Therapeutic plasma exchange (TPE) has been reported as a possible treatment for osmotic demyelination syndrome – central pontine myelinolysis (ODS-CPM), a degeneration of myelin within the central nervous system related to rapid hyponatremia correction, which though uncommon, has significant morbidity, and has no established specific treatment. We present our experience with a 41-year-old male with chronic kidney disease, maintained on steroids, who presented with lethargy and behavioral changes. Initial metabolic panel showed severe hyponatremia (Na 109 mEq/L). Despite cautious sodium correction, the patient’s sensorium decreased further and was intubated. Involuntary movements of the left face and arm were later seen. T2/FLAIR hyperintensities in the brainstem and thalami affirmed the diagnosis of ODS. A total of nine cycles (one cycle every two to three days) of TPE were completed. The patient was discharged with improved sensorium, from E2VxM4 to E4VxM6, and with no indication for hemodialysis due to improved creatinine. One year later, the patient has no remaining neurologic deficits. Our experience supports other case reports that TPE is a viable therapy for ODS-CPM.
Myelinolysis, Central Pontine ; Renal Insufficiency, Chronic

Extrapontine myelinolysis is a demyelinating disorder usually caused by rapid correction of chronic hyponatremia. We present an 82-year-old female patient with general weakness, and severe hyponatremia (103 mEq/L) which was corrected 14 mEq/L during 21 hours. She was stuporous and presented status epilepticus. Brain T2-weighted image showed high signal intensities of bilateral thalami and electroencephalogram monitoring indicated right hemisphere lateralized periodic discharges, maximal in the right frontal region. This case shows that status epilepticus can occur in a patient of extrapontine myelinolysis involved bilateral thalami without pontine lesions.
Aged, 80 and over ; Brain ; Demyelinating Diseases ; Electroencephalography ; Female ; Humans ; Hyponatremia ; Myelinolysis, Central Pontine ; Status Epilepticus ; Stupor

Rotavirus is the major cause of gastroenteritis in children under the age of 5. Rotavirus infection may lead to several neurological complications as meningitis, encephalitis, convulsion, encephalopathy, hemorrhagic shock, central pontine myelinolysis, Guillain-Barre syndrome, and Reye's syndrome. Further, some reports have described diffuse cerebral white matter lesions on diffusion-weighted magnetic resonance imaging (MRI) in neonates with rotavirus induced seizures. Here, we report on three neonates with rotavirus induced seizures with cerebral white matter abnormalities on MRI.
Brain Diseases ; Child ; Diffusion Magnetic Resonance Imaging ; Encephalitis ; Gastroenteritis ; Guillain-Barre Syndrome ; Humans ; Infant, Newborn ; Magnetic Resonance Imaging* ; Meningitis ; Myelinolysis, Central Pontine ; Reye Syndrome ; Rotavirus Infections* ; Rotavirus* ; Seizures* ; Shock, Hemorrhagic ; White Matter*

Osmotic demyelination syndrome (ODS) is a demyelinating disorder related to the rapid correction of hyponatremia. It usually affects the pontine area; hence, the name central pontine myelinolysis (CPM). However, it rarely occurs with the correction of hypernatremia and hyperosmolarity and involves extrapontine areas. A 56-year-old chronic alcoholic had been admitted with a history of confusion. He had been in alcohol withdrawal for 4 days. Laboratory examinations showed severe hypernatremia and hyperosmolarity. After serum sodium level was normalized; however, his clinical course did not improve and deteriorated to semicoma progressively. Magnetic resonance imaging (MRI) revealed abnormal signal intensity in the pontine and extrapontine areas, including the basal ganglia, thalamus, and cerebral cortices. This is the first case report of combined central pontine and extrapontine demyelination after alcohol withdrawal and correction of hypernatremia in a chronic alcoholic.
Alcoholics* ; Basal Ganglia ; Cerebral Cortex ; Demyelinating Diseases ; Humans ; Hypernatremia* ; Hyponatremia ; Magnetic Resonance Imaging ; Middle Aged ; Myelinolysis, Central Pontine* ; Sodium ; Thalamus

Central pontine myelinolysis (CPM) is a demyelinating disorder characterized by the loss of myelin in the center of the basis pons, and is mainly caused by the rapid correction of hyponatremia. We report the case of a young woman who presented with gait disturbance and alcohol withdrawal, and who was eventually diagnosed with CPM. Generally, the cause and pathogenesis of CPM in chronic alcoholics remain unclear. In this cases, the CPM may be unrelated to hyponatremia or its correction. However, it is possible that the osmotic pressure changes due to refeeding syndrome after alcohol withdrawal was the likely cause in this case. This case illustrates the need for avoiding hasty, and possibly incomplete diagnoses, and performing more intensive test procedures to ensure a correct diagnosis.
Alcoholics ; Demyelinating Diseases ; Diagnosis ; Female ; Gait ; Humans ; Hyponatremia ; Myelin Sheath ; Myelinolysis, Central Pontine* ; Osmotic Pressure ; Pons ; Refeeding Syndrome

Central pontine myelinolysis (CPM) is well-recognized osmotic demyelination syndrome that is related to various conditions such as rapid correction of hyponatremia and chronic alcoholism. Acute ataxia as a sole clinical sign in CPM is rare. We report a case of a 59-year-old man with dysarthria, intention tremor, and a significant gait ataxia starting after alcohol withdrawal, with radiological evidence of CPM. CPM should be included in the differential diagnosis of alcoholic patients who develop a sudden ataxia. Chronic alcohol abuse is one of the most commonly encountered predisposing factors. Alcohol withdrawal represents an additional vulnerability factor, being responsible for electrolyte imbalances which are not always demonstrable but are certainly involved in the development of CPM.
Alcoholics* ; Alcoholism ; Ataxia ; Causality ; Demyelinating Diseases ; Diagnosis, Differential ; Dysarthria ; Gait Ataxia ; Humans ; Hyponatremia ; Middle Aged ; Myelinolysis, Central Pontine* ; Tremor

Few preoperative extrapontine myelinolysis (EPM) cases with pituitary adenoma have been reported. No such case had long follow-up to see the outcome of EPM. We reported a 38-year-old man complaining of nausea, malaise and transient loss of consciousness who was found to have severe hyponatremia. Neurologic deficits including altered mental status, behavioral disturbances, dysarthria and dysphagia developed despite slow correction of hyponatremia. Endocrine and imaging studies revealed hypopituitarism, nonfunctional pituitary macroadenoma and extrapontine myelinolysis. Transsphenoidal surgery was performed after three weeks of supportive therapy, when neurological symptoms improved significantly. The patient recovered function completely 3 months after surgery. Our case indicates that outcome of EPM can be good even with prolonged periods of severe neurologic impairment.
Adult ; Deglutition Disorders ; Dysarthria ; Follow-Up Studies ; Humans ; Hyponatremia ; Hypopituitarism ; Myelinolysis, Central Pontine* ; Nausea ; Neurologic Manifestations ; Pituitary Neoplasms* ; Unconsciousness

A 65-year-old female visited us due to gait disturbance. A neurological examination showed cognitive impairment, dystonia, myoclonus, bradykinesia, postural instability, and freezing of gait (FOG). She was diagnosed with extrapontine myelinolysis based on her history of hyponatremia and high signal intensities (HSIs) in both striata on T2-weighted images. Her neurological problems including FOG improved over 25 days. In a follow-up MRI 50 days after the onset, HSIs disappeared in the striata but new ones appeared in the pons. FOG may have been related to striatal dysfunction in this patient.
Aged ; Cognition Disorders ; Dystonia ; Female ; Follow-Up Studies ; Freezing* ; Gait* ; Humans ; Hypokinesia ; Hyponatremia ; Magnetic Resonance Imaging ; Myelinolysis, Central Pontine* ; Myoclonus ; Neurologic Examination ; Pons ; Weather

A 60-year-old woman presented with cerebellar signs including dysarthria and ataxia, after intravenous infusion of cisplatin-based chemotherapy. Several blood tests showed mild neutropenia, normocytic normochromic anemia, but no evidence of a marked hyponatremia. Brain magnetic resonance imaging with diffusion-weighted sequences showed hyper-intense signal abnormalities in the extrapontine region, sparing the basis pontis. Here, we report on the case of a patient with reversible cerebellar ataxia related to extrapontine myelinolysis without hyponatremia after treatment with cisplatin-based chemotherapy for cholangiocarcinoma and discuss the literature on cerebellar ataxia in patients who underwent recent chemotherapy for malignancy.
Anemia ; Ataxia ; Brain ; Cerebellar Ataxia* ; Cholangiocarcinoma* ; Cisplatin ; Drug Therapy* ; Dysarthria ; Female ; Hematologic Tests ; Humans ; Hyponatremia* ; Infusions, Intravenous ; Magnetic Resonance Imaging ; Middle Aged ; Myelinolysis, Central Pontine* ; Neutropenia

Osmotic demyelination syndrome is a demyelinating disorder associated with rapid correction of hyponatremia. But, it rarely occurs in acute hypernatremia, and it leads to permanent neurologic symptoms and is associated with high mortality. A 44-year-old woman treated with alternative medicine was admitted with a history of drowsy mental status. Severe hypernatremia (197mEq/L) with hyperosmolality (415mOsm/kgH2O) was evident initially and magnetic resonance imaging revealed a high signal intensity lesion in the pons, consistent with central pontine myelinolysis. She was treated with 0.45% saline and 5% dextrose water and intravenous corticosteroids. Serum sodium normalized and her clinical course gradually improved. Brain lesion of myelinolysis also improved in a follow-up imaging study. This is the first report of a successful treatment of hypernatremia caused by iatrogenic salt intake, and it confirms the importance of adequate fluid supplementation in severe hypernatremia.
Adrenal Cortex Hormones ; Adult ; Brain ; Complementary Therapies ; Demyelinating Diseases* ; Female ; Follow-Up Studies ; Glucose ; Humans ; Hypernatremia* ; Hyponatremia ; Magnetic Resonance Imaging ; Mortality ; Myelinolysis, Central Pontine ; Neurologic Manifestations ; Pons ; Sodium ; Water