Naslov
Lance-Adams syndrome in a patient with congenital coagulation factor XIII deficiency and spontaneous atypical subdural hematoma: case report

Autori
Hajnšek, Sanja ; Petelin, Željka ; Malojčić, Branko ; Mijatović, Dragana ; Poljaković, Zdravka ; Bašić, Silvio ; Pavliša, Goran ; Milat, Davorka

Izvornik
Neurologia Croatica (0353-8842) 59 (2010), 1-2; 63-66

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
Lance-Adams syndrome; post-hypoxic myoclonus; coagulation factor XIII; spontaneous subdural hematoma

Sažetak
Lance-Adams syndrome (LAS), first described in 1963, is a condition characterized by development of chronic post-hypoxic action myoclonus due to a temporary lack or inadequate brain supply of oxygen. A coagulative disorder should be suspected when an unexplained hemorrhage occurs. Patients with LAS show a favourable response to sodium valproate and clonazepam. The authors present a case of a 22-year-old young man, who was admitted to the Department of Neurology of the University Hospital Center Zagreb in January 2008, clinically presenting with headache, nausea, vomiting, mild right hemiparesis and truncal ataxia. Brain MRI revealed spontaneous atypical subdural hematoma in the region of the right middle cerebellar peduncle, vermis and left cerebellar hemisphere. Laboratory findings revealed deficiency in coagulation Factor XIII. Two weeks after admission patient developed spontaneous subdural hematoma in the right frontoparietal region. In the meantime he clinically also presented with generalized action myoclonus that was, according to our beliefs, a part of chronic posthypoxic action myoclonus - Lance-Adams syndrome (secondary to intracranial hemorrhage). He had a complete regression of myoclonus after an oral administration of sodium valproate and clonazepam, and after normalization of coagulation Factor XIII concentration following replacement therapy.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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