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.

[Reversible central pontine myelinolysis without hyponatremia in a child with acute lymphoblastic leukemia: a case report]. / Myélinolyse centropontine réversible sans hyponatrémie chez un enfant atteint de leucémie aigüe lymphoblastique: à propos d'un cas.

Central pontine myelinolysis is a demyelinating disorder mainly affecting the central pons. In some cases, it is associated with extrapontine myelinolysis. It is usually caused by rapid correction of hyponatremia and osmotic shock. We here report the case of a 3.5-year-old girl diagnosed with acute lymphoblastic leukemia admitted to our Oncology Unit with neutropenic fever and diarrhea. Laboratory tests showed mild neutropenia, normochromic normocytic anemia. Electrolyte tests were normal without hyponatremia. She received antibiotic therapy with Metronidazole. Five days later, she developed flaccid quadriparesis with mutism. Computerized tomography (CT) scan was normal, cerebrospinal fluid (CSF) examination was normal (there was no evidence of leukemic cells) and ophthalmological examination did not show any abnormalities. Brain MRI found hyperintense signal in the pons. The child improved without specific treatment, and clinical and complete neurological recovery was noted. This case highlights that myelinolysis can occur under some circumstances not related with hyponatremia such as malignancy, chemotherapy.

An Unusual Cause of Bulbar Palsy in the Emergency Department.

BACKGROUND: Osmotic demyelination syndrome commonly occurs after rapid correction of hyponatremia. But it has also been reported after graded correction of hyponatremia in the presence of other risk factors like chronic alcoholism, malnutrition, liver disease, and hypokalemia. CASE REPORT: We report a case of a 67-year-old man who presented with dysphagia and nasal regurgitation and had features suggestive of bulbar palsy on neurological examination. He had spontaneous rapid correction of hyponatremia from a serum sodium level of 122 mEq/L to 132 mEq/L after discharge from our hospital. Neuroimaging was suggestive of extrapontine myelinolysis involving the basal ganglia. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: An emergency physician should be aware of this because osmotic demyelination syndrome should also be considered in the differential diagnosis of patients presenting with bulbar palsy to the emergency department.

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.

Pituitary tumor apoplexy associated with extrapontine myelinolysis during pregnancy: A case report.

RATIONALE: Pituitary tumor apoplexy (PTA) is a rare clinical syndrome which requires urgent diagnosis and treatment due to its life-threatening consequences. Management of undiagnosed pituitary tumor before pregnancy is a problem during pregnancy. PATIENT CONCERNS: We reported a case with PTA which was not diagnosed before pregnancy presenting with vomiting associated with hyponatremia during the third trimester. After supplying the sodium the patient presented with dysarthria and hemiplegia. DIAGNOSES: MRI examination showed PTA accompanied with extrapontine myelinolysis (EPM). INTERVENTIONS: The patient was given hydrocortisone according to the symptoms gradually to taper off dose, at the same times oral levothyroxine therapy (25µg/day) was given. OUTCOMES: The patient delivered a healthy baby via cesarean section at hospital at 38 + 1 week of gestation. We performed MRI examination regularly and the tumor regressed significantly 8 months postpartum. LESSONS: We reported a case as PTA associated with EPM. Headache during pregnancy is often nonspecific, so careful medical history inquiry is very important.

[Central pontine myelinolysis during qualified alcohol withdrawal therapy. A case report]. / Das Auftreten der zentralen pontinen Myelinolyse während des qualifizierten Entzugs von Alkohol. Ein Fallbericht.

Central pontine myelinolysis is a rare but severe disease that often occurs in alcohol-dependent and malnourished patients. One pathological mechanism is the rapid correction of chronic hyponatremia, even though the disease can occur independently of decreased serum sodium levels. Here, we present a patient suffering from malnutrition, alcohol dependency, and a severe depressive disorder, who presented himself to our clinic wishing for qualified withdrawal treatment. Because the patient reported significant weight loss and nocturnal sweating without fever, we performed different diagnostic investigations and examinations. Cranial MRI revealed the presence of a central pontine myelinolysis. In the clinical neurological examination, the patient only showed slight gait ataxia. The depressive symptoms had improved while the patient now showed problems in his short-term memory. At presentation, only slight hyponatremia was present, while no rapid correction occurred throughout treatment. The presented case reveals the importance of considering osmotic demyelination disorders as a differential diagnosis in patients suffering from neurological symptoms during alcohol withdrawal therapy. This is important independently of hyponatremia.

Mielinólisis central pontina de curso subagudo secundario a hiperglucemias / Subacute central pontine myelinolysis secondary to hyperglycaemia

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Central Pontine Myelinosis and Osmotic Demyelination Syndrome.

BACKGROUND: Osmotic demyelination syndrome (ODS), which embraces central pontine myelinolysis (CPM) and extrapontine myelinosis (EPM), is often underdiagnosed in clinical practice, but can be fatal. In this article, we review the etiology, patho- physiology, clinical features, diagnosis, treatment, and prognosis of ODS. METHODS: Pertinent publications from the years 1959 to 2018 were retrieved by a selective search in PubMed. RESULTS: The most common cause of ODS is hyponatremia; particular groups of patients, e.g., liver transplant recipients, are also at risk of developing ODS. The pathophysiology of ODS consists of cerebral apoptosis and loss of myelin due to osmotic stress. Accordingly, brain areas that are rich in oligodendrocytes and myelin tend to be the most frequently affected. Patients with ODS often have a biphasic course, the first phase reflecting the underlying predisposing illness and the second phase reflecting ODS itself, with pontine dysfunction, impaired vigilance, and movement disorders, among other neurological abnormalities. The diagnostic modality of choice is magnetic resonance imaging (MRI) of the brain, which can also be used to detect oligosymptomatic ODS. The current mainstay of management is prevention; treatment strategies for manifest ODS are still experimental. The prognosis has improved as a result of MRI-based diagnosis, but ODS can still be fatal (33% to 55% of patients either die or remain permanently dependent on nursing care). CONCLUSION: ODS is a secondary neurological illness resulting from a foregoing primary disease. Though rare overall, it occurs with greater frequency in certain groups of patients. Clinicians of all specialties should therefore be familiar with the risk constellations, clinical presentation, and prevention of ODS. The treatment of ODS is still experimental at present, as no evidence-based treatment is yet available.

[Osmotic demyelination syndrome complicating the quick correction of a severe hyponatremia associated with hypokalemia]. / Syndrome de démyélinisation osmotique compliquant la correction rapide d'une hyponatrémie sévère associée à une hypokaliémie.

Osmotic demyelination syndrome is characterized by the loss of myelin in the center of the basis pontis and other areas of the central nervous system. We report a case of osmotic demyelination syndrome in a 55-year-old female, with a past medical history of arterial hypertension and multi-level cervical spondylosis, hospitalized for acute altered mental status complicating an array of acute gastroenteritis, the patient was afebrile. The course was marked by neurologic aggravation with confusion, aphasia, tetraplegia and osteo-tendinous areflexia. Initial cerebral magnetic resonance imaging did not show any specific abnormalities. The diagnosis of Central pontine myelinolysis and extrapontine myelinolysis was confirmed by a cerebral magnetic resonance imaging done after 20 days of the first. The rapid correction of hyponatremia was the main cause of this syndrome, without neglecting the very likely role of the associated hypokalemia. The evolution of centropontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) is variable. The treatment is primarily preventive based on the careful correction of severe hyponatraemia and contributing factors.

Treatment response in osmotic demyelination syndrome presenting as severe parkinsonism, ptosis and gaze palsy.

Osmotic demyelination syndrome commonly affects the pons and infrequently involves the extrapontine region. We report a patient with severe hyponatraemia who developed osmotic demyelination syndrome as a consequence of rapid sodium correction. The condition manifested as acute severe parkinsonism, bilateral ptosis and gaze impairment. MRI revealed typical features of central pontine and extrapontine myelinolysis. The patient improved gradually after treatment with a combination of levodopa, intravenous immunoglobulin and dexamethasone. However, it is important to emphasise that the improvement of neurological symptoms is not necessarily causal with these experimental therapies.

Desmielinización osmótica pontina y extrapontina durante un puerperio complicado / Osmotic demyelination pontine and extrapontine during complicated puerperium

La mielinolisis pontina central es una rara alteración neurológica de etiología desconocida. La causa más frecuente es el tratamiento de una hiponatremia cuando los niveles de sodio se elevan demasiado rápido. La lesión básica consiste en la destrucción de las vainas de mielina, que afecta parte o toda la base de la protuberancia en forma simétrica (desmielinización pontina). Los pacientes con esta enfermedad suelen presentar tetraparesia progresiva subaguda acompañada de parálisis seudobulbar con disartria e imposibilidad de protruir la lengua. Se presenta un estudio con el objetivo de describir el comportamiento de un caso de desmielinización osmótica en una paciente como parte de un puerperio complicado. Se trata de una paciente femenina, mestiza, de 33 años de edad, con embarazo complicado, secundario a cesárea por óbito fetal, tras la cual lleva ingreso en terapia intensiva. Luego de varias alteraciones hidroelectrolíticas se detecta cuadriplejia fláccida, con síndrome de enclaustramiento, con diagnóstico por neuroimagen de una desmielinización osmótica, con evolución favorable(AU)

Central pontine myelinolysis is a rare neurological disorder of unknown etiology. The most frequent cause is the treatment of hyponatremia when sodium levels rise too fast. The destruction of the myelin sheaths is the basic lesion, which affects part or the entire base of the protuberance symmetrically (pontine demyelination). Patients with this disease usually present subacute progressive tetraparesis accompanied by pseudobulbar paralysis with dysarthria and the inability to protrude their tongue. A study is presented with the objective of describing the behavior of a case of osmotic demyelination in a patient as part of complicated puerperium. This is a 33-year-old female patient, with complicated pregnancy, secondary to cesarean section due to fetal death, after which she is admitted to intensive care. After several hydroelectrolyte alterations, flaccid quadriplegia was detected, with Locked-in Syndrome, with neuroimaging diagnosis of osmotic demyelination, with a favorable evolution(AU)

Tongue fasciculations with denervation pattern in osmotic demyelination syndrome: a case report of diagnostic dilemma.

BACKGROUND: The pathogenesis of osmotic demyelination syndrome is not completely understood and usually occurs with severe and prolonged hyponatremia, particularly with rapid correction. It can occur even in normonatremic patients, especially who have risk factors like alcoholism, malnutrition and liver disease. Bilateral tongue fasciculations with denervation pattern in electromyogram is a manifestation of damage to the hypoglossal nucleus or hypoglossal nerves. Tongue fasciculations were reported rarely in some cases of osmotic demyelination syndrome, but the exact mechanism is not explained. CASE PRESENTATION: A 32-year-old Sri Lankan male, with a history of daily alcohol consumption and binge drinking, presented with progressive difficulty in walking, dysphagia, dysarthria and drooling of saliva and alteration of consciousness. On examination he was akinetic and rigid resembling Parkinsonism with a positive Babinski sign. Clinical features were diagnostic of osmotic demyelination syndrome and MRI showed abnormal signal intensity within the central pons and basal ganglia. He also had tongue fasciculations. The electromyogram showed denervation pattern in the tongue with normal findings in the limbs. Medulla and bilateral hypoglossal nerves were normal in MRI. CONCLUSION: We were unable to explain the exact mechanism for the denervation of the tongue, which resulted in fasciculations in this chronic alcoholic patient who developed osmotic demyelination syndrome. The hypoglossal nuclei are located in the dorsal medulla and radiologically undetected myelinolysis of the medulla is a possibility. Hypoglossal nerve damage caused by methanol or other toxic substances that can contaminate regular ethyl alcohol is another possibility, as it is known to cause neurological and radiological features similar to osmotic demyelination syndrome with long-term exposure. So these toxic substances might play a role in chronic alcoholic patients with central pontine myelinolysis.

Osmotic demyelination syndrome in type 1 diabetes in the absence of dyselectrolytaemia: an overlooked complication?

Central pontine myelinolysis (CPM) is a demyelinating disorder of central nervous system which involves central portion of the pons and sometimes extrapontine areas also. It is commonly reported in settings of hyponatraemia or its rapid correction, but in the last few years it has also been reported in patients with diabetes in the absence of electrolyte disturbances or correction of serum osmolality. Here we report a case of a 20-year-old female patient, with a known history of type 1 diabetes mellitus, who presented with acute onset spastic quadriparesis with dysarthria and mild ataxia which evolved over 2 weeks. Her MRI brain showed well-defined, bilateral symmetric hyperintense lesion involving central pons showing area of diffusion restriction which was consistent with CPM. Patient was treated conservatively and improved over a period of few weeks. To diagnose more number of cases, we should not overlook CPM in patients with diabetes.

[A case report of central pontine and extrapontine myelinolysis in the combination with spinal cord damage in a patient with tuberculosis in the lung]. / Sluchai tsentral'nogo pontinnogo i ekstrapontinnogo mielinoliza v sochetanii s porazheniem spinnogo mozga u bol'nogo tuberkulezom legkikh.

A case report of central pontine and extrapontine myelinolysis in the combination with spinal cord damage is presented. The authors analyze literature data on this problem and discuss the pathogenesis and diagnostic issues of myelinolysis.

Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management.

Central pontine myelinolysis (CPM) is a rare condition usually caused by rapid sodium correction in hyponatraemia after a severe neurological syndrome. Only few cases have been reported during pregnancy, most of which were reported in patients with hyperemesis. We describe the successful management of the first case of twin pregnancy in a patient who presented with CPM after treatment for premature labour and then review the literature on CPM in pregnancy (aetiology, diagnosis and management). Our patient required emergency delivery to achieve electrolyte and fluid balance. At six months, the twins remained asymptomatic and the mother had minor sequelae. The aetiology is not clear, and there is no evidence regarding the optimal treatment or prognosis of CPM. In our patient, desmopressin-contaminated atosiban showed a certain probability in the Karch-Lasagne algorithm of a causality relationship between hyponatraemia and the drug. To our knowledge, this is the first case of myelinolysis reported in a twin pregnancy possibly related to desmopressin-contaminated atosiban.