presence of Antibody
Primary VCA IgM or IgG (usually high) EA (usually high), no EBNA
Convalescent or Past VCA IgG EA (low), EBNA
Reactivation VCA IgG (high) EA (high), EBNA
7 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Fever with Vesicular/Bullous and Pustular Rash
Risk Factors for Progressive Varicella
Immunodeficiency
Malignancy
Corticosteroid therapy
Pregnant
Newborns
Adolescents and adults Treatment
Neonatal varicella o VZIG and acyclovir
Herpes zoster o Acyclovir (oral) 20 mg/kg/dose max 800 tid to
shorten the course (optional)
Primary varicella o Option of no treatment in a healthy child, unless
with complications treatment with IV acyclovir at 500 mg/m2 or 10 mg/k/dose every 8 hours for 7 days
Prevention
Varicella vaccine live attenuated vaccine given in 2 doses (12-18 months and at 4-6 years)
Post-exposure prophylaxis options o Varicella vaccine within 3-5 days from exposure o VZIG within 96 hours from exposure (recommended
for immunocompromised children, pregnant women, and exposed newborns)
o Pooled IVIG within 96 hours from exposure Pathogenesis
Viral infection begins at a cutaneous portal of entry such as the oral cavity, genital mucosa, conjunctiva, or breaks in keratinized epithelia
Replicate in the sensory neurons, released in nerve endings and replicate again on the skin
Viremia does not play a role in immunocompetent hosts HSV Encephalitis
Leading cause of non-epidemic, sporadic encephalitis in children and adults
It is an acute necrotizing infection involving the frontal and/or temporal and limbic system.
Beyond the neonatal period, it is almost always caused by HSV-1.
Symptoms are non-specific but focal signs are common. If untreated, it can progress to come and death in 75% of cases.
Non-Polio Enteroviruses
Picornaviridae family
Very common viruses with a worldwide distribution
Produce a broad range of important illnesses
The genus name reflects the importance of the GI tract as a primary site of viral invasion and replication and transmission
Enteroviral Exanthems Maculopapular
Rubelliform Echovirus 9, also 2, 4, 11, 19, 25 (Size 1-3 mm) Coxsackie virus A9
Roseola-like Echovirus 16 (Boston exanthema) (Size 5-15 mm) Echovirus 11, 25 Coxsackie virus B1, B5
Vesicular
Hand, foot Coxsackie virus A16, and mouth also A5, A7, A9, A10, B2, B5
Herpangina Coxsackie virus A22, also A1-A10, A16
Insect bite-like Coxsackie virus A4; crops, last 1-2 weeks
Non-pustule Coxsackie virus, also Echovirus 11, 30 forming
Petechial
Coxsackie virus B, A9; echovirus 9
Problem separating from meningococcemia Hand, Foot and Mouth Disease
Most commonly caused by Coxsackie virus A16
The oropharynx is inflamed and contains scattered vesicles on the tongue, buccal mucosa, posterior pharynx, palate, gingiva and/or lips
Maculopapular/vesicular lesions appear in the hands/fingers, feet. Lesions are tender and are common on the dorsal surfaces but can also occur on the palms and soles.
HFMD caused by Enterovirus 71 is more severe with increased rates of neurologic disease.
Herpangina
Sudden onset of fever, sore throat, dysphagia
Characteristic lesions present on the anterior tonsillar pillars, soft palate, uvula and posterior pharyngeal wall, occasionally posterior buccal mucosa
Discrete vesicles that ulcerate, surrounded by erythematous rings
Resolves in 3-7 days Cutaneous Bacterial Infection Impetigo Contagiosa
Erythematous macules that very rapidly evolve into thin-walled vesicles and pustules
The vesiculopustular stage is brief and following rupture, sticky, heaped-up, honey-colored crusts are formed
Risk factors: insect bites, scabies, cutaneous injuries, and preceding dermatitis serve as portals of entry for the organism, which does not penetrate intact skin
Caused by group A -hemolytic streptococcus or S. aureus
Non-bullous or bullous impetigo o Bullous impetigo often caused by S. aureus, most
often phage group 2
Mainly an infection of infants and young children Dengue Fever/Dengue Hemorrhagic Fever Dengue Virus
Causes dengue and dengue hemorrhagic fever
Transmitted by mosquitos
Has 4 serotypes (DEN-1, 2, 3, 4) Aedes aegypti
Dengue transmitted by infected female mosquito
Primarily a daytime feeder
Lives around human habitation
Lays eggs and produces larvae preferentially in artificial containers
Clinical Characteristics of Dengue Fever
Benign syndrome
Biphasic fever
Headache
Myalgia
Arthralgia
Rash morbilliform, maculopapular
Leukopenia
Lymphadenopathy
Petechiae Dengue Hemorrhagic Fever
Known as Philippine, Thai or Singapore hemorrhagic fever, hemorrhagic dengue, acute infectious thrombocytopenic purpura
Severe disease
Occurs where multiple types of dengue virus are simultaneously or sequentially transmitted
Difference in severity is due to difference in immunologic status
Characterized by: o Thrombocytopenia o Increased capillary permeability o Coagulopathy
Phases of Dengue
Febrile phase. Fluid-like symptoms and dehydration
Critical phase. Cardiovascular compromise to shock from leaky capillaries, thrombocytopenia and coagulopathy
Recovery phase. Reabsorption of fluid, may develop hypervolemia in overly aggressive fluid management.
8 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Suggested Dengue Case Classification and Levels of Severity
Course of Dengue Illness
Immunity
Incubation period is 4-10 days
Primary infection can produce a wide spectrum of disease
Individuals are protected from critical illness with other serotypes following a primary infection for the first 2-3 months but with no long-term cross-protective immunity
Risk Factors that Determine Disease Severity
Secondary infection
Age
Ethnicity
Co-morbid chronic diseases: cardiac, pulmonary
Children in general are less able to compensate with capillary leakage than adults
Severe dengue should be considered if the patient is from an area of dengue risk presenting with fever of 2-7 days plus any of the following features:
There is evidence of plasma leakage, such as: o High or progressively rising hematocrit o Pleural effusions or ascites o Circulatory compromise or shock (tachycardia, cold
and clammy extremities, capillary refill time greater than three seconds, weak or undetectable pulse, narrow pulse pressure or, in late shock, unrecordable blood pressure)
There is significant bleeding
There is an altered level of consciousness (lethargy or restlessness, increasing or intense abdominal pain, jaundice)
There is severe organ impairment (acute liver failure, acute renal failure, encephalopathy or encephalitis, cardiomyopathy) or other unusual manifestations
Hemorrhagic Manifestations of Dengue
Skin hemorrhages: petechiae, purpura, ecchymoses
Gum bleeding
Nose bleeding
Gastrointestinal bleeding: hematemesis, melena, hematochezia
Hematuria
Increased menstrual flow Warning Signs for Dengue Shock Four Criteria for DHF
Fever, or recent history of acute fever
Hemorrhagic manifestations
Low platelet count (100,000/mm3 or less)
Objective evidence of leaky capillaries (excessive capillary permeability)
o Elevated hematocrit (20% or more over baseline) o Low albumin o Pleural or other effusions
Initial Warning Signals
Disappearance of fever
Drop in platelets
Increase in hematocrit When Patients Develop DSS
3-6 days after onset of symptoms Alarm Signals
Severe abdominal pain
Prolonged vomiting
Abrupt change from fever to hypothermia
Change in level of consciousness (irritability or somnolence) Differential Diagnosis
Chickungunya (Africa, India, Southeast Asia)
Onyong-nyon (East Africa)
West Nile fever (Europe, Africa, Middle East, India)
Four arboviral diseases with dengue-like course without rash o Colorado tick o Rift valley o Ross river o Sandfly
Meningococcemia and rickettsial diseases Conditions that Mimic the Febrile Phase of Dengue
Flu-like Influenza, chikungunya, infectious mononucleosis
Fever with Rash Rubella, scarlet fever, meningococcal, drug reactions
Diarrheal diseases Rotavirus, other enteric infections
Infections Acute gastroenteritis, malaria, typhoid, leptospirosis, bacterial sepsis and septic shock
Malignancies Acute leukemia and other malignancies 9 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Dengue Hemorrhagic Fever
Complications
Carditis
Encephalitis
Severe pleural effusion
End organ damage in severe shock CNS Management
No hemorrhagic manifestations, patient is well-hydrated home treatment
Hemorrhagic manifestations or hydration borderline consider hospitalization
Warning signs (even without profound shock) or DSS hospitalize
Meningococcal Infections
Caused by N. meningitides
Cell wall has lipid A-containing lipo-oligosaccharides, with endotoxin and covered by polysaccharide capsule
At least 13 serotypes identified
Majority of diseases worldwide are caused by serogroups A, B, C, w 135 and Y
Serogroup B most common in infants
Death usually occurs within 6-18 hours Clinical Manifestations
Spectrum o Occult meningococcal bacteremia
Fever with or without symptoms o Meningitis develops in 58% of cases o Acute meningococcemia
Meningococcemia
Purpuric viral exanthema
Petechial facial rash
Ecchymotic lesions Epidemiology
Endemic disease
Male predominance
Invasive disease is most common among young children
50% occur in 25 cases/100,000 in the 1st 4 months of life
25% in people >30 years Pathogenesis
Acquired via respiratory route
Nasopharyngeal colonization usually asymptomatic (because it produces IgA protease)
Meningococcal pili attach to CD46 proteins that induces microvilli formation and endocytosis of the microbe which gain access to the bloodstream
These interactions lead to the production of proinflammatory cytokines, activation of extrinsic and intrinsic coagulation cascade
Capillary leak, DIC leads to multiple organ failure, septic shock and sometimes death
Heart, CNS, skin, adrenals are all affected
Myocarditis >50% of patients
Diffuse adrenal hemorrhage (Waterhouse-Friderichsen syndrome)
Host Factors
Congenital deficiencies of complement components: properdin, factor D and terminal components
Acquired complement deficiencies: SLE, nephrotic syndrome, hepatic failure
Diagnosis
High index of suspicion
Isolation by culture from sterile sites: blood, CSF, synovial fluid
Gram stain and culture of petecchial or purpuric lesions
Gram stain of buffy coat of spun blood Treatment
Drug of choice: Penicillin G 250,000 to 400,000 u/k/day
Cefotaxime and ceftriaxone alternative treatments
Supportive care fluids, vasopressor support, component transfusions
Complications
Acute complications related to vasculitis, DIC and hypotension
Gangrene, adrenal hemorrhage, endophtalmitis, arthritis, endocarditis, pericarditis, pneumonia, renal infarcts, avascular necrosis of epiphyses
Deafness most frequent neurologic sequelae (5-10% of children with meningitis)
Poor Prognostic Factors
Mortality rate as high as 110%
Hypertension, hypothermia or extreme hyperpyrexia on presentation
Purpura fulminans/ptechiae 55 years
Vaccine is unreliable in
Meningitis
Acute Subacute/Chronic
Acute bacterial meningitis Viral meningitis
TB meningitis Fungal meningitis Partially treated BM
Clinical Presentation
Meningitis produces increased ICP because of cerebral edema, obstruction to flow and absorption of CSF and impairing venous outflow
Newborn early and late onset infection o Initial symptoms are non-specific o Later: fever, seizures, bulging fontanel, shock
Older children fever, irritability, neck stiffness, seizures
Kernigs sign, Brudzinskis sign Acute Purulent Bacterial Meningitis: Main Etiologic Agents
Newborn to 3 months o Group B streptococci o E. coli o L. monocytogenes o Enterococci o S. pneumonia
3 months to 6 years o H. influenzae, Type B o S. pneumonia o N. meningitides
>6 years o N. meningitides o S. pneumonia
Viral Meningitis (Aseptic Meningitis)
Usually accompanying the viral infection
Patient manifests signs of the viral infection, as diarrhea, respiratory, mumps, measles, varicella
CSF is usually normal, or with slight increase in cells or protein
Treatment is supportive as the course itself is self-limiting Diagnosis and Treatment
CSF analysis
Neuroimaging if with focal deficits or non-response to tx
Blood, tissue culture
Treatment: antibiotics, antiviral, Anti-Kochs CSF Findings
Type Appearance WBCS Glucose Proteins
Acute Bacterial
Turbid Increased with PMNs predominating
Low Increased
Viral Clear, colorless Normal or slightly increased
Normal Normal or slightly increased
TB/ Fungal
Xanthochromic Increased with lymphocyte predominating
Low Markedly increased
Lumbar Puncture and CSF Analysis
Opening/closing pressure
Appearance clear, colorless, xanthochromic, bloody, turbid, purulent
White blood cell count and differential
Glucose concentration
Protein concentration
Gram stain, AFB stain, fungal stain
Culture
PCR Treatment (Antibiotics used in the treatment of bacterial meningitis depending on the age of the patient and possible agent) Amikacin Ampicillin Cefotaxime
Ceftriaxone Gentamicin Meropenem
Nafcillin Penicillin G Rifampicin
Tobramycin Vancomycin
Duration of Treatment
For uncomplicated PCN sensitive S. pneumonia meningitidis = 10-14 days
For resistant strep vancomycin
For uncomplicated N. meningitidis, IV PCN for 5-7 days
Uncomplicated H. influenza Type B 7-10 days
Gram (-) meningitis 3rd generation cephalosporin, 3 weeks
Complications 1. Seizures 2. Subdural effusions 3. SIADH 4. Prolonged fever 5. DIC
Sequelae 1. Epilepsy 2. Learning disability 3. Motor deficit 4. Visual/hearing
impairment TB Meningitis Three Stages
1. Prodromal fever, headache, irritability 2. Neurologic signs meningeal signs, decreased level of
sensorium 3. Coma, irregular respiration, rigidity, opisthonus
Diagnosis
Clinical: subacute course, (+) TB exposure, (+) tuberculin test
CSF: ground glass appearance with tendency to coagulate, increased WBC with lymphocytosis, extrememly high protein, low sugar
Acid fast staining (+), PCR, ELISA or latex agglutination antigen Treatment
Quadruple anti-TB regimen (HRZ(S/E) 2 mos, HR 10 mos)
Steroids
Treat complications metabolic
Manage increased ICP, hydrocephalus Encephalitis
Usually viral 1. Herpes simplex virus. Presents with signs of increased ICP,
infection and deterioration in consciousness. Tx: acyclovir. 2. Dengue encephalopathy/encephalitis. 3. Subacute sclerosing panencephalitis (SSPE). Subacute and
degenerative disease of the entire brain. Symptoms: behavioral and personality changes, myoclonic jerks, decrease in cognitive function.
Clinical Features
Subtle changes in behavior
Stage 1. Mild symptoms, fever, headache, irritability.
Stage 2. Myoclonus, jerks, but consciousness retained.
Stage 3. Involuntary movements disappear, rigidity, decreased sensorium.
Stage 4. Critical stage with breathing and circulatory problems and death.
SSPE
Diagnosis Treatment
Clinical course (+) measles antibody in the CSF Characteristic EEG findings
No treatment Antiviral drugs under investigation Supportive care
Pseudotumor Cerebri
Condition characterized by increased ICP, normal CSF cell count and protein, normal ventricular size documented by MRI
Causes: metabolic, steroid therapy, hypervitaminosis A, obesity, intake of tetracycline, among others
Clinical features: headache, diplopia, papilledema
Diagnosis: CT, MRI, lumbar tap, CSF analysis
Treatment: determination of the underlying cause, decreased ICP, specific treatment
11 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Pediatric Neurology: Seizures in Children Rosalinda Q. de Sagun October 4, 2007
** Notes in red are from whoever owned the handout I photocopied. Thank you! :3 Seizures
- Refers to excessive neuronal discharge with change in motor activity or behavior (but not always motor manifestation
- Not a diagnosis but a SYMPTOM of an underlying CNS disorder Convulsions
- Refers more to motor attacks Partial Seizures
- Classification depends on site and size of focus
- Focal lesions in brain - From one side/part of brain
A. Simple partial seizures
- Maintained consciousness; may verbalize during the seizure
- Motor (Jacksonian marchfinger, hand, arm, shoulder, face), sensory (numbness in one area), autonomic, psychic
- Pins and needles somatosensory - No automatism
- Often referred to as auras which may consist of an abnormal sensation (e.g., smells, flashing lights) & experimental phenomena (e.g., dj vu)
- Presence of auras always suggests a focal onset of seizure
- If seizure is restricted to a small area in the motor cortex, there is focal clonic activity
B. Complex partial seizures
- Also known as temporal lobe seizures (no longer used) & psychomotor seizures
- With impairment of consciousness (usually starts at prolonged blank stares; confused, prolonged dream-like state)
- Most often behavioral manifestations (violent, disoriented, talking nonsense)
- Involves association fibers
- Temporal & partial cortex lesions - Parietal, frontal, temporal, etc. can also present as this
- With automatism (common feature in children and infants develop after LOC)
- May begin with a simple partial seizure; when the seizure activity spreads, there may be impaired consciousness leading to a complex partial seizure
- Interictal EEG - (+) aura
- May remember start and end of seizure
C. Partial seizures with secondary generalization
- Spreading of the epileptiform discharge during CPS
- Simple or complex evolving to GTC Generalized Seizures
- Due to a diffuse lesion in the brain
- Both hemispheres - Usually from thalamus
- Always with loss of consciousness
A. Absence seizures
- Attacks of fixed blank stares (sometimes with automatisms)
- Always with loss of consciousness - Typical (usually very short duration; no longer than 30 seconds)
or atypical, only in seconds therefore person does not fall on the ground
- With automatic movements such as grimacing - Preschool & early school age renders to have poor academic
performance
- Can occur 20x a days - Diagnosis: EEG2-3 cycles/sec + manifestations =
typical/atypical, EEG not characteristic
- (-) aura - (-) post-ictal
- Hyperventilation for 3 minutes can activated absence seizures - Generalized 3-4 Hz slow spike and wave discharges
B. Tonic clonic
- Grand mal seizure - e.g. Petit mal/Benign febrile seizure (most benign)
- Most dramatic eyes roll upward/back, entire body musculature undergoes tonic contractions, rapidly become cyanotic in association with apnea
C. Tonic
- Extension
D. Clonic - To and fro movement; seizure
E. Myoclonic
- Sudden jerky movements shock like contractions - Difficult to treat; use 1 or 2 anticonvulsants
- Can be a seizure or not
- May be a presenting symptom of a hereditary inborn error of metabolism/ metabolic disease
- Poor prognosis (hard to treat & with underlying abnormality) Infantile Spasm
Severe epileptic syndrome (lifetime)
Very brief; (-) impairment of consciousness
F. Atonic (Astatic) - drop attack prone to injury - Sudden loss of muscle tone, sudden drop down
- Must be differentiated from syncopal attacks - With no risks or predisposing factors
G. Infantile spasms
- Salams seizure like saluting
- Jerky movements
- Attacks are myoclonic; spasm is flexion/bending - Very poor prognosis & difficult to treat
- May be seen in babies with previous asphyxia - Part of the West syndrome
H. Unclassified seizures
- Neonatal seizures rowing movement, grimacing, apnea - Subtle seizures of the newborn
Notes: Automatism alimentary including lip smacking, chewing, swallowing, excessive salivation (infants) semi-purposeful, incoordinated & unplanned gestrural automatisms like picking & pulling at clothing (older children)
Seizure-like Events
A. Night terrors
- Sleep disorder - Wakes up in the middle of the night, does not recognize
parents, does not know what is happening, does not remember anything the next morning
- Related to stress during the day - In contrast with nightmares in which case you remember
your dreams
- Like seizures seizures usually occurs during sleep - Usually seen in school-age children
B. Breath-holding spells
- infants cry holds breath becomes cyanotic (inadequate H20)
- difficult to manage
- check blood Hgb, if low give Fe - do not give anticonvulsants
C. Syncopal attacks
- Usually in adolescence
- Related to stress, heat & environment; Autonomics
D. Shuddering attacks - gigil
E. Pseudoseizures
- adolescents, psychogenic
F. GERD Note: To differentiate : EEG 12 - Pediatrics 2
Causes of Seizures
Non-neurologic Neurologic
Metabolic disorders Electrolyte imbalance (Na, Mg, Ca) Hypoglycemia (esp. newborn) Hypoxia Fever (benign febrile seizure) Systemic infections (UTI, Roseola infantum) Toxins Drug-related (INH low Vitamin B6 pyridoxine)
Tumors CNS malformations Vascular disorders Idiopathic epilepsy CNS infections
*Non-neurologic is easier to confirm Epilepsy
- Recurrent episodes of afebrile seizures - >2 unprovoked seizures occur at an interval >24 hours
- First attack of unprovoked seizures may not result into epilepsy - Type depends on the predominant seizure type e.g. grandmal, petit
mal Status Epilepticus
- Continuous seizures lasting longer than 30 minutes or serial seizures wherein between each seizure, there is no return of consciousness
- A medical emergency case
- Prognosis depends on how case was managed - Subtypes:
- Febrile seizures most common - Idiopathic status epilepticus e.g. patient with menignits
suddenly have seizures
- Symptomatic status epilepticus (tumor) result of an underlying neurologic disorder
- Can be caused by sudden discontinuation of anticonvulsants (most common cause)
- Give circulatory support - Medications:
- Diazepam: short acting; halt seizure - Phenytoin: expensive
- Phenobarbital: can have behavioral manifestations
- Valproate: myoclonic seizures - Phenobarbital coma: for anesthesia
Benign Febrile Seizure
- Temperature >38.4C, evidence of causative disease (CNS infection, metabolic abnormality)
- Children >1 month of age
- Simple: 15minutes in duration - May need investigation to rule out epilepsy
- With focal manifestations
- Increase risk for later epilepsy with the ff characteristics: - Presence of atypical features of seizure or postictal period
- (+) family history of epilepsy - Initial febrile seizure
B. Surgical
- for epilepsy - to cure intractable seizures unresponsive to anticonvulsants
- Vagus nerve stimulation - pacemaker
C. Ketogenic diet
- for epilepsy
- restrict quantity of CHO and CHON and most calories provided as fat
- e.g. Ketocal milk for epileptic patients; only for intractable patients
- must monitor acid-base balance Prophylaxis
- Controversial & prolonged prophylaxis is no longer recommended
- Anti-epileptic drugs (e.g., Phenytoin, Carbamazepine) have no effect gum swelling, behavioral changes
- Phenobarbital is effective but can decrease cognitive functioning
- Na Valproate is effective but there is an increased risk of fatal Valproate-induced hepatotoxicity (highest in 18 months
Complex first febrile seizures
Persistent lethargy
Strongly recommended for children on prior antibiotic therapy Evaluation
History exact description
PE, neuro exam
Age of onset
Precipitating events: prenatal/postnatal insults to the brain
Occurrences of prior seizures (non-convulsive)
Classify the seizure
Family history of seizure most idiopathic are genetic PE
Head size, dysmorphic features
Port-wine stain on face
Trauma
Increased ICP
Metabolic disorders r/t feeding Lab tests (no routine lab tests, to r/o provoked seizures)
Serum glucose determination
Electrolytes
Drug screens
BUN and creatinine
Thyroid function test Diagnosis
Usually clinical
EEG
Neuroimaging
Videotelemetry o Extended EEG (4 hours)
Management
If clearly metabolic, EEG is unnecessary
Treatment with antiepileptic drugs may be needed to control seizures until the underlying pathology responsible for acute encephalopathy is corrected
o 2 weeks seizure free
EEG
To classify the seizures
Provide additional clues in making an etiologic diagnosis
(N) EEG is of limited value and does not rule out that seizure episode has occurred (interictal normal EEG) SSPE periodic lateralized epileptic discharges
To differentiate paroxysmal events
Generalized vs. partial
(+) abnormality o 10-40% absent epileptic o 1-5% present non-epileptic
(may be a genetic variant carrier) Epilepsy
0-4 years old, >70 y/o (bimodal
Pediatric primary (idiopathic)
Older secondary Manifestations
NB hypoxia, metabolic, birth trauma
1-5 febrile seizures, intracranial infection
5-13 genetic epilepsies Neuroimaging
Partial onset (especially adult)
Progressive neurologic disease loss of milestones
Intractable seizures
Seizures increase in frequency and intensity despite adequate treatment
MRI > CT in sensitivity for structural epileptogenic foci Other ILAE Dx Scheme
Syndromic o Predominant seizure type o Age of onset
Infantile Spasm (West Syndrome)
Age-specific
3-12 months, peak 4-7 months
Infantile spasm, MR, hypsarrythmia on EEG
Brief but recurrent spasm (50-100x/d)
Flexor, extensor/mixed
Associated with perinatal injury Lennox-Gastaut Syndrome
Continuum of West
Seizure disorder, MR, atypical pattern on EEG (slower)
Seizure types are variable
Tonic more frequent
Resistant to therapy Benign Rolandic Epilepsy
3-13 years old, peak 9-10
Males > females
More frequent 2-3 hours (p-?) sleep
Grunting, facial twitching secondary generalized (bed wet?) (focal)
EEG: characteristic cortico-temporal spikes
Affected children otherwise normal
Complete remission 15-16 years old Childhood Absence
6-7 years old
Female > male
Simple (blank stare) or complex (automatism/motor component)
Increased Risk for Occurrence in Febrile Seizures
Complex febrile seizures
Family history
Seizures in Children: An Overview
** Additional notes from Panda Manilas photos.
Seizure. An abnormal excessive neuronal discharge arising from the brain, capable of causing alteration in function and/or behavior.
Convulsion. A type of seizure with a motor component.
Epilepsy. Recurrent unprovoked seizures (operational definition more than two.)
Diagnosis of Epilepsies
Everyone is entitled to a diagnosis for prognosis and management to be specific and precise.
The diagnostic label epilepsy is unsatisfactory for both physician and patient because epilepsy is not a single disease entity.
Conditions that Mimic Seizures
Breath-holding spells
Benign paroxysmal vertigo
Night terrors
Syncope
Shuddering attacks
Paroxysmal torticollis
Rage attacks
Masturbation
Hereditary chin trembling
Narcolepsy/cataplexy
Paroxysmal kinesigenic choreoathetosis
Pseudoseizures Absence Seizures
Blank stare beginning and ending abruptly and lasting only a few seconds
Rapid blinking
Chewing
Child is unaware of seizure
Learning difficulties are a possibility
What to do: o No first aid required o If first event, get medical evaluation
Infantile Spasms
Can occur between 6 months and 2 years of age
Appear in clusters of quick, sudden movements
Often mistaken for colic
Most often occur in relation to waking and sleeping times
What to do: o No first aid is needed o Observe duration of clusters o Talk to doctor
Atonic Seizures
Sudden collapsing and falling down
Atonic seizures come in clusters
In less than one minute, child usually regains consciousness and can stand and walk again
Often mistaken for clumsiness
What to do: o Keep child safe during the event o No first aid required o Get medical evaluation
Uses of EEG
Differential diagnosis for other paroxysmal events
Differentiate partial from generalized seizures
Identification of certain epilepsy syndromes
Abnormal epileptiform activities o Absent in 10-40% of epileptic patients o Present in 1-5% of non-epileptic people
Abnormalities of Tone and Movement in Children
Developmental Delay
Delayed acquisition of milestones expected for chronological age
Important to distinguish from progressive neurological disorders, which manifest as loss of previously acquired skills
Delays can involve any developmental parameter/s: motor, language, psychosocial
UMN LMN
Bulk Minimal atrophy Profound atrophy
Tone Increased; spastic Decreased; flaccid
DTRs Hyperreflexia Decreased/absent
Fasciculations Absent Present/absent
Babinski Present Absent
Sensory deficit May be present May be present
Clinical Clues
1. Central Nervous System. UMN (spasticity, hyperreflexia); may be accompanied by cerebral manifestations (seizures, cognition, language and sensory problems)
2. Peripheral Nervous System. LMN (decreased to absent reflexes, flaccid) Guillain-Barre Syndrome
o Acute, autoimmune, polyradiculoneuropathy affecting the PNS, usually triggered by an acute infectious process
o Exhibits an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face, along with complete loss of DTRs
Hypotonia o Low neck muscle tone o Child hangs upside down U with little or no
movement o May be transient only child may become spastic
later o More difficult to teach how to walk o Rag doll sign o Floppy
Disorders of the motor system may be:
1. Acute strokes/vascular, metabolic disorders, infections o Seizures
2. Chronic cerebral palsy (static), congenital CNS lesion, degenerative disorders (progressive)
Cerebral Palsy
2/1000
Most common cause of delay
Laymans term
Referred to a group of disorders characterized by motor disorders (tone, posture, or movement), which are neither progressive nor episodic
o Progressive degenerative disease o Episodic seizures
The brain lesions are static and result from disorders of early brain development, usually insults in the perinatal period
o Most consistent factor anoxia, hypoxia of the newborn
o 40% (
(N) reversal of tone from: Axial hypotonia hypertonia Appendicular hypertonia hypotonia Traction test most sensitive test for hypotonia Clinical Manifestations
1. Delay in development (i.e., poor head control, delays in gross motor or fine motor development)
a. Poor head control (usually achieved by 4 months) b. Delays in gross motor or fine motor development
2. Motor deficit depending on the area of the brain involved and usually the risk factors present
3. Associated developmental disabilities mental retardation, epilepsy, visual, hearing, speech and behavioral abnormalities
** 2, 3 severity is proportional Motor Disorders in CP Three main criteria in classification:
1. Type of motor disorder 2. Topographical distribution 3. Gross motor function
Types of Cerebral Palsy and the Major Causes
Physiologic Topographic Etiologic Functional
Spastic Athetoid Rigid Ataxic Tremor Atonic Mixed Unclassified
Monoplegia Paraplegia Hemiplegia Triplegia Quadriplegia Diplegia Double hemiplegia
Prenatal (e.g., infection, metabolic, anoxia, toxic, genetic, infarction) Perinatal (e.g., anoxia) Postnatal (e.g. toxins, trauma, infection)
Class I no limitation of activity Class II slight to moderate limitation Class III moderate to great limitation Class IV no useful physical activity
Spastic Diplegia Periventricular leukomalacia
Prematurity Ischemia Infection Endocrine/metabolic/genetic
Spastic quadriplegia
PVL/multicystic encephalomalacia Malformations
Hemiplegia Stroke in utero or neonatal
Thrombophilic disorders Infection Genetic Hemorrhagic Infarction
Extrapyramidal/ Athetoid
Pathology in the basal ganglia, putamen, globus pallidus, thalamus
Asphyxia Kernicterus Mitochondrial Genetic/metabolic
Hemiplegia
Arm and leg on one side
Arm bent; hand spastic or floppy, often of little use
Walks on tiptoe or outside of foot on affected side
Other side completely or almost normal Paraplegia, Diplegia
Paraplegia both legs only
Diplegia with slight involvement elsewhere
Upper body usually normal or with very minor signs
Child may develop contractures of ankles and feet Quadriplegia
Both arms and both legs
Arms, head, and mouth may twist strangely when walking
Often have severe brain damage, are never able to walk again
Knees press together
Legs and feet turned inward Notes:
Spastic: seesawing of the legs; increase in tone
Athetoid: too much involuntary movement
Rigid: all throughout movement
Monoplegia: uncommon
Diplegia: both lower extremities
Hemiplegia: Right or left half of body
Quadriplegia: Upper and lower extremities
Jitteriness: observe during movement Child with spastic diplegia tip-toe always
*As long as insult happens in neonatal-perinatal period (4 months AOG to 1 months of life) child with meningitis can have cerebral palsy.
Clinical Manifestations
Movement disorders o Spasticity
UE: flexor spasticity LE: extensor spasticity
o Athetosis o Dystonia abnormal posturing o Rigidity o Ataxia ataxia - incoordination o Mixed motor problems
Associated with a spectrum of developmental disabilities: mental retardation, epilepsy, hearing and visual problems, speech, cognitive and behavioral
Physiological Classification Spastic Hemiplegia
spontaneous movements on the affected side
Arm > leg
Delayed walking until 18-24 months of age
Circumductive gait
Growth arrest in extremities especially if contralateral parietal lobe is abnormal
Spasticity in the affected extremities
Equinovarus deformity (walks on tiptoes)
Ankle clonus + Babinski sign
CT: atrophic cerebral hemisphere with dilated lateral ventricle contralateral to the side of the affected extremities
Spastic Cerebral Palsy
Less commonly the head and shoulders may stiffen forward, or the arms may stiffen straight across the body, with the head pressed back
Spastic Quadriplegic Cerebral Palsy
Head may twist to one side; shoulders, head may press back
Fist grips thumbs; arm: may stiffen straight out, or stiffen bent
Legs: may stiffen with knees pressed together, or turn in
Body may stiffen like a board
Stiffness, with the knees bent or with legs separated, occurs more commonly in the child with spasticity and athetosis combined
Most severe form because of involvement of all four limbs and the high association of mental retardation and seizures
Feeding problems (swallowing difficulty) are common due to supranuclear bulbar palsies
Common neuropathologic findings o PVL, multicystic encephalomalacia
Spastic Diplegic Cerebral Palsy
When you try to stand the child, the legs often stiffen
Bilateral spasticity of legs> arm
If severe, there is excessive adduction of the hips making diaper change difficult
Scissoring posture of lower extremities
PVL: most common neuropathologic finding
Prognosis for normal intelligence is good
Minimal seizure Athetoid Cerebral Palsy
Typical athetoid arm and hand movements may be as a regular shake or as sudden spasms. Uncontrolled movements are often worse when the child is excited or tries to do something.
Poor balance, arm and head movement
Child who learns to walk may do so in a stiff, awkward position, with the knees pulled together and bent. Feet often turn in.
Less common than the spastic type.
Hypotonic infants who do not have UMN signs but later on develop increased variable tone (rigidity) and dystonia and other dyskinesias
Intellect is preserved in many cases
Hypotonic with poor head control and marked head lag
Speech affected due to oropharyngeal muscle involvement
UMN signs (-), no seizures, preserved intellect
Most likely associated with birth asphyxia Ataxic Cerebral Palsy
To keep balance, child walks bent forward with feet wide apart, and takes irregular steps (like sailor on a rough sea, or someone who is drunk.)
16 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]
Diagnosis
Clinical diagnosis
Neuroimaging will document the extent of the structural pathology, aid in diagnosis and prognostication, and rule out treatable causes and slowly progressive neurological disorders
Thorough history to identify risk factors, developmental assessment, physical and neurological examinations
Hearing and vision screening
EEG if with seizures
If no possible etiology or risk factors for CP, do diagnostic tests o Neuroimaging CT/MRI
Document extent of structural pathology o Metabolic screening o Chromosomal studies
Differential Diagnosis
1. Motor delays from congenital structural malformations 2. Progressive disorders of the brain white matter diseases 3. Muscle disorders myopathies, dystrophies 4. Anterior horn cell disease spinal muscular atrophy (SMA)
Differentials
1. Dysmyelinating/demyelinating Disorders o Abnormal myelin formation as in some metabolic
disorders o Abnormal myelin secondary to brain insults from
infection, trauma, autoimmune o Clinically: white matter involvement, with
progressive neurologic deficits (spasticity, hyperreflexia, hearing and vision may be affected)
2. Spinal Muscular Atrophy o Disorders of the anterior horn cell o Usually progressive o Of three varieties depending on the age of onset of
symptoms Infantile type Wednig-Hoffman disease Intermediate Juvenile Kugelberg Welander disease
o Presents with LMN signs: fasciculations, floppy, areflexic
3. Muscle-Congenital Myopathy 4. Nerve disorders
o Manifestations of lower motor disease, decreased reflexes, tone (e.g., hereditary motor-sensory neuropathy)
Effective management requires:
1. Understanding of the pathophysiology of the disorder 2. Careful assessment of the patients capabilities and limitations 3. Knowledge of available treatment regimens, their applications
and limitations Management (Multidisciplinary)
1. Pediatrician 2. Neurologist: management of seizures, botulinum toxin
injections 3. Rehabilition specialists 4. Physical and occuptational therapists 5. Developmental psychologists 6. Education specialists 7. Orthopedic surgeons 8. Social workers
Autism Spectrum Disorder
Autism is a neurodevelopmental disorder of unknown etiology but with a strong genetic basis
Behavioral phenotype o Qualitative impairment in language/communication o Impaired social interactions and reciprocity o Lack of imaginative play
Signs and Symptoms
No pathognomonic symptoms or behavior
Most will present with: o Impairment in joint attention (use of eye contact
and pointing to share experiences with others), which normally develops at 18 months
o Lack of protoimperative pointing (to get an object of desire)
o Lack of protodeclarative pointing (to share an object of interest/naming)
o Lack of imaginative play
Manifestations
Poor eye contact
Verbal abilities vary: non-verbal to advanced speech
Speech may have odd prosody or intonation, echolalia, pronoun reversal, nonsense rhyming
IQ from MR to superior functioning
Stereotypical/ritualistic behaviors
Marked need for sameness Diagnosis
DSM-IV-criteria for autism
Modified checklist for autism in toddlers (M-CHAT)
Pervasive developmental disorder screening test (PDDST) Treatment
Multidisciplinary
Developmental pediatrician, pediatric neurologist, child psychiatrist
Psychologist
Occupational/speech therapist
Special education teacher (if necessary)
Medications depending on co-morbid conditions Attention Deficit Hyperactivity Disorder
Most common neurobehavioral disorder in childhood
Characterized by (DSM IV) o Inattention o Poor impulse control o Motor overactivity/restlessness
Symptoms should be present for more than 6 months in at least 2 settings are significantly affects social, academic or occupational functioning
Etiology
Multifactorial
Genetic susceptibility
Maternal complications during pregnancy
Maternal smoking and alcohol intake during pregnancy
Abnormal brain structures
Psychosocial family stressors exacerbate and/or contribute to the symptoms
Pathogenesis
Smaller prefrontal cortex and basal ganglia with 5-10% less blood flow in these areas.
o Rich in dopamine receptors dopamine hypothesis (disturbances in dopamine system are related to the onset of ADHD)
Diagnosis
Clinical interview and history o Fulfills criteria o Family history of ADHD o Family discord, situational stress
Behavior rating scales (e.g., Conners rating scale) help establish the magnitude and pervasiveness of symptoms and can be used for monitoring improvement with intervention
Treatment
Psychosocial treatments
Behaviorally oriented treatments
Medications: methylphenidate and atomoxetine Prognosis
Persists throughout the life span
Reduction in hyperactive behavior with age
Other symptoms: impulsivity, disorganization and inattention become prominent affecting relationships and occupational functioning
High risk for risk-taking behaviors (substance abuse) and psychiatric disorders
Notes in purple: Transcribed by: Fred Monteverde Notes from: Emy Onishi Cecile Ong Denise Zaballero Notes in red: whoever the notes on my photocopy came from. Thank you!
Fred Monteverde Emy Onishi Cecile Ong Mitzel Mata Regina Luz Section C 2009!
17 - Pediatrics 2 1st semester || AY 2012-2013 [email protected]