A Case of Hemichorea

PHYSICIANS MEETPHYSICIANS MEET

Prof T.V.K’S UNIT

Dr.D.Premkumar

HISTORYHISTORY

A 85yrs old lady admitted with complaints of spontaneous movements of left upper and left lower limb- one weeks

Involuntary movements- one week,sudden in onset,involving left upper and left lower limb,fast flinging movement, increased during emotions, decreased during rest.

No H/O--- Loss of consciousness, headache, vomiting, visual disturbance, weakness, seizures,cranial nerve,sensory involvement, bladder bowel disturbance,speech disturbance.

No H/O--- Fever, joint pain, skin lesions No H/O---Chest pain,palpitation, syncope,

dyspnoea No H/O---Vomiting, loose stools, abdominal pain,

abdominal distension

No H/O trauma, TIA,RIND. PAST HISTORY: History of previous similar

episodes present- one month back. No H/O chronic fever, Rheumatic fever, dog

bite,vaccination, chronic drug intake. Not-DM,SHT, CAD, BA, PT. PERSONAL HISTORY: Not a smoker,

alcoholic, tobacco chewing, No similar episodes in the family.

EXAMINATIONEXAMINATION

GENERAL EXAMINATION : Patient conscious,co-operative afebrile moderately built& nourished no pallor, not icteric no-cyanosis, clubbing no-pedal edema, lymphadenopathy No Neurocutaneous Markers

VITALS

pulse rate-84/min

Blood pressure-130/80

respiratory rate-24/min

SYSTEMIC EXAMINATIONSYSTEMIC EXAMINATION

CENTRAL NERVOUS SYSTEMHigher motor function: NormalCranial nerves examination:NormalMotor system: bulk-normal tone- slightly reduced power-4+/5 reflexes- normal, Plantar- b/l flexor co-ordination-normal

Involuntary movements- non-rhythmic,rapid,flinging type of movement is present in the left upper and lower limb,which is aggravated during activity& emotion and decreased or absent during rest and sleep.

Sensory system- intact

Cerebellum- intact

Autonomic nervous system-no bladder bowel disturbance

Fundus – normal

Other systems- CVS, RS, ABDOMEN- Normal

investigationsinvestigations

Hb %-9.7gm% Total count-8200 DC:P68,L30,E2 ESR-5/10 Platelet count-2.5lakhs MCV-88.1 MCH-29.0 MCHC-32.9 PCV-29.2

RBS-122mg% Urea-38 Creatinine-1.1 S.Electrolytes:Na-144,K+-4.5,HCO3-22, CI-98 Urine Examination:alb-nil, sugar-nil, deposits-2-3

pus cells HPF ECG-NSR CXR-NAD

CT BRAIN- normalMRI BRAIN- Degenerative changes

in the CAUDATE NUCLEUS and PUTAMEN.

MRI BRAINMRI BRAIN

MRI BRAINMRI BRAIN

MRI BRAINMRI BRAIN

OPINIONSOPINIONS

Neurologist opinion: HEMICHOREA

Cardiologist opinion:mild AV sclerosis, no

RWMA, normal LV function

DIAGNOSISDIAGNOSIS

This is a case of involuntary movement affecting the left side of the body without signs of cerebrovascular disease, rheumatic fever- HEMICHOREA (Senile Chorea)

Treatment : Haloperidol 1mg BD Trihexyphenidyl

HYPERKINETIC MOVEMENT HYPERKINETIC MOVEMENT DISORDERSDISORDERS

CHOREA/HEMICHOREAHEMIBALLISMUSATHETOSISCHOREO-ATHETOSISDYSTONIASMYOCLONUSFASCICULATIONS

CHOREACHOREA

Semi-purposive, flowing,irregular, non-repatitive and brief,jerky movement arising in the proximal joints and apper to flit from one part of the body to another randomly.

Absent during sleep and increased on attempting voluntary movement.

Due to lesion in CAUDATE NUCLEUS.

Causes of choreaCauses of chorea

Hereditary: 1.Huntington’s disease 2.Neuroacanthocytosis 3.Benign hereditary/senile chorea 4.Wilson’s disease 5.Hallervorden-Spatz syndrome

Post-infectious/autoimmune: 1.syndenham’s chorea

2.SLE 3.AIDS

Endocrinology

1.hyperthyroidism

2.chorea gravidarum

Drug induced Chorea

1.OCP

2.Levodopa

3. Anticonvulsants

4.neuroleptics

5.Antidepreesants

Vascular

1.Strokes

2.Polycythemia vera

Others

Neoplastic/metabolic

Signs elicitale in choreaSigns elicitale in chorea

‘Jack in the box’tongueRespiratory irregularityMilk maid signHung up reflexPronator sign

studystudy

Piccolo et al studied 51 cases of sporadic chorea. On follow up only 20 cases had chorea at the end of six months.

Analysis revealed1.vascular related-21 cases

2.vasculitis-2 cases3.hypoxia-2 cases4.drug induced- 7 cases

5.AIDS-related-5 cases

6.Borreliosis-1 case7.Syndenham’s Chorea-1 case

8.Hyperglycemia-2 cases9.Hyponatremia-2

cases 10.Huntington’s chorea-5 cases11.Neuroacanthocytosis-1 case

12.No causes found-3 cases

Areas involvedAreas involved

Chorea- caudate nucleusAthetosis-putamenHemiballismus-subthalamic nucleusDystonias-putamen

HemiballismusHemiballismus

Usually affects proximal joint of one arm resulting in wild,rapid,flinging movement of wide radius,occuring constantly, interspersed with short period of freedom.

Absent during sleepIncreased tone,reflexesSub thalamic nucleus

AthetosisAthetosis

Slow writhing movement,best seen at wrist, fingers and ankle.

Lesion in putamenAbsent during sleep

DystoniasDystonias

Idiopathic/primary torsion dystonias is a disorder charecterised by involuntary sustained muscle contractions frequently causing twisting and repetitive movements or abnormal posture

Types-generalized, segmental,focal

Common causes of secondary dystonias1.drugs-phenothiazine

2.Wilsons disease3.Degenerative

ataxias 4.kernicterus5.sickle

cell disease6.stroke,tumor,trauma

MyoclonusMyoclonus

Consists ofrapid,brief shock like muscle jerks which are often repetitive, sometimes rhythmical

Normal phenomenon or may be a manifestation of major seizures

FasciculationsFasciculations

Irregular non-rhythmical contraction of muscle fascicles.

Best seen in large muscles(deltoid/calf muscle) Present at rest and may be increased after

voluntary movement Sign of LMN lesion Precipitated – contracting the musce,

hyperventilation, cooling the muscle

Causes of fasciculation1.MND

2.Syringomyelia3.Cervical

spondylosis 4.PMA 5.Thyrotoxic myopathy 6.carcinomatous myopathy 7.OPC poisoning

Presence of fasciculation excludes myopathy except thyrotoxic and carcinomatous myopathies

TREATMENTTREATMENT

CHOREAHuntington’s- no specific, phenothiazines or

haloperidol, reserpineSyndenham’s chorea- Dopamine receptor blockers

and anti-convulsants(valproic acid,carbamezapine)

Wilson’s disease- D-Penicillamine & trientin&zinc

HEMIBALLISMUS: haloperdol,clonazepam,baclofen

Dystonias :anticholinergics,BDZ,Baclofenbotulinum toxin

Myoclonus : valproic acid, clonazepam, oxitriptan