Images in Neurology

White matter changes on magnetic resonance imaging in a patient with neurodegenerative disease Manjula C. Caldera, Inuka Kishara Gooneratne, Ranjanie Gamage, Sujith Priyankara Perera Institute of Neurology, National Hospital Sri Lanka, Colombo, Sri Lanka For correspondence: Dr. Inuka Kishara Gooneratne, 10/1, Borella Cross Road, Colombo 8, Sri Lanka. E‑mail: [email protected] Ann Indian Acad Neurol 2013;16:670‑1

A 14‑year‑old Sri Lankan male had progressive dysarthria, dysmetria, gait ataxia, tremor, bradykinesia for 6 years with cognitive decline ensuing 1 year prior to admission. His sister was suffering from a similar illness. His parents were non‑consanguineous. On examination, the patient had a Glasgow coma score of 8. He had quadrihyperreflexia with extensor plantar response. Cerebellar assessment was difficult. Cogwheel rigidity was noted. There was no flapping tremor. Hepatosplenomegaly was elicited on abdominal examination. Kayser‑Fleischer (KF) rings were confirmed by slit lamp examination. Magnetic resonance imaging  (MRI) showed increased signal intensity on T2‑weighted image in basal ganglia and supratentorial with infratentorial gray and white matter [Figure 1]. Biochemical analysis is as follows: Serum ceruloplasmin level ‑ 1 mg/dl (18‑45), urinary copper excretion ‑ 4.5  µmol/24 h, Aspartate transaminase (AST) (SGOT) ‑  48 U/l  (10‑35), Alanine transaminase (ALT) (SGPT) ‑ 57 U/l (10‑40), alkaline phosphatase ‑ 226 U/l (100‑360), serum bilirubin ‑ 9.2 µmol/l (5‑21), serum protein ‑  70  g/l, albumin ‑  39  g/l, globulin ‑  31  g/l, prothrombin time ‑ 12.8 s International normalized ratio (INR 1.3), APTT ‑ 20 s, serum ammonia ‑ 25 µmol/l (

White matter changes on magnetic resonance imaging in a patient with neurodegenerative disease.

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