APPROACH TO DEMENTIA Guide: DR.PARIDHI SHIVDE

Canditate: DR.SARATH MENON .R

DEPARTMENT OF NEUROLOGY,

MGM MEDICAL COLLEGE ,INDORE.

DEMENTIA- “OUT OF ONE’S MIND”

DEMENTIA- the disease with acquired deterioration in cognitive/ intellectual abilities without impairment of consciousness

Cognitive deficit represent a decline from previous level of functioning

DSM – IV – DIAGNOSTIC CRITERIA

1. Memory impairment

2. At least one of the following: Aphasia Apraxia Agnosia Disturbance in executive functioning

3. Disturbance in 1 and 2 interferes with daily function

4. Does not occur exclusively during delirium

EPIDEMIOLOGY

~ 5 to 8 % at age 65 to 70

~ 15 to 20 % at age 75 to 80

up to 40 to 50 % over age 85

• Alzheimer's disease is most common dementia 50-75%

• Dementia with Lewy bodies 15 to 35 %

• Vascular dementia 5 – 20 %

ETIOLOGYNEURO-DEGENERATIVE

Alzheimer's Ds; Dementia with Lewy Bodies; Fronto-temporal dementia; Parkinson’s Ds

VASCULAR Infarction; Hemodynamic insufficiency

NEUROLOGICAL Multiple Sclerosis; Normal Pressure Hydrocephalus

ENDOCRINE Hypothyroidism

NUTRITIONAL Def. of Vit. B12, Thiamine, Niacin

INFECTIOUS HIV; Prion Ds; Neurosyphilis; Cryptococcus

METABOLIC Hepatic/ Renal Insufficiency; Wilson’s Ds

TRAUMATIC Subdural Haematoma; Dementia pugilistica

TOXIC AGENTS Alcohol; Heavy Metals; Anticholinergic Med; CO

CORTICAL VS. SUBCORTICAL DEMENTIA

Cortical Symptoms: major changes in memory, language deficits,

perceptual deficits, praxis disturbances,lack of extrapyramidal

features

Affected brain regions: temporal cortex (medial), parietal cortex, and frontal lobe cortex

Examples: Alzheimer’s disease, diffuse Lewy body disease, vascular dementia, frontotemporal dementias

Subcortical Symptoms: behavioral changes, impaired affect and mood,

motor slowing, executive dysfunction, less severe changes in memory, extra pyramidal findings

Affected brain regions: thalamus, striatum, midbrain, striatofrontal projections

Examples: Parkinson’s disease, progressive supranuclear palsy, normal pressure hydrocephalus, Huntington’s disease, Creutzfeldt-Jakob disease, chronic meningitis

CORTICAL VS. SUBCORTICAL DEMENTIA

MIXED

Both cortical and sub-cortical area involved.

Example: vascular dementia, Dementia with Lewy bodies, Corticobasal degeneration, Neurosyphilis

D = Delirium E = Emotions (depression)& Endocrine Disease M= Metabolic Disturbances E = Eye & Ear Impairments N = Nutritional Disorders T = Tumors, Toxicity, Trauma to Head I = Infectious Disorders A= Alcohol, Arteriosclerosis

REVERSIBLE DEMENTIA

Alzheimer’s Lewy Body Dementia Pick’s Disease (Frontotemperal Dementia) Parkinson’s Vascular Huntington’s Disease Jacob-Cruzefeldt Disease

IRREVERSIBLE DEMENTIA

HOW TO DIAGNOSE A CASE OF DEMENTIA IN NEUROLOGY OPD

Clinical history

Symptoms analysis

Focussed physical examination

Cognitive and neuropsychiatric examination

Laboratory evaluation

CLINICAL SYMPTOMS COGNITIVE IMPAIRMENT

FUNCTIONAL IMPAIRMENT

NEURO-PSYCHIATRIC MANIFESTATIONS

BEHAVIOURAL DISTURBANCES

MOOD CHANGES

ANXIETY

PERSONALITY CHANGES

PSYCHOSIS

SLEEP DISTURBANCES

FOCUSED HISTORY

Chronology of the problem- from loved ones - mode of onset – abrupt vs gradual - progression - stepwise vs continous

decline - duration of symptoms Medical history Family history Socio-economic history Evaluation for toxic agent exposure

PHYSICAL EXAMINATION

Neurological examination-mobility and balance assessment

Focal neurological deficits Extra-pyramidal signs Vision & hearing screening Cardiac and pulmonary evaluation

COGNITIVE & NEUROPSYCHIATRIC EXAMINATION

Folstein Mini-Mental Status Examination (MMSE)

MMSE

SCORE RANGE

24-30 Normal18-23 Mild10-17 Moderate< 10 Severe

INVESTIGATIONS

ASSESSMENTS RATIONALE

Labs: Complete blood count, serum electrolytes, renal and hepatic function, glucose, albumin and protein, vitamin B12 and folate, rapid plasma reagin (syphilis), thyroid- stimulating hormone, urinalysis

Rule out correctable or contributory causes of dementia

Imaging: Computed tomography without contrast or magnetic resonance imaging

Rule out infarcts, mass lesions, tumors, and hydrocephalus

Neurological examination Correlate imaging findings with clinical examination

Neuropsychological testing Mini-Mental State Examination: Screening test of cognitive function

DIFFERENTIAL DIAGNOSIS DELIRIUM

MILD COGNITIVE IMPAIRMENT (MCI)

AGE-RELATED COGNITIVE DECLINE

MENTAL RETARDATION

SCHIZOPHRENIA

DEPRESSION

FACTITIOUS D/O

ALCOHOL ABUSE

DIAGNOSTIC APPROACHCOGNITIVE IMPAIRMENT ?

DETERIORATION FROM APREVIOUSLY HIGHER LEVEL ?

CONSCIOUSNESS ALTERED?

MULTIPLE COGNITIVE FUNCTIONSINVOLVED ?

DEMENTIA

YES

YES

YES

NO

NO

YES

NO

MENTALRETARDATION

DELIRIUM

APHASIAAMNESTIC D/O, etc

DEMENTIA –SUB TYPES

CASE 1

70 yr old female present with progressive memory loss for past 1 yr.She also complaints of difficulty in naming objects and driving car and house keeping.for the past 1 month she has difficulty in dressing ,eating and gets agitated easily and wanders around at night.

MMSE – 15/30 Neurological exam- normal Vision & hearing- normal

MRI FINDINGS- DIAGNOSIS?

ALZHEIMER’S DISEASE (AD)

About 70% of all cases of dementia in elderly Incidence increases with age Occurs in up to 30% of persons >85 years old Characterized by:

Progressive loss of cortical neurons Formation of amyloid plaques (beta-amyloid is major component)

and intraneuronal neurofibrillary tangles (hyperphosphorylated tau proteins is major constituent)

DIAGNOSTIC CRITERIA FOR DEMENTIA OF THE ALZHEIMER TYPE(DSM-IV, APA, 1994)

A. Development of multiple cognitive deficits 1. Memory impairment

2, other cognitive impairment B. These impairments cause dysfunction in

In social or occupational activities C. Course shows gradual onset and decline D. Deficits are not due to: 1. Other cns conditions

2. Substance induced conditions F. Do not occur exclusively during delirium G. Are not due to other psychiatric disorder

CLINICAL MANIFESTATION Begin with memory impairment language

visuospatial skills

Anosognosia- unaware of difficulties Cognitive decline-driving,shopping,house-keeping Language impaired- naming,comprehension then - fluency Apraxia- seq. motor task can’t perform

Visuo spatial deficits Delusions ,capgras syndrome – late stages End stage-rigid,mute ,incontinent & bed-ridden

AD DIAGNOSIS

Neurological exam & neuropsychological testing Brain imaging: brain atrophy due to extensive

neuronal loss and hippocampal atrophy Diagnosis confirmed by histology of post-mortem

brain ‘Plaques’ & ‘tangles’ in hippocampus & cerebral

cortex.

CASE 2

76 yr old male presented in neuro opd with c/o progressive memory loss,emotional lability,gait disturbance for past 5 months

h/o of 3 episodes of cerebrovascular accidents +

recent attack 7 months back h/o HTN,DM,CAD+ O/E- incresed tone in all limbs,power 3+ in

RT.UL &LL. 4+ in LT side, B/L extensor plantar

VASCULAR DEMENTIA

Refers to cognitive decline caused by ischemic, hemorrhagic, or oligemic injury to the brain as a consequence of cerebrovascular or cardiovascular disease.

Part of a spectrum of vascular disease causing cognitive impairment, which also includes mild cognitive impairment of vascular origin & mixed Alzheimer's disease plus cerebrovascular disease.

Kraepelin first described “arteriosclerotic dementia” in 1896

DIAGNOSIS

&

CLINICAL

FEATURES

(NINDS-AIREN)

VASCULAR DEMENTIA

Multi-infarct dementia- recurrent strokes - step wise progression - HTN,DM,CAD MRI- multiple areas of infarction Binswanger’s d/s– Diffuse white matter disease - lacunar infarction C/F:confusion,personality changes,psychosis pyramidal signs & cerebellar signs + gait disorder,urinary

incontinence,dysarthria emotional lability

CASE 3

55 YR old woman presented with 2yr history of progressive alteration in social behavior. The pt had h/o social disinhibation,abusive language,euphoria.there is complaints of excessive food intake and weight gain for past 1 yr and pt was taken to psychiatrist once.

o/e- vitals stable..neurological exam –WNL MMSE-18/30

MRI

FRONTOTEMPORAL DEMENTIAS

Often begins with marked behavioral disturbances, unlike AD

Classic form – Pick’s disease Patients frequently hot-tempered and socially

disinhibited memory & visuo spatial skills spared Impaired planning,judgement and language Echolalia + Overlap with PSP,CBD, motor neuron disease Illness progresses for years, like AD Inevitable decline MRI- lobar atrophy of frontal and/or temporal About 50% of patients have family history

CASE 4

82 yr old male came to opd with c/o progressive decline in memory for the past 6-8 months.He also complaints of having decreased sleep and occasional nightmares.He occasional sees his deceased wife at times.

o/e- vitals stable ,rigidity of limbs+ - gait- slow stepping gait,bradykinesia+

MMSE- 21/30

WHAT IS THE DIAGNOSIS?

DIFFUSE LEWY BODY DISEASE

Patients have clinical parkinsonism with early and prominent dementia

Lewy bodies found in brain stem, limbic system, and cortex

Visual hallucinations and cognitive fluctuations common, capgras syndrome & REM sleep disorder

Longstanding PD without cognitive decline develop dementia

Better memory but severe visuospatial deficit Patients sensitive to adverse effects of neuroleptics May be second most common cause of dementia

after AD

PARKINSON’S DISEASE About 50% of patients have dementia by 85 years old Affects executive function disproportionately Dementia occur in later stage, or as a result of co

morbidities- AD,DLB or side effects of drug Associated depression & anxiety Frontal lobe dysfnct- complex tasks,planning,

-memorizing Language & mathematical skills spared Predictors- late onset,akinetic-rigid,severe depression

- advanced stage

CASE 5

65 YR old male presented to neuro opd with c/o gait disturbance for past 1 yr. On history taking his son complained his father is having memory loss for past 6 months and it is progressing.

The pt also c/o of urinary incontinence+ Neurolog exam- no focal deficits MMSE- 23/30

MRI- DIAGNOSIS ?

NORMAL PRESSURE HYDROCEPHALUS

Triad

1. Dementia: typically subcortical

2. Gait instability

3. Urinary incontinence Walk with “feet stuck to floor” Symptoms progress over weeks to months CT shows ventricular enlargement with no

eviddence of cortical atrophy

Most important test – therapeutic LP1. Remove large amount of CSF

2. Examine gait and cognitive function

Ventriculoperitoneal shunt may correct if: Patients improve within minutes to hours of removal of 30

to 40 mL of spinal fluid Trauma or subarachnoid hemorrhage

Cause is derangement of CSF hydrodynamics

NORMAL PRESSURE HYDROCEPHALUS

CASE 6

50 YR old woman was admitted with c/o progressive memory loss and gait problem ,slurred speech within one month; The pt also had behavioral problem – insomnia,agitation,aggression duration of 3 weeks.the pt also c/o abnormal jerky hand movements for past 1 month

o/e- limb & gait ataxia +, reflexes-exagg.

- tone increased all limbs, plantar b/l extensor

- no focal weakness

MMSE- 16/30

MRI- DIAGNOSIS?

CRUETZFELDT-JAKOB SYNDROME(CJD)

Rapid progressive dementing prion disorder Focal cortical signs, rigidity Onset between 40- 75 years 90% has MYOCLONUS vs 10% in AD Progressive dementia and personality

changes over weeks to months Death <1 year from first symptom EEG- diffuse slowing and periodic sharp

waves or spikes MRI- basal gangla abnormalities CSF- detect specific aminoacid sequence

(PrPSc)

Aging Mild loss of memory: names and dates Most sensitive indicator of cognitive change:

poor performance on delayed-recall tasks Verbal fluency remain intact and vocabulary

may increase

DISORDERS OF MEMORY FUNCTION (AMNESTIC DISORDERS)

Transient global amnesia Dramatic memory disturbance Affects patients >50 years Usually have only one episode, lasting 6 to 12 hrs. Complete temporal and spatial disorientation Orientation for person preserved May be confused with psychogenic amnesia, fugue state, or partial

complex status epilepticus May be due to vascular insufficiency to hippocampus or midline

thalamic projections

DISORDERS OF MEMORY FUNCTION

DISORDERS OF MEMORY FUNCTION

Head injury Retrograde amnesia > antegrade amnesia With time, memories usually return but rarely to recall events

surrounding trauma Korsakoff’s syndrome

Near-total inability to establish new memory Patients confabulate about recent events Immediate memory NL,attention NL Most common cause: thiamine and other nutritional

deficiencies with chronic alcoholism

PSEUDO DEMENTIA

Severe depression Memory & language intact Vegetative symptoms –insomnia,anergy, - loss of appetite Abrupt onset

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