Central pontine and extrapontine myelinolysis: from epileptic and other manifestations to cognitive prognosis

Abstract

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.

Clinical practice guideline on diagnosis and treatment of hyponatraemia

“…We found 54 cases (63% female, median age 45 years, interquartile range 45-58 years) of osmotic demyelination syndrome published since 1997: 45 individual case reports and three case series including a total of nine patients (72,129,130,131,132,133,134,135,136,137,138,139,140,141,142,143,144,145,146,147,148,149,150,151,152,153,154,155,156,157,158,159,160,161,162,163,164,165,166,167,168,169,170,171,172,173,174,175). In 96% (52/54), the diagnosis of osmotic demyelination syndrome was based on magnetic...…”

MRI findings of corticosubcortical lesions in osmotic myelinolysis: report of two cases

“…Gocht et al [2] examined lesion distribution in 58 autopsy cases with OM and found cerebral corticosubcortical lesions in 15%. To the best of our knowledge, corticosubcortical lesions associated with OM have been unfamiliar clinically, and their imaging findings have been reported only sporadically [4][5][6][7][8][9]. Okeda et al [3] studied the pathological features of corticosubcortical lesions in autopsy cases with OM and reported two distinct subtypes of lesions: cortical and subcortical.…”

“…In the literature, MRI findings of OM consist primarily of hyperintensity on T 2 weighted images (T 2 WI) or fluid-attenuated inversion-recovery (FLAIR) images in the central pons (central-pontine myelinolysis), midbrain, bilateral thalami and/or basal ganglia (extrapontine myelinolysis), or a combination of these [1]. Although cerebral corticosubcortical involvements have been described in pathological studies [1][2][3], a description of the MRI findings has been mostly limited to case reports [4][5][6][7][8].We report serial MRI findings in two patients with corticosubcortical lesions associated with OM. One patient's lesions were disclosed on MRI that included diffusion-weighted imaging (DWI) and T 1 weighted imaging (T 1 WI) with contrast enhancement that were obtained 8 days (late acute phase) and 26 days (subacute phase) after the onset of neurological symptoms.…”

Wide Central Pontine, Bulbar and Thalamic Myelinolysis with Sequela

“…The classical symptoms of myelinolysis are spastic quadriparesia and pseudobulbar paralysis, which The frequently observed symptoms of this disorder include acute para-or quadriparesis, dysphagia, dysarthria, diplopia, loss of consciousness, and other neurological symptoms associated with brainstem damage [7,8]. Our patient presented with tetraparesis that progressed to pseudobulbar paralysis and inability to breathe.…”