Case 1
41 years lady, a diagnosed case of linear scleroderma/morphea involving her right upper limb followed by lower limb and face and treated initially with topical steroid followed by short course of oral steroid and Chloroquine since the age of 8 years, which subsequently stabilized even without medication. She developed recurrent GTCS since the age of 13 years, well responded to anti-epileptic medications, namely, Phenytoin sodium, Gardenal etc. However, on attempted withdrawal of anti-epileptic medications, she had recurrence of seizures for several times. She developed history suggestive of right homonymous hemianopia interfering her day-to-day activities since last 6 months, being further progressed over last 3 months.

Her earlier EEG was normal. MRI of brain revealed left hemiatrophy with gross dilatation of occipital horn of lateral ventricle. Automated Perimetry confirmed the diagnosis of homonymous hemianopia. There are several reports regarding temporal relation between linear scleroderma with seizure disorder and Parry Romberg Syndrome. This case represents one spectrum of such disorder

CASE 2
50 years old gentleman, without any known medical illness was admitted with complaints of acute onset abnormal movements of left upper limb since 3 days prior to admission. No history of headache, vomiting, confusional state, motor weakness, gait disturbances etc. Clinical examination revealed stable vitals. BP – 120/70 mm Hg, well oriented with normal speech and cranial nerve functions. Motor system examination revealed normal power with prominent Choreoathetotic movements, predominantly involving left upper limb. His cranial imaging including MRI brain was all normal. His random serum blood glucose level examination was quite high (540 mg/dl) but urine ketone came out to be negative.

He was treated with IV fluids, Insulin, oral haloperidol, Clonezepam etc. He had rapid symptomatic improvement with correction of blood glucose and Choreoathetotic movement subsided completely within 2-3 days of hospital stay.

Take home message: Acute choreoathetosis in the previously healthy person with normal neuro-imaging merits exclusion of treatable disorder like non-ketotic hyperosmolar state or recent onset diabetes with very high blood sugar level.