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MALIGNANT NEUROLEPTIC SYNDROME

A rare condition associated with prescribing dopamine antagonist and long-acting depot neuroleptic preparations. Drowsiness, fever, tremor and rigidity occur suddenly. Muscle necrosis (rhabdomyolysis) results in myoglobinuria and occasionally renal failure. Early identification and treatment with dopamine receptor agonists (bromocriptine) and muscle relaxants (sodium dantrolene) may be life saving.

WILSON'S DISEASE (hepatolenticular degeneration) A rare autosomal recessive disorder of copper metabolism in which extrapyramidal features arc evident. The gene abnormality has been located to chromosome 13. Pathology

Cavitation and neuronal loss occurs within the putamen and the globus pallidus.

The liver shows the appearance of coarse cirrhosis. Copper accumulates in all organs, especially in Descemet's membrane in the eye, nail beds and kidney.

Liver

Globus pallidus

Putamen

Lentiform nucleus

Biochemistry

There is deficiency of n2 globulin - Ceruloplasmin - which normally binds 98% of copper in the plasma. This results in an increase in loosely bound copper/albumin, and deposition occurs in all organs. Urinary copper is increased.

Clinical features

There are two clinical forms: 1. Acute (Children) Bradykinesia Behavioural change Involuntary movements Liver involvement common

Untreated: death in 2 years from hepatic and renal failure

2. Chronic (Young adults)

Marked proximal 'wing beating' tremor

Dysarthria, dystonia and rigidity

Choreoathetoid movements

Psychosis, behavioural disorders and dementia

Liver involvement less severe

Untreated: death in 10 years

The deposition of copper in Descemet's membrane produces the golden brown Kayser-Fleischer ring, which when seen by naked eye or slit-lamp is diagnostic. V

Diagnosis

Clinical findings supported by biochemical evidence:

- Low ceruloplasmin (less than 20 mg/'dl)

- Elevated unbound serum copper

- High urinary copper excretion

- Liver biopsy and copper metabolism tests with radioactive MCu.

- MR I (T2) shows thalamic and putaminal hyperintensity.

In families, biochemical tests will identify low ceruloplasmin in carriers and in presymptomatic patients. These relatives require appropriate genetic counselling and treatment when indicated.

Treatment

Low copper diet and a chelating agent, e.g. penicillamine 1-1.5g daily. Side effects such as anaphylaxis, skin rash, bone marrow suppression and glomerulonephritis are common in which case trientinc is an effective alternative.

Therapy is necessary for the rest of the patient's life. Adequate treatment is compatible with normal life expectancy. Kayser-Fleischer rings will disappear with time.

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