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.

Dramatic Response to Pramipexole in Delayed-Onset Parkinsonism from Osmotic Demyelinating Syndrome.

Background: Delayed parkinsonism and dystonia are recognized phenomena in osmotic demyelinating syndrome (ODS). Dopamine receptor agonists and levodopa have been reported to benefit select patients. Case report: We report a patient with ODS with severe pseudobulbar deficits, parkinsonism and dystonia, poorly responsive to levodopa, who experienced a remarkable improvement with pramipexole. Discussion: A marked response to pramipexole with lack of response to levodopa suggests a pre-synaptic source for his deficits coupled with injuries to non-nigral compensatory structures. Highlights: This case highlights a dramatic response of osmotic demyelination-induced parkinsonism/dystonia to pramipexole. A lack of response to levodopa suggests deficits in the pre-synaptic nigral as well as non-nigral compensatory structures.

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.

Simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis: A case report of a challenging diagnosis.

RATIONALE: Marchiafava-Bignami disease (MBD) is a rare disease characterized by demyelination of the corpus callosum. It is most commonly seen in patients with chronic alcoholism. The clinical diagnosis of MBD can be difficult due to its nonspecific manifestation. Central pontine myelinolysis (CPM) occurs mostly as a complication of severe and prolonged hyponatremia, especially when corrected too rapidly. However, CPM can be associated with chronic alcoholism and its clinical presentation can be heterogeneous. Because both MBD and CPM can have fatal outcomes, early recognition and treatment can result in a better prognosis. We present a very rare case of simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis in a patient with chronic alcoholism who was diagnosed unexpectedly using brain magnetic resonance imaging and improved after proper treatment. PATIENT CONCERNS: We presented a case of a 39-year-old patient who visited the hospital with general weakness and an altered neurologic condition after a week of vomiting. DIAGNOSIS: The patient was diagnosed with simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis using brain magnetic resonance imaging. INTERVENTION: Administration of a high dose of thiamine. OUTCOMES: The neurologic signs improved after a week of thiamine administration. LESSONS: This case suggests that Marchiafava-Bignami disease and central pontine myelinolysis might have a common pathogenesis, and brain magnetic resonance imaging is of crucial importance in chronic alcoholic patients presenting with nonspecific neurological deterioration. The appropriate administration of thiamine may prevent poor outcomes.

Secondary parkinsonism in a patient of psychogenic polydipsia.

A 44-year-old man presented with history of slurring of speech, slowness in activities, abnormal posturing of the upper limbs and drooling of saliva from the mouth. He had a 5-yearlong history of compulsive water drinking, consuming 12-15 L of water every day. He was earlier evaluated for the same and found to have low serum sodium (126 mEq/L). Presently, he was admitted at a primary care facility with fever and altered sensorium. He was found to have serum sodium of 94 mEq/L. His sensorium improved with hypertonic saline infusion; however, after a lucid interval of 48 hours, he developed florid extrapyramidal symptoms. Contrast-enhanced MRI brain revealed bilaterally symmetric T2-weighted fluid-attenuated inversion recovery hyperintensity in caudate and lentiform nuclei. A diagnosis of extrapontine myelinolysis with secondary parkinsonism was made. He improved significantly with levodopa therapy.Psychogenic polydipsia is an important cause of hyponatraemia. Overzealous correction of hyponatraemia can be counterproductive.

Diffuse large B-cell lymphoma presenting with central pontine myelinolysis: a case report.

INTRODUCTION: The most common cause of central pontine myelinolysis is an overly rapid correction of hyponatremia, although it can also occur in patients with any condition leading to nutritional or electrolyte stress. We report a case of diffuse large B-cell lymphoma with central pontine myelinolysis developing at the onset of disease. To the best of our knowledge, hematological malignancies presenting with central pontine myelinolysis have been rarely reported, especially in previously untreated patients, as in our case report. CASE PRESENTATION: A 78-year-old Japanese woman presented to a neighborhood clinic with persistent high fever, edema, and general weakness. Despite the absence of specific neurological findings, brain magnetic resonance imaging showed an abnormal lesion in the central pons area of her brain (hyperintense on T2-weighted and hypointense on T1-weighted sequences), compatible with central pontine myelinolysis. She was admitted to our emergency department in a state of shock one month later. The results of her blood tests showed greatly elevated C-reactive protein and lactate dehydrogenase levels. She had severe hypoalbuminemia and mild hyponatremia, and showed signs of disseminated intravascular coagulation. Mild bilateral pleural effusion, prominent subcutaneous edema, and splenomegaly were detected on her systemic computed tomography scan. Her body fluid cultures did not show signs of infection and her spinal aspiration did not show pleocytosis or abnormal cells. A diagnosis of diffuse large B-cell lymphoma was made based on the results of her bone marrow examination. As she was critically ill before the diagnosis was made, she was treated with methylprednisolone pulse therapy, followed by systemic chemotherapy (rituximab with modified THP-COP regimen, including cyclophosphamide, pirarubicin, vindesine, and prednisolone), which resulted in complete remission and recovery without any neurological defects, and resolution of her abnormal findings on magnetic resonance imaging. CONCLUSIONS: Central pontine myelinolysis is a serious condition that may result in neuropathological sequelae and mortality, and clinicians should be aware of its potential presence in patients with malignancies.

[A case of central pontine and extrapontine myelinolysis, without hyponatremia, during alcohol withdrawal with favorable outcome]. / Un cas clinique de myélinolyse centropontine et extrapontine, sans hyponatrémie, au décours d'un sevrage alcoolique et d'évolution favorable.

Central pontine and extra-pontine myelinolysis (CPM/EPM) is a rare neurological disorder, well documented for more than 50 years but whose pathogenesis remains obscure. The existence of predisposing factors occurs in the most cases; chronic alcohol abuse is one of the most commonly encountered, among many others. 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 and/or EPM. CPM/EPM may be responsible for severe morbidity and is potentially life-threatening. The diagnosis of CPM/ EPM remains mostly clinical and is confirmed by magnetic resonance imaging of the brain. It should be considered in the setting of any unexplained neurological symptoms during the course of alcohol withdrawal or for any patient with chronic alcohol abuse, as promptly as possible, given the potentially fatal outcome.

Central pontine myelinolysis in advanced HIV infection with tuberculosis and multicentric Castleman's disease.

We present a case of central pontine myelinolysis (CPM) in a patient with advanced HIV infection and miliary tuberculosis. While hospitalized the patient developed an unusual ataxic variant of CPM with full clinical recovery. Follow-up imaging revealed resolution of pontine lesions. To our knowledge, this is the first report of a clinical and radiological recovery from CPM in advanced HIV disease. Our report extends our knowledge of neurological presentations in patients with advanced HIV infection. It highlights the importance of considering CPM in patients with advanced HIV disease presenting with an ataxic syndrome, even in the absence of an electrolyte derangement.

Respuesta favorable al uso de inmunoglobulinas intravenosas en la mielinólisis central pontina / Favourable response to the use of intravenous immunoglobulins in central pontine myelinolysis

No disponible

Desmielinización osmótica. Descripción de un caso de mielinolisis central extrapontina / Osmotic demyelination. A description of a case of central pontine myelinolysis

No disponible

Clinical and functional outcome and factors predicting prognosis in osmotic demyelination syndrome (central pontine and/or extrapontine myelinolysis) in 25 patients.

AIMS: To assess the functional and clinical outcome in a sizeable cohort of patients with osmotic demyelination syndrome (ODS) and to characterise the factors which could predict the final outcome. METHODS: Twenty five consecutive patients with ODS formed the study cohort. The diagnosis of ODS was based on clinical features with corroborating imaging findings. Two functional scales--Functional Independent Measure (FIM) and Disability Rating Scale (DRS)--were applied to assess the functional status at the time of admission, discharge and last follow-up. Patients who became independent for activities of daily living (ADL) at last follow-up were classified as favourable outcome, and those who died or became dependent for ADL were classified as a poor outcome group respectively. The Fisher exact test and Mann-Whitney U test were used to assess categorical and continuous variables respectively. RESULTS: The mean age at diagnosis was 53 ± 14 years. Five (20%) had central pontine myelinolysis, seven (28%) had extrapontine myelinolysis, and 13 (52%) had both. Hyponatraemia and hypokalaemia were noted in 20 (80%) and 10 (40%) patients respectively. Six (24%) received intravenous methylprednisolone. Eleven (46%) had a favourable outcome at a mean follow-up of 2.2 ± 2.5 years. Hyponatraemia ≤ 115 mEq (p=0.04), associated hypokalaemia (p=0.04) and low Glasgow Coma Scale (GCS) (p=0.008) at presentation were predictive of poor outcome. The mean FIM score at admission (p=0.05) and at discharge (p=0.01), and mean DRS at admission (p=0.05) were predictive of poor outcome. CONCLUSIONS: Higher GCS scores, better scores in functional scales in hospital, less severe hyponatraemia and absence of superadded hypokalaemia predicted favourable outcome.

Central pontine myelinolysis in a patient with epilepsia partialis continua and hyperglycaemic hyperosmolar state.

Central pontine myelinolysis (CPM) is a demyelinating disorder, which is associated most commonly with the rapid correction of hyponatraemia and other abrupt changes in physiological osmotic conditions. This includes the treatment of hyperosmolar hyperglycaemia in diabetes mellitus (DM) sufferers. Herein, we report a case of CPM in a 55-y-old patient with new-onset DM who presented with partial focal seizures and a sudden-onset right-sided hemiplegia. Magnetic resonance imaging revealed a lesion in the central pons. The patient responded to glucose control and antiepileptic medication, and achieved a recovery of limb function within 17 d of admission. CPM occurred in this patient before the correction of hyperglycaemic hyperosmolar state, and a disturbance in his initial electrolytes was not found. This report is the first documented case of long-term hyperglycaemic hyperosmolar state leading to CPM, and highlights that CPM can present as an isolated hemiplegia.

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.

Central pontine and extrapontine myelinolysis in a pediatric patient following rapid correction of hypernatremia.

Central pontine and extrapontine myelinolysis are uncommon disorders characterized by distinctive clinical features and typical findings on neuroimaging. Only a few cases are reported in the pediatric age group. We describe the case of a leukemic, malnourished 14-year-old boy with a high serum sodium concentration that gradually increased to 170 mmol/L. During a septic shock episode, hydration with a low sodium concentration at the rate of 104 mL/h for 24 h was administered. A rapid correction of the high serum sodium occurred, exceeding 0.5 mmol/L/h. The following day the patient developed rapid and progressive neurological impairment with clinical features characteristic of central pontine and extrapontine myelinolysis. Magnetic resonance imaging confirmed the diagnosis 11 days later. The patient was treated with steroids and intravenous immunoglobulins. He achieved an almost full neurological recovery and radiological improvement. The reported case demonstrates that central pontine and extrapontine myelinolysis can occur after excessively rapid correction of hypernatremia.