Cerebellar signs

Bichile LS, Kuloor PL, Hegde AV, et al. 1983. Acute reversible cerebellar signs after Diazinon poisoning [letter], J Assoc Physicians India 31(ll) 745-746. 

This disease develops when an abnormal prion protein present in the cadaveric material induces a cascade of conformational changes in host protein. Creutzfeldt-Jakob disease in recipients of somatropin differs from the sporadic form, in that it usually presents with cerebellar signs rather than cognitive impairment, and also in the prominence of prion protein amyloid plaques in nervous tissue (18). In a review, 139 cases of Creutzfeldt-Jakob disease were identified worldwide in people treated with cadaveric somatropin before recombinant human growth hormone became available in the mid-1980s (19). The prevalence of this fatal neurodegenerative condition in recipients of somatropin ranges from 0.3% in the USA to 4.4% in France. Creutzfeldt-Jakob disease has been reported to start at 4-30 years after therapy with cadaveric somatropin (18), so that further cases are anticipated and continue to be reported (20). 

Adverse Side Effects. Gastric irritation, confusion, sedation, dizziness, headache, cerebellar signs (nystagmus, ataxia, dysarthria), gingival hyperplasia, increased body and facial hair (hirsutism), and skin disorders are typical adverse effects. 

In two reports from China 10 cases (nine men and one woman) of heroin-induced spongiform leukoencephalopathy have been reviewed. The first report discussed six cases with cerebellar signs and symptoms, with symmetrical lesions on neuroimaging in the white matter of the cerebellum, basal ganglia, posterior crus of the internal capsule, and the semi-oval center (25). The second report, described four cases that occurred during the abstinence period and showed improvement after 4 weeks of comprehensive treatment (26). 

Nicolaides KH, Campbell S, Gabbe SG, Guidetti R. Ultrasound screening for spina bifida cranial and cerebellar signs. Lancet 1986 2 8498) 72-4. 

Neurological Tremor, myoclonus, chorea, spasticity, ataxia, convulsions, neuropathy, exaggerated tendon reflexes, headache, vertigo, incoordination, cerebellar signs, delirium, seizures, coma, psychiatric symptoms (see Table 8—4)... 

Neurological Headache, pain—generalized and dental, visual disturbance, vertigo or syncope, tinnitus, deafness, cerebellar signs, ataxia, gaze-evoked nystagmus, paresthesias, electroencephalogram abnomalities— diffuse and focused, seizures, paralysis, papilledema, death... 

Treatment has been attempted with y-vinyl GABA (vigabatrin, Sabril ), an inhibitor of GABA transaminase, in order to reduce substrate accumulation [6]. This resulted in a decrease of CSF y-hydroxybutyric acid levels and improvement of cerebellar signs in a majority of patients. 

When there is a suspicion of SSD deficiency, a trial with vigabatrin (Sabril) 50-100 mg/kg/day (divided in two daily doses) is indicated. In most patients this treatment improves cerebellar signs. 

Refsum s syndrome starts in late childhood and beyond. Generalized, branny dirty scales are present in some cases. Retinitis pigmentosa, progressive polyneuritis and cerebellar signs are the cardinal features of this autosomal recessive disorder. Phytanic acid is present in serum and deposits of lipids are found in all tissues. 

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