Tremors and spasticity

Case Report:- A 1½ year old boy born of non consanguineous marriage presented with fever 15 days ago which subsided after 5 days with oral medications and reappeared again after 2 days. He also had involuntary repetitive clonic movements of both upper limbs without uprolling of eyes or unconsciousness. These clonic movements used to persist in sleep also. He was a full term normal delivery and was kept in NICU for 12 days for meconium aspiration. Milestones are normal. He is immunized till date. On examination, he had severe hypertonia (which persisted even in sleep). Reflexes were normal. Other systems were normal. He had persistent tremors. Other systems were normal.

Investigations showed:

·          MRI brain = Normal

·          CSF = Normal

·          Hemoglobin = 9.7 gm/dl

·          WBC = 8,900/cumm [87% polymorphs, 13% lymphocytes]

·          Platelets = 3,13,000/cumm

·          SGPT = 24 IU/L, RFT & electrolytes = Normal

·          VBG = No acidosis

·          Serum ammonia = 243 µg/dl

·          Leptospirosis Rapid test = Negative

·          ASLO = Negative

·          Vitamin B12 = 733.2 pg/ml (Normal = 200 to 950 pg/ml)

·          Anti mycoplasma IgM = Negative

·          CPK = 585 IU/L (Normal = 57-180)

·          Plasma methionine, cystine, homocysteine = Normal

·          Anti basal ganglion antibody = Negative

·          Ophthalmology – Fundus = Normal

·          Peripheral smear – Acanthocytes seen

·          EMG/NCV = Normal

He was treated with Pacitane, L-Dopa and Vitamin B12 but hypertonia persisted through tremors subsided. He recovered on his own after one month but then again redeveloped spasticity and tremors in next week.