A 14-year old girl with Wilson´s disease and another her 8 year old brother with asym, omatic Wilson´s disease were treated with potassium sulfide, penicillamine and british antilewisite (BAL) for 3 months. Clinically they are excellent, the girl patient´s neurological manifestations have discppeared and both patient´s Kayser-Fleischer rings have almost faded.
Case 1 In December 1969, a 14 year old girl complained of disturbances of speech, swallowing, writing and walking of four months duration which appeared 6 months after an´ episode of hepatitis. On examination she was rather emaciated. but anicteric. There were neither exanthoma, no cutaneous stigmata of chronic liver, disease. The liver and spleen. could not be felt, and fluid retent on was absent Neurologic Examination:- She was dysphonic and could not form distinct words. She w. alert, coherent, cooperative. and showed natural emotion. There was occasional facial grimacing, ith the mouth held open.´ Drooling was noticed. ´Chewing was unorganized and impaired by spasm of e jaw. The tonguee protruded, ´and showed some fibrillation. Gait was slowed, ,and after some inertia, showed a shuffling pattern. On turning, she made quick rotation steps. There were occasional tremulous movements of the right upper extremity. There were no ataxia, nor nystagmus. The pupils were equal, and fundi were normal. A dark brown ring was seen at the corneal function, which was confirmed by slit lamp examinatio ~ . The upper extremities revealed marked spasticity on passive motion during flexion and ´extension. There was no cogwheel rigidity and no atrophy. The fingers as well showed slight spasticity:
The reflexes were normal. The lower extremities also revealed moderate spasticity oto passive motion at the knees and hips. There was no, clonus, and Babinski was negative. Investigations. Hgb 13.3 gm o. RetL count Hct 41%. W. B. C. 7,450. Total serum protein 5.7 gm °o. Alhumin 3.8 gm%, globulin 1.9 gm%, A/G 2 1, Serum aspartate transaminase´ (SGPT)- 6. units. Serum oxaloacetic transaminase (SGOT) 8/unit. Lactic acid dehydrogenase 360 unit. phosphorus 4. mg%. prothrombin time 15"(14") BSP 3%. Serum copper content 40 micrograms/100 ml (norm ´ range 100-´200 micrograms. Ceruloplasmin 0 milligrams/100 ml (normal range 27´´37 mg). Urine pH 6 protein negative urobilinogen positive suger negative 24 hour urinary copper content 1 mcg/24 hours Spinal fluid: cells 15/3: "protein -50mg%,, suger 40mg%. pandy reaction ±, Nonne reaction-.
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