1/17. immunoglobulins are effective in pontine myelinolysis.Although the exact pathogenesis of central pontine myelinolysis (CPM) is unknown, correction of hyponatremia, thyreotropin releasing hormone, plasmapheresis, and corticosteroids seem to be effective. Assuming intravenous immunoglobulins (IVIG) to also be effective in CPM, 0.4 g/kg body weight/d immunoglobulins were applied to a 48-year-old patient who developed CPM with double vision, dysarthria, dysphagia, and left-sided hemiparesis 3 weeks after spontaneous normalization of hyponatremia. After 5 days of IVIG, his symptoms markedly improved, confirmed by improvement in the Norris score (42%), Frenchay score (19%), Kurtzke score (20%), Disability score (54%), vital capacity (26%), and peak torque (69%). The promising clinical effect of IVIG was assumed to be caused by the reduction of myelinotoxic substances, the development of antimyelin antibodies, and the promotion of remyelination. In conclusion, IVIG appear to be a promising therapeutic option in CPM.- - - - - - - - - - ranking = 1keywords = dysarthria (Clic here for more details about this article) |
2/17. Slowly progressive dystonia following central pontine and extrapontine myelinolysis.A 28-year-old woman was hospitalized with dysarthria and oro-mandibular and upper limb dystonia. Approximately 8 years prior to the current admission, the woman became severely hyponatremic due to traumatic subarachnoid hemorrhage-related SIADH. brain MRIs showed a signal increase in the central pons, thalamus and striatum on T2 weighted images compatible with central pontine and extrapontine myelinolysis. From a few months after that event, dystonia progressed slowly over the subsequent 8 years. We speculate that the particular damage chiefly to the myelin structures by myelinolytic process may have caused an extremely slow plastic reorganization of the neural structures, giving rise to progressive dystonia.- - - - - - - - - - ranking = 1keywords = dysarthria (Clic here for more details about this article) |
3/17. Central and extrapontine myelinolysis in a patient in spite of a careful correction of hyponatremia.We report the case of a 54-year-old alcoholic female patient who was hospitalized for neurologic alterations along with a severe hyponatremia (plasma Na : 97 mEq/l). She suffered from potomania and was given, a few days before admission, a thiazide diuretic for hypertension. A careful correction of plasma Na levels was initiated over a 48-hour period (rate of correction < 10 mEq/l/24h) in order to avoid brain demyelination. After a 2-day period of clinical improvement, her neurologic condition started to deteriorate. By the 5th day of admission, she became tetraplegic, presented pseudobulbar palsy, ataxia, strabism, extrapyramidal stiffness and clouding of consciousness. Scintigraphic and MRI investigations demonstrated pontine and extrapontine lesions associated with Gayet-wernicke encephalopathy. After correction of ionic disorders (hyponatremia, hypokaliemia) and vitamin B (thiamine) deficiency, the patient almost completely recovered without notable disabilities. This case illustrates that profound hyponatremia, in a paradigm of slow onset, can be compatible with life. It also demonstrates that demyelinating lesions, usually considered as a consequence of a too fast correction of hyponatremia, may occur despite the strict observance of recent guidelines. There is increasing evidence to suggest that pontine swelling and dysfunction may sometimes occur in alcoholic patients even in absence of disturbance in plasma Na levels. It is therefore of importance, while managing a hyponatremic alcoholic patient, to identify additional risk factors (hypokaliemia, hypophosphoremia, seizure-induced hypoxemia, malnutrition with vitamin b deficiency) for brain demyelination and to correct them appropriately.- - - - - - - - - - ranking = 1021.2629440189keywords = pseudobulbar, pseudobulbar palsy, palsy (Clic here for more details about this article) |
4/17. Central pontine myelinolysis.Central pontine myelinolysis (CPM), a neurologic disorder caused most frequently by rapid correction of hyponatremia, is characterized by demyelination that affects the central portion of the base of the pons. There are no inflammatory changes, and blood vessels are normal. Clinical features usually reflect damage to the descending motor tracts and include spastic tetraparesis, pseudobulbar paralysis, and the locked-in syndrome. magnetic resonance imaging of the brain, the imaging procedure of choice, shows an area of prolonged T1 and T2 relaxation in the central pons, which may have a characteristic shape. Recovery varies, ranging from no improvement to substantial improvement. To avoid CPM, correction of serum sodium in patients with hyponatremia should not exceed 12 mEq/24 h. We describe a case of CPM in a hyponatremic patient who presented with a cerebellar syndrome with no pyramidal tract involvement and in whom the rate of correction of serum sodium was within the recommended limits.- - - - - - - - - - ranking = 726.3818695214keywords = pseudobulbar, spastic (Clic here for more details about this article) |
5/17. Idiopathic central pontine myelinolysis in childhood.Central pontine myelinolysis (CPM) is rare in childhood with only a few cases reported in world literature. We report a 7-year-old male who presented with acute ataxia, swallowing difficulties, dysarthria, and radiological features consistent with the disorder. He improved remarkably with oral prednisolone therapy and was almost back to normal by 2 weeks. A review of the literature is also included.- - - - - - - - - - ranking = 1keywords = dysarthria (Clic here for more details about this article) |
6/17. EBV associated hemophagocytic syndrome accompanied by central pontine myelinolysis.The development of central pontine myelinolysis (CPM) has rarely been reported in association with hemophagocytic syndromes (HPS). Here we report a unique case of Epstein-Barr Virus (EBV)-related HPS which was accompanied with CPM. A 72-year-old man who had no significant medical history was admitted to our hospital due to high fever and progressing dysphasia and dysarthria. physical examination revealed anisocoria of the right pupil, fixed reaction to light, and paralysis of the left vagus nerves. magnetic resonance imaging revealed low signal intensity on T1-weighted images and high signal intensity T2-weighted images in the patient's central midpontine lesion. Initial work-up showed anemia and thrombocytopenia with elevated levels of serum ferritin, lactate dehydrogenase, and soluble IL-2 receptor. bone marrow aspiration revealed hemophagocytosis. The EBV genome was detected in the peripheral blood using the polymerase chain reaction method. He was diagnosed as having EBV-related HPS and CPM. Despite intensive treatment with methylpredonisolone, immunoglobulin, and etoposide, he died due to progressive disease and fungal septicemia. The etiology and relation between CPM and HPS are discussed.- - - - - - - - - - ranking = 1keywords = dysarthria (Clic here for more details about this article) |
7/17. Osmotic demyelination syndrome: reversible MRI findings in bilateral cortical lesions.A 70-year-old man who suffered from osmotic demyelination syndrome (ODS) is presented. dyspnea, pseudobulbar palsy and motor weakness were seen. MRI in the acute stage revealed focal abnormal high-signal lesions in the pons, thalamus and bilateral cortical areas on T1-weighted and FLAIR images. With corticosteroid therapy he recovered from his dyspnea and severe pseudobulbar palsy, and the spastic quadriplegia gradually improved. One year later the brain lesions had disappeared on T1-, T2-weighted and FLAIR images. To detect the cortical or subcortical lesions in ODS, FLAIR imaging should be performed routinely.- - - - - - - - - - ranking = 2042.5940630331keywords = pseudobulbar, pseudobulbar palsy, spastic, palsy (Clic here for more details about this article) |
8/17. Central pontine myelinolysis central pontine myelinolysis manifesting with massive myoclonus.Central pontine myelinolysis is a rare neurologic disorder defined by symmetric demyelination in the central base of the pons. It usually manifests with neurologic signs such as impaired consciousness, ataxia, spastic quadriparesis, pseudobulbar signs, and the locked-in syndrome which is related to a disconnection syndrome at the pontine level. We report a 17-month-old patient with kwashiorkor and hyponatremia who developed acute massive myoclonus. magnetic resonance imaging revealed a central pontine lesion. Central pontine myelinolysis is rare in infants, with only a few cases reported in the literature so far. This report presents the first infantile case of central pontine myelinolysis manifesting with massive myoclonus.- - - - - - - - - - ranking = 726.3818695214keywords = pseudobulbar, spastic (Clic here for more details about this article) |
9/17. Central pontine and extrapontine myelinolysis associated with type 2 diabetic patient with hypokalemia.Central pontine myelinolysis (CPM) is a demyelinating disease of the pons often associated with the demyelination of extrapontine areas of the central nervous system. Although the etiology and pathogenesis are unclear, CPM is usually associated with hyponatremia or its rapid correction, and chronic alcoholism is also a common underlying condition. We observed a 43-year-old man with diabetes mellitus who developed central pontine and extrapontine myelinolysis with no apparent evidence of hyponatremia, serum hyperosmolality or associated rapid correction, or history of alcohol abuse. On admission, the patient was lethargic with dysarthria, dysphagia, and mild tetraparesis and his face and lower extremities were severely edematous. Laboratory examination showed normoglycemia and normonatremia, although hypokalemia, elevated HbA1c, and nephrotic syndrome were also present. magnetic resonance imaging (MRI) revealed abnormal signal intensity in the pons, the deep layers of the cerebral cortex, and the adjacent white matter consistent with central pontine and extrapontine myelinolysis. Generalized edema was reduced by the use of diuretics and extracorporeal ultrafiltration without significant changes of serum sodium or osmolality. His consciousness level and paresis gradually improved within a few weeks. Our patient is a rare case of CPM associated with diabetes without apparent evidence of sodium or glucose imbalances.- - - - - - - - - - ranking = 1keywords = dysarthria (Clic here for more details about this article) |
10/17. Central pontine myelinolysis: case series and review.OBJECTIVE: To review central pontine myelinolysis (CPM) and osmotic demyelination syndrome (ODS) and describe the clinical features, etiologic factors, and clinical outcomes of 6 patients diagnosed with CPM or ODS. STUDY DESIGN: A retrospective case series. methods: medical records of patients diagnosed with CPM or ODS at Marshfield Clinic/St. Joseph's Hospital from 1986 to 2003 were reviewed. Chart abstraction was completed with a standardized data abstraction form. RESULTS: Six patients were identified, ranging in age from 31-73 years (mean age = 51.5 years). Clinical presentations were nonspecific. Common symptoms included lethargy and dysarthria. Five of the 6 patients had chronic alcoholism. All had improvements in their clinical conditions upon hospital discharge. CONCLUSIONS: CPM and ODS are rare demyelinating diseases of the pons and extrapontine sites. Prompt diagnosis and management of associated complications are essential for favorable clinical outcomes.- - - - - - - - - - ranking = 1keywords = dysarthria (Clic here for more details about this article) |
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