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Dyssynergia cerebellaris progressiva (Ramsay Hunt syndrome) - case report (CROSBI ID 516867)

Prilog sa skupa u zborniku | sažetak izlaganja sa skupa | domaća recenzija

Šepić-Grahovac, Dubravka ; Sepčić, Juraj ; Rudež, Josip ; Pancić, Branko Dyssynergia cerebellaris progressiva (Ramsay Hunt syndrome) - case report // Abstracts 3rd Croatian Symposium on Epilepsy (with international participants) / Škarpa, D. (ur.). Zagreb: Hrvatsko neurološko društvo, 1998. str. 61-62-x

Podaci o odgovornosti

Šepić-Grahovac, Dubravka ; Sepčić, Juraj ; Rudež, Josip ; Pancić, Branko

engleski

Dyssynergia cerebellaris progressiva (Ramsay Hunt syndrome) - case report

Presentation is made of a male patient with generalized myoclonus, dysarthria, pronounced cerebral ataxia, polyneuropathy, and rare primary generalized tonic-clonic epileptic seizures. The patient had excavated feet. He occasionally exhibited hallucinatory-paranoid psychotic episodes with suicidal attempts. EEG showed diffuse paroxysmal abnormalities with multiple spikes and spike-wave complexes. Computed tomography of the brain showed cerebellar atrophy. Visual evoked brain potentials were extremely prolonged. Muscle biopsy showed average values of respiratory chain enzymes. Testing for possible sialidosis was negative. Cerebrospinal fluid analysis produced normal finding. Brain biopsy showed marked diffuse astrocyte type gliosis of the white and gray matter, with a varying stage degeneration of ganglial cells. Polytherapy with valproate, clonazepam and barbiturates was only partially efficient for tonic-clonic seizures, and minimally for myoclonus. The disease assumed a progressive course and resulted in lethal outcome after a 30-year duration. Differential diagnosis of the syndrome was quite complex. We were inclined to make the diagnosis of Ramsay-Hunt syndrome, because the myoclonus was primarily of the action type, prevailing over ataxia and very rare grand mal epileptic seizures. In spite of the longterm disease, the patient's mind remained preserved.

Dyssynergia cerebellaris progressiva

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nije evidentirano

nije evidentirano

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nije evidentirano

Podaci o prilogu

61-62-x.

1998.

nije evidentirano

objavljeno

Podaci o matičnoj publikaciji

Abstracts 3rd Croatian Symposium on Epilepsy (with international participants)

Škarpa, D.

Zagreb: Hrvatsko neurološko društvo

Podaci o skupu

3rd Croatian Symposium on Epilepsy (with international participants)

predavanje

01.10.1998-03.10.1998

Hvar, Hrvatska; Stari Grad, Hrvatska

Povezanost rada

Kliničke medicinske znanosti