Central pontine and extrapontine myelinolysis: from epileptic and other manifestations to cognitive prognosis.
The objective of this study is to review the presentation, outcome and aetiology of central pontine and extrapontine myelinolysis (CPEPM) in a tertiary hospital center. The study method is a case series and included identification of patients from University of Montreal Health Centre archives database (1995-2007). All diagnoses were confirmed by neuroimaging or brain autopsy. Twelve individuals (25-66 years old) presented heterogeneous manifestations. Co-morbidities included diabetes insipidus (n = 2), haemodialysis (n = 1), cirrhosis (n = 3), gastroenteritis (n = 2) and potomania (n = 1). Aetiologies included rapid correction of severe hyponatremia (n = 6)/acute hypernatremia (n = 1); immediate (n = 2) or remote (n = 1 with recurrent cirrhosis) orthotopic liver transplantation (OLT) with tacrolimus-induced immunosuppression (n = 3); and chronic alcoholism (n = 4, two with hyponatremia). Four individuals died acutely. Two were lost to follow-up. Six had good motor or cerebellar recovery. Neuropsychological evaluations (n = 5/6) revealed a subcortical/frontal dysfunction. Cognitive impairment represented the major remaining lasting sequel (n = 4). Three salient clinical syndromes were observed: (1) predominant cerebellar presentation in individuals with alcoholism (n = 4); (2) significant alteration of consciousness at presentation (n = 4), all resulting in death (OLT, n = 3); (3) seizures persisting after natremia correction (n = 2). Clinical presentation of CPEPM is heterogeneous and can even include seizures. Cognitive impairment should be screened as it is a significant factor limiting return to normal life.
Neurology Division, Department of Medicine, Montreal University Health Centre (CHUM), CHUM-Hôpital Notre-Dame, 1560 rue Sherbrooke Est, Montréal, QC, Canada.
Céline
C
Odier
Dang Khoa
DK
Nguyen
Michel
M
Panisset
D000328:Adult; D000368:Aged; D000437:Alcoholism / Q000150:complications; D003072:Cognition Disorders / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D015897:Comorbidity; D003244:Consciousness Disorders / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D004827:Epilepsy / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007010:Hyponatremia / Q000150:complications; D016867:Immunocompromised Host / Q000502:physiology; D007166:Immunosuppressive Agents / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D017590:Myelinolysis, Central Pontine / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D009434:Neural Pathways / Q000473:pathology / Q000503:physiopathology
D007166:Immunosuppressive Agents