Naslov
Dyssynergia cerebellaris progressiva (Ramsay Hunt syndrome) - case report

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

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
Abstracts 3rd Croatian Symposium on Epilepsy (with international participants) / Škarpa, D. - Zagreb : Hrvatsko neurološko društvo, 1998, 61-62

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

Mjesto i datum
Hvar, Hrvatska; Stari Grad, Hrvatska, 01.10.1998. - 03.10.1998

Vrsta sudjelovanja
Predavanje

Vrsta recenzije
Domaća recenzija

Ključne riječi
Dyssynergia cerebellaris progressiva

Sažetak
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.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

Napomena
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