Wilsons Disease Information

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Wilsons Disease Information - symptom, cause, picture, treatment of Wilsons Disease

Wilsons Disease Information

Occasional myoclonic jerks may occur in anyone, especially when drifting into sleep. General or multifocal myoclonus is common in patients with idiopathic epilepsy and is especially prominent in certain hereditary disorders characterized by seizures and progressive intellectual decline, such as the lipid storage diseases. It is also a feature of various rare degenerative disorders, notably Ramsay Hunt syndrome, and is common in subacute sclerosing panencephalitis and Creutzfeldt-Jakob disease. Generalized myoclonic jerking may accompany uremic and other metabolic encephalopathies, result from levodopa therapy, occur in alcohol or drug withdrawal states, or follow anoxic brain damage. It also occurs on a hereditary or sporadic basis as an isolated phenomenon in otherwise healthy subjects.

Segmental myoclonus is a rare manifestation of a focal spinal cord lesion. It may also be the clinical expression of epilepsia partialis continua, a disorder in which a repetitive focal epileptic discharge arises in the contralateral sensorimotor cortex, sometimes from an underlying structural lesion. An electroencephalogram is often helpful in clarifying the epileptic nature of the disorder, and CT or MRI scan may reveal the causal lesion.

Wilsons Disease may respond to certain anticonvulsant drugs, especially valproic acid, or to one of the benzodiazepines, particularly clonazepam (Table 24–3). It may also respond to piracetam (up to 16.8 g daily). Wilsons Disease following anoxic brain damage is often responsive to oxitriptan (5-hydroxytryptophan), an investigational agent that is the precursor of serotonin, and sometimes to clonazepam. Oxitriptan is given in gradually increasing doses up to 1–1.5 mg daily. In patients with segmental myoclonus, a localized lesion should be searched for and treated appropriately.

 

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