Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of neuro-Behcet disease presenting as a thalamo-lenticular expanding lesion is reported. A 41-year-old female was admitted with mental deterioration and right hemiparesis. She had been suffering from recurrent oral and genital ulcers and erythema nodosum for several years before admission. Neurological examination on admission revealed poor mental activity, dysarthria and right hemiparesis. Lumbar puncture showed CSF pleocytosis. CT and MRI revealed a thalamo-lenticular expanding lesion. CT showed a homogeneous hypodense lesion of the left lenticulothalamic region, which was enhanced in the central part, exerting a mild mass effect. MRI also revealed the lesion better. The T2 weighted images showed a high intensity signal in the left thalamo-lenticular region and the left peduncle. The T1 weighted images showed a low signal in the corresponding areas with a central enhancement with Gd-DTPA. EEG, SPECT and angiography indicated normal findings. Histologic study of the brain biopsy tissue ruled out a tumor but did not show any specific diagnosis. The patient improved with steroid therapy. In conclusion, the clinical and radiological presentation of neuro-Behcet disease can mimic a cerebral tumor. In such cases, stereotactic biopsy is useful to exclude suspicion of a cerebral tumor.
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PMID:[Neuro-Behcet disease mimicking a thalamic tumor]. 1145 98

Most movement disorders, reflecting degenerative disorders, develop in a slowly progressive fashion. Some movement disorders, however, manifest with an acute onset. We wish to give an overview of the management and therapy of those acute-onset movement disorders.Drug-induced movement disorders are mainly caused by dopamine-receptor blockers (DRB) as used as antipsychotics (neuroleptics) and antiemetics. Acute dystonic reactions usually occur within the first four days of treatment. Typically, cranial pharyngeal and cervical muscles are affected. Anticholinergics produce a prompt relief. Akathisia is characterized by an often exceedingly bothersome feeling of restlessness and the inability to remain still. It is a common side effect of DRB and occurs within few days after their initiation. It subsides when DRB are ceased. Neuroleptic Malignant Syndrome is a rare, but life-threatening adverse reaction to DRB which may occur at any time during DRB application. It is characterised by hyperthermia, rigidity, reduced consciousness and autonomic failure. Therapeutically immediate DRB withdrawal is crucial. Additional dantrolene or bromocriptine application together with symptomatic treatment may be necessary. Paroxysmal dyskinesias are childhood onset disorders characterised by dystonic postures, chorea, athetosis and ballism occurring at irregular intervals. In Paroxysmal Kinesigenic Dyskinesia they are triggered by rapid movements, startle reactions or hyperventilation. They last up to 5 minutes, occur up to 100 times per day and are highly sensitive to anticonvulsants. In Paroxysmal Non-Kinesiogenic Dyskinesia they cannot be triggered, occur less frequently and last longer. Other paroxysmal dyskinesias include hypnogenic paroxysmal dyskinesias, paroxysmal exertional dyskinesia, infantile paroxysmal dystonias, Sandifer's syndrome and symptomatic paroxysmal dyskinesias. In Hereditary Episodic Ataxia Type 1 attacks of ataxia last for up to two minutes, may be accompanied by dysarthria and dystonia and usually respond to phenytoin. In Type 2 they can last for several hours, may be accompanied by vertigo, headache and malaise and usually respond to acetazolamide. Symptomatic episodic ataxias can occur in a number of metabolic disorders, but also in multiple sclerosis and Behcet's disease.
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PMID:Diagnosis and management of acute movement disorders. 1620 29

Micturitional disturbances are reported in 5-20% of patients with Behcet disease (BD) affecting the central nervous system. However, corresponding data regarding urodynamic and electrophysiological findings are limited. A patient with known BD presented with dysarthria, diplopia and urinary frequency (36 times/day). MRI revealed an extensive lesion involving the lateral and tegmental pons, reaching the pontomedullary junction. Auditory evoked potentials indicated a left-side lesion between superior olivary nucleus and superior colliculus. Blink reflex examination indicated a location caudal to the left trigeminal root. Pudendal nerve somatosensory evoked potentials and transcranial magnetic stimulation of the perineal muscles were slightly affected. Bulbocavernosus reflex latencies were normal. EMG of the bulbocavernosus muscles showed a normal maximal voluntary contraction activity. Urodynamic studies revealed normal urine volume, maximum flow rate and residual volume. After intravenous administration of methylprednisolone diplopia and dysarthria resolved within 3 weeks. Urinary frequency remained almost unchanged for the first 8 weeks, but clearly improved during the following months. We assume that the present case of urinary frequency is the result of vasculitic lesion affecting the pontine micturition inhibitory area on the ground of Neuro-Behcet disease.
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PMID:Urinary frequency in a case of Neuro-Behcet disease involving the brainstem - clinical, electrophysiological and urodynamic features. 1769 85

A 47-year-old man with Behcet's disease presented with a five day history of right side weakness and dysarthria. Ten years ago, he was diagnosed as Behcet's disease. Since then, he was treated with low dose steroids. T1-weighted magnetic resonance images with gadolinium injection revealed ring enhancing lesions with internal hypointensities at the left side of the pons and at the left parietal cortex suggesting multiple metastatic tumors. Diffusion-weighted image and ADC map revealed central hyperintensity on diffusion with low ADC values at both lesions. The histopathology of stereotactic biopsy specimen showed frequent perivascular lymphocytic cuffings, focal necrotic lesions and reactive gliosis. The most common neuropathologic findings in neuro-Behcet's disease are focal necrotic lesions, but the location in cerebral cortex has not been reported before. Long term steroid therapy and antibiotics were administered and the patient recovered gradually. Neuro-Behcet's disease could be presented as brain tumor like lesion, and stereotactic biopsy should be considered in such situation.
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PMID:Neuro-Behcet's disease mimicking multiple brain tumors: diffusion-weighted MR study and literature review. 1785 Aug 21

We report a case of a 52-year-old woman with Behcet's disease who presented with dysarthria and right-sided hemiparesis. T2-weighted and diffusion-weighted images (DWI) showed a hyperintense lesion in the left pons with a relatively decreased apparent diffusion coefficient (ADC). Imaging showed almost complete resolution of the lesion after treatment with prednisolone. The atypical DWI and ADC findings in this case may reflect cytotoxic edema due to excitotoxic brain injury. This case thus illustrates the radiological diversity of neuro-Behcet's lesions.
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PMID:Cytotoxic edema in neuro-Behcet's disease ? 1904 64

The central nervous system involvement in Behcet's disease, usually called neuro-Behcet's disease (NBD), includes acute type and chronic progressive type. Acute NBD is characterized by acute meningoencephalitis with focal lesions, presenting high-intensity areas on T2-weighted images or FLAIR images on magnetic resonance imaging (MRI). Cyclosporin A (CyA)-related acute NBD is almost identical to CyA-unrelated acute NBD in terms of clinical manifestations, laboratory data, and responses to steroids, except for the paucity of relapse on discontinuation of CyA. Chronic progressive NBD is characterized by intractable slowly progressive dementia, ataxia, and dysarthria with persistent elevation of the cerebrospinal fluid (CSF) IL-6 activity and progressive brain stem atrophy on MRI. Chronic progressive NBD is strongly associated with male gender, HLA-B51, and cigarette smoking. Diagnostic criteria have been proposed on the basis of the results of a retrospective multicenter cohort study, using CSF cell counts for acute NBD, and CSF IL-6 activity with brain stem atrophy on MRI for chronic progressive NBD, respectively. Attacks of acute NBD respond well to moderate-to-high doses of corticosteroids. Moreover, colchicine is effective in preventing relapses of acute NBD, especially in patients who developed the disease in the absence of CyA. Chronic progressive NBD is resistant to conventional treatment with steroid, cyclophosphamide, or azathioprine, but responds to low-dose methotrexate. Infliximab has also been found to be effective in patients with chronic progressive NBD when the response to methotrexate is inadequate.
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PMID:[Recent aspects of Neuro-Behcet's disease]. 2420 Jun 2