Pseudo-Central Pontine Myelinolysis.

ABSTRACT: Central pontine myelinolysis is most commonly associated with rapid correction of hyponatremia and has historically been associated with alcoholism. In this case report, 2 deaths with gross findings of central pontine lesions led to the possibility that CPM may have been a potential mechanism of death. Subsequent analysis revealed that these lesions were incidental findings. This case report discusses the importance of appropriate microscopic and immunohistochemical analysis of suspected CPM cases.

Neuroimaging Spectrum of Severe Hypernatremia in Infants with Neurological Manifestations.

BACKGROUND: Variable neurological manifestations and imaging findings have been described in children with severe hypernatremia. We aimed to describe the spectrum of neuroimaging changes in infants with severe hypernatremia. METHODS: This retrospective study included infants with severe hypernatremia (serum sodium >160 mEq/L), abnormal neurological examination, and an abnormal magnetic resonance imaging (MRI) of the brain over a period of 2 years in a tertiary care hospital. Relevant clinical data, including the feeding practices, clinical features, complications, and biochemical and radiological parameters, were entered in a structured pro forma. MRI findings were classified as vascular (hemorrhages and cerebral sinus venous thrombosis), osmotic demyelination syndrome (pontine and extrapontine myelinolyses), and white matter changes. RESULTS: The common clinical features in the neonates were poor feeding (n = 4) and decreased urine output (n = 4); the older infants presented with gastrointestinal losses (n = 5). All cases had dehydration with encephalopathy. The patterns of radiological injury were vascular (hemorrhages, n = 5 and venous thrombosis, n = 3), osmotic demyelination (n = 8), and white matter changes (n = 7). Coagulopathy was correlated with the vascular complications (r = 0.8, p < 0.0001); the degree of dehydration was correlated with the venous thrombosis (r = 0.7, p < 0.04) and acute kidney injury (r = 0.8, p < 0.001). Neurological sequelae were seen in four cases and correlated with hypernatremia (r = 0.6, p = 0.03) and hyperosmolarity (r = 0.6, p = 0.03). CONCLUSION: Characteristic neuroimaging findings are vascular changes in the form of venous thrombosis and hemorrhages, osmotic demyelination and white matter tract injury, and/or mostly combinations of these findings. Severe hypernatremia and resulting hyperosmolarity frequently cause neurological sequelae in neonates and infants.

Hyperglycemia-related central pontine demyelinization after a binge-eating attack in a patient with type-2 diabetes: a case report.

BACKGROUND: Here, we report a case of central pontine demyelinization in a type-2 diabetes patient with hyperglycemia after a binge-eating attack in the absence of a relevant hyponatremia. CASE PRESENTATION: A 55-year-old, male type-2 diabetic patient with liver cirrhosis stage Child-Pugh B was admitted due to dysmetria of his right arm, gait disturbance, dizziness, vertigo, and polyuria, polydipsia after a binge-eating attack of sweets (a whole fruit cake and 2 Liters of soft drinks). A recently initiated insulin therapy had been discontinued for 8 months. A serum glucose measurement obtained 5 days prior to hospitalisation was 38.5 mmol/l (694 mg/dl). The patient graved for sweets since stopping alcohol consumption 8 months earlier. On admission, venous-blood glucose was 29.1 mmol/l (523.8 mg/dl), glycated hemoglobin was 168.0 mmol/mol or 17.6%. No supplementation of sodium chloride was reported. Laboratory exams revealed an elevated serum ammonia level (127.1 µmol/l), rendering a hepatic encephalopathy very likely. After initiation of insulin therapy, capillary glucose normalized, and serum sodium rose from 133 on admission to 144 mmol/l during the hospital stay. In retrospect, the mild hyponatremia on admission was classified as pseudohyponatremia due to hyperglycemia. The patient had an insulin resistance (HOMA-IR 7.8 (normal range < 2.5)). A T2-weighted magnetic resonance imaging (MRI) of the head and a cranial computed tomography scan were obtained demonstrating a symmetric central pontine demyelinization. After 26 days in hospital, the patient was discharged with an inkretin-mimetic therapy (dulaglutide SC, 1.5 mg/week) and an intensified conventional insulin therapy (insulin aspart: 14 units/d in euglycemia, insulin glargin 20 units/d). CONCLUSIONS: Central pontine and/or cerebellar myelinolysis can be caused by sudden, severe, and sustained hyperglycemia, especially when another risk factor (in this case, liver cirrhosis) is present. Functional neurological deficits in the context of hyperglycemia should prompt for the consideration of this differential diagnosis in all diabetes patients.

A case of chronic asymptomatic central pontine myelinolysis with histological evidence of remyelination.

Central pontine myelinolysis (CPM) is a neurological demyelinating disease of the pons. Although usually associated with rapid correction of hyponatremia, CPM may occur despite normonatremia, is often associated with chronic alcoholism and may be asymptomatic. Histological confirmation of asymptomatic CPM is rare. We describe an unusual post-mortem case of extensive but asymptomatic CPM in a chronic alcoholic patient with normonatremia. The affected part of the pons contained thinly myelinated axons with appearances supporting remyelination. We suggest that remyelination may account for the subclinical nature of this patient's CPM.

[Extrapontine myelinolysis developed after aneurysmal subarachnoid hemorrhage (a case report and literature review)]. / Ekstrapontinnyi mielinoliz, razvivshiisya posle anevrizmaticheskogo subarakhnoidal'nogo krovoizliyaniya (sluchai iz praktiki i obzor literatury).

TOPICALITY: Central pontine and extrapontine myelinolysis is a rare and dangerous form of the demyelinating process of undefined origin, the development of which is associated with a very low sodium level in the blood (hypernatremia). OBJECTIVE: To describe a rare case of extrapontine myelinolysis as a complication occurring in the hemorrhagic period of anterior communicating artery aneurysm rupture as well as to demonstrate that this condition can be diagnosed intravitally. CONCLUSION: Clinical vigilance of extrapontine myelinolysis may promote the timely diagnosis and treatment of this disease, which is a potential cause of death. Pulse-therapy with glucocorticoids provides the opportunity to achieve regression of neurological symptoms and to stabilize the patient's condition.

[Case Report of a Suspected Extrapontine Myelinolysis due to Hyperglycemic Hyperosmolar Syndrome].

A 68-year-old female, who suffered from depression, fell down the stairs in her house. She was soon transferred to an accident and emergent ward by an ambulance. Two days later, she was transferred and admitted to our hospital. Next day after admission to our ward, her consciousness level changed for the worse. She developed diabetic ketoacidosis, and was administered insulin during reinfusion. Next day, her diabetic ketoacidosis improved;however, her consciousness level was still poor. Brain magnetic resonance imaging(MRI)showed suspected osmotic myelinolysis lesions in the bilateral cerebellar hemisphere and globus pallidus. After conservative therapy, her lesions almost disappeared in 2 months. We diagnosed the lesions as reversible extrapontine myelinolysis.

Identifying risk factors for central pontine and extrapontine myelinolysis after liver transplantation: a case-control study.

BACKGROUND: Central pontine and extrapontine myelinolysis (CPEPM) is a rare but potentially fatal complication after orthotopic liver transplantation (OLT). The aim of this study was to identify risk factors for development of CPEPM after OLT and to assess patient outcome. METHODS: We reviewed the clinical data of 1,378 patients who underwent OLT between 1987 and 2009 in Geneva, Switzerland and Edmonton, Canada. Nineteen patients (1.4 %) developed CPEPM. We compared their characteristics with control patients, matched by age, gender, date of OLT, and MELD score. RESULTS: The 19 patients with CPEPM (7F, mean age 52.1 ± 2 years) had a mean MELD score of 26 ± 2.2. Before OLT, patients who develop CPEPM presented more frequently low (<130 mmol/l; p < 0.04) and very low (<125 mmol/l; p < 0.009) sodium than controls. In patients developing CPEPM, the number of platelet units and fresh frozen plasma transfused during surgery was higher (p = 0.05 and 0.047), hemorrhagic complications were more frequent after OLT (p = 0.049), and variations of sodium before and after OLT were higher (p = 0.023). The association of >2 of these conditions were strongly associated with CPEPM (p = 0.00015). Mortality at 1 year of patients developing CPEPM was higher (63 vs. 13 %, p < 0.0001). CONCLUSIONS: High MELD score patients undergoing OLT, receiving massive perfusions of Na-rich products, experiencing surgery-related hemorrhagic complication and important fluctuations of Na are at risk of developing CPEPM. Therefore careful monitoring of natremia in the perioperative period and use of water-free perfusion in case of massive blood-products transfusion are critical points of this patient management.

Value of diffusion-weighted imaging in central pontine and extrapontine myelinolysis.

INTRODUCTION: Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are rare neurological disorders characterized by demyelination in and/or outside the pons. Whether diffusion-weighted imaging (DWI) might facilitate an earlier diagnosis has not yet been studied systematically. METHODS: We describe demographics, clinical presentation, and early magnetic resonance imaging (MRI) findings with special emphasis on the relevance for diagnosis of CPM and/or EPM in eight patients. RESULTS: Of the analysed eight patients (aged 37-70 years; two men, six women), CPM was diagnosed in three, EPM in one, and a combination of CPM and EPM in four patients. Aetiology was rapid correction of sodium in two patients; a combination of hyponatremia, alcoholism and alcohol withdrawal in five patients and unclear in one patient. Seven patients suffered from chronic alcoholism and four from malnutrition. Demyelinating lesions were found in the pons, thalamus, caudate nucleus, putamen and midbrain. While the lesions could be clearly delineated on T2- and T1-weighted images, DWI demonstrated a strong signal in only six patients. Furthermore, DWI demonstrated lesions only to some extent in two patients and was completely negative in two patients on initial MRI. In none of the patients did the demonstration of hyperintense lesions on DWI precede detection on conventional MRI sequences. Apparent diffusion coefficient (ADC) values were heterogenous with a decrease in two cases and an increase in the remainder. CONCLUSIONS: We conclude that early DWI changes are a common finding in CPM/EPM but do not regularly precede tissue changes detectable on conventional MRI sequences. Heterogenous ADC values possibly represent different stages of disease.

A woman with focal neurological deficit following treatment for cholera.

A 41-year old woman was treated for cholera at one of the health centers in Blantyre. Two days after discharge from the treatment unit, she developed weakness of all 4 limbs and difficulties with speech. She was referred to the Queen Elizabeth Central Hospital. A CT scan of the brain showed hypodense lesions in the pons. A diagnosis of central pontine myelinolysis was made. She recovered slowly and was discharged from hospital 17 days after admission.

Cognition and functional performance in daily activities before and after pontine and extrapontine myelinolysis: a case study.

Central pontine and extrapontine myelinolysis (CPEM) is a rare neurological condition usually due to rapid correction of hyponatremia. CPEM is mainly characterized by motor deficits (typically quadraparesis or extrapyramidal features) but neurobehavioral symptoms were also reported in the literature. However, very few studies on the neuropsychological and functional performance in daily activities have been performed in this population. In this case study, a 55-year-old woman had neuropsychology and functional evaluations prior and after the first manifestations of CPEM. The patient presented motor impairments characterized by parkinsonism symptoms and decreased dexterity. Several neurobehavioral deficits were observed including emotional lability, impulsivity, altered judgment, and disinhibition. Executive dysfunctions and impulsivity were observed during neuropsychological and functional evaluations, limiting the patient's autonomy in complex activities of daily living. The patient recovered progressively from most motor, neurobehavioral and cognitive deficits.

Centropontine myelinolysis related to refeeding syndrome in an adolescent suffering from anorexia nervosa.

Centropontine myelinolysis (CPM) is a rare neurologic disorder defined by symmetric demyelination in the central pons, mostly due to alcoholism, malnutrition, or water-electrolyte abnormalities. We report an unusual case of CPM likely due to hypophosphatemia, related to a refeeding syndrome in the context of mental anorexia. A 15-year-old girl with mental anorexia presented with hypophosphatemia in the following days of enteral refeeding, and then suffered from confusion, neurological signs, and typical MRI lesions of CPM. Hypophosphoremia may be considered as a causative agent in CPM related to refeeding syndrome. This clinical observation also highlights the importance of recognizing patients at high risk of refeeding syndrome to initiate a balanced nutrition with careful monitoring.

Central pontine and extrapontine myelinolysis associated with acute hepatic dysfunction.

Central pontine myelinolysis and extrapontine myelinolysis are rare demyelinating diseases of the central nervous system. These diseases are related frequently to rapid correction of hyponatremia. They have also been described in association with other underlying conditions such as alcoholism and malnutrition. In the present study, we report a case of central pontine and extrapontine myelinolysis with acute hepatic dysfunction. The patient had no apparent evidence of hyponatremia and no history of alcohol abuse. On admission, the patient was lethargic; dysphagia, dysarthria, and quadriplegia were noted. Laboratory examination showed significantly increased transaminase without hyponatremia. Magnetic resonance imaging revealed abnormal signal intensities in the pons and thalamus. Consciousness level and clinical symptoms improved gradually within a week. We suggest that acute hepatic dysfunction may play an important role in the development of central pontine myelinolysis and extrapontine myelinolysis.

Peduncular hallucinosis secondary to central pontine myelinolysis.

Peduncular hallucinations are generally associated with lesions in the midbrain. They have rarely been associated with central pontine myelinolysis, a condition associated with rapid alterations in serum sodium and chronic alcoholism. Described herein is the case of a 46-year-old man who developed typical peduncular hallucinations, whose imaging demonstrated central pontine myelinolysis. After alcohol cessation and neuroimaging resolution, the patient's hallucinatory phenomena abated.

Central pontine and extrapontine myelinolysis despite careful correction of hyponatremia: clinical and neuropathological findings of a case.

We report clinical, radiological and pathological findings in a patient with central pontine and extrapontine myelinolysis. The patient was a 61-year-old woman who had a radical mastectomy for breast cancer. Based on clinical evidence, acute hyponatremia had set in only a few days before onset of symptoms. The patient's disease progressed in two stages and became more severe during slow hyponatremia correction after 9 days from onset. Diffusion MRI provided early evidence of neurological lesions. In spite of a therapeutic attempt, the patient died unexpectedly 18 days after onset of her neurological disease due to massive pulmonary embolism. Histologically, our findings confirmed that the major features of central pontine myelinolysis in the acute stage are demyelination, the presence of large amounts of macrophages with no lymphocytic inflammatory reaction, and moderate astrocytosis. It is interesting to note that a monotypic immunological reaction persisted 19 days after radiological demonstration of parenchymal alterations.

A case of asymptomatic pontine myelinolysis.

Central pontine myelinolysis is an acquired, non-inflammatory demyelinating lesion usually localized in the brainstem pons basis; it usually affects patients with a history of chronic alcoholism, malnutrition or dysionemia. The exact pathogenesis of myelinolysis is still unclear. A 69-year-old Caucasian male presented intensive headache and underwent cranial MRI that showed the typical feature of central pontine myelinolysis. Neurological valuation was negative. Other examinations included extensive blood tests, electroencephalogram and multimodal evoked potentials which all gave normal results. Alcohol abuse and malabsorption syndrome were excluded. The medical history revealed a continuative use of anti-depressive drugs and exposure to glue for years. Our patient may represent one of the rare cases of asymptomatic CPM. The actual reason why he presented this lesion is not clear, but we discuss the possible role in the etiopathogenesis of his chronic use of anti-depressive drugs and the exposure to glue and chemical agents.

Central pontine myelinolysis: historical and mechanistic considerations.

Central pontine myelinolysis (CPM) is a demyelinating condition affecting not only the pontine base, but also involving other brain areas. It usually occurs on a background of chronic systemic illness, and is commonly observed in individuals with alcoholism, malnutrition and liver disease. Studies carried out 25-30 years ago established that the principal etiological factor was the rapid correction of hyponatremia resulting in osmotic stress. This article reviews progress achieved since that time on its pathogenesis, focusing on the role of organic osmolytes, the blood-brain, barrier, endothelial cells, myelinotoxic factors triggered by osmotic stress, and the role of various factors that predispose to the development of CPM. These advances show great promise in providing novel therapeutic options for the management of patients afflicted with CPM.

Central pontine myelinolysis in a pediatric head injury patient.

Central pontine myelinolysis occurs inconsistently as a complication of severe and prolonged hyponatremia, particularly when corrected too rapidly. It is a concentrated, frequently symmetric, noninflammatory demyelination within the central basis pontis. We describe a head injury patient who suffered this clinical entity as a consequence of rapidly corrected hyponatremia. Clinical manifestation and radiological findings are described along with a review of the pertinent literature.

Osmotic myelinolysis with malignant cerebellar edema occurring after DDAVP-induced hyponatremia in a child.

Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are dire neurological disorders, characterized by severe damage to the myelin sheath of neurons, which typically result from rapid correction or overcorrection of systemic hyponatremia. For many years, both conditions have been considered universally fatal, though survivors have been reported more recently. Pediatric cases are rare. We present a 13-year-old boy with panhypopituitarism secondary to repair of a nasofrontal encephalocele in infancy, managed on long-term corticosteroid, deamino arginine vasopressin and thyroid hormone. He presented with severe hyponatremia (116 mEq/l), which during correction rapidly and unexpectedly increased to 176 mEq/l, resulting in profoundly impaired consciousness. Brain imaging revealed multiple bilateral changes in the basal ganglia, thalamus, pons and cerebral white matter, consistent with both CPM and EPM. Malignant cerebellar edema necessitated emergent suboccipital craniectomy, with subsequent improvement in level of consciousness and imaging postoperatively. However, he succumbed to acute cardiorespiratory arrest 8 weeks later. Nine similar cases from the literature are reviewed.