Naslov
Central pontine myelinolisis in Wilson's disease

Autori
Marčinko, Anita ; Budinčević, Hrvoje ; Oršolić, Krešo ; Bielen, Ivan.

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

Izvornik
Neurologia Croatica 55(Suppl 2) / - , 2006, 152-153

Skup
46th International Neuropsychiatric Pula Symposium

Mjesto i datum
Pula, Hrvatska, 14.06.2006. - 17.06.2006

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Central pontine myelinolysis. Wilson’s Disease

Sažetak
Central pontine myelinolysis (CPM) is a demyelinating disease of the pons often associated with demyelination of other areas of the central nervous system. Clinical presentation of CPM is highly variable: the symptoms range from severe neurological disorders to mild neurological disturbances only. The etiology and pathogenesis of this disorder are still not fully understood. CPM is related to the various conditions: alcoholism, chronic renal failure being treated with dialysis, hepatic failure, lymphoma, carcinoma, cahexia, changes in serum sodium concentration and hypoglycaemia. The aim of this report is to present a rarely reported relation between CPM and Wilson's disease. Case report: We report a 26-years old woman with insulin-dependent diabetes mellitus who presented with dysarthria, gait disturbance and vomiting. Initial laboratory findings showed hypoglycemia, mild hyponatremia and hypokalemia. During the next two days, the patient's neurological deficit progressed to anarthria, limited conjugate eye movements and tetraparesis. MRI of the brain showed T2 hyperintensity in the central and dorsal part of the pons, midbrain, thalamic and caudate nucleus. Cytological and biochemical examinations of the CSF were normal. Laboratory studies showed a decreased ceruloplasmin and copper serum levels and an increased urinary copper excretion. An ophthalmologic slit-lamp examination verified Kayser-Fleischer rings. Three weeks later improvement in neurological symptoms has been noted. Three months later on the control examination following rehabilitation only mild dysarthria was present. Control MRI of the brain eight months later showed hyperintense signal in the dorsal part of the pons, midbrain, thalamic and caudate nucleus on T2 weighted images, but not in the central part of the pons. Based on the presented case-report, we think that the Wilson's disease could play a role in the pathogenesis of CPM. In addition, in cases of CPM with extrapontine lesions Wilson's disease should be considered as one of the conditions in the differential diagnosis of etiologically unclear CPM.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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