1/204. Cervicomedullary astrocytoma simulating a neuromuscular disorder.A 12-year-old male developed progressive proximal upper extremity weakness over a 3- to 4-year period. The clinical findings of proximal upper extremity weakness and atrophy, prominent scapular winging, and no sensory deficits or upper motor neuron signs suggested a neuromuscular disorder. electromyography was consistent with a chronic denervating disorder involving the upper cervical anterior horn cells or their axons. A cervical magnetic resonance image revealed a large intramedullary mass extending from the inferior aspect of the fourth ventricle down to the level of T2. A biopsy of the lesion was consistent with a low-grade astrocytoma.- - - - - - - - - - ranking = 1keywords = motor neuron, anterior horn cell, motor, anterior horn, neuron, horn cell, horn, upper (Clic here for more details about this article) |
2/204. An uncommon mechanism of brachial plexus injury. A case report.PURPOSE: To report a case of brachial plexus injury occurring on the contralateral side in a patient undergoing surgery for acoustic neuroma through translabrynthine approach. CLINICAL FEATURES: A 51-yr-old woman underwent surgery for acoustic neuroma through translabrynthine approach in the left retroauricular area. She had a short neck with a BMI of 32. Under anesthesia, she was placed in supine position with Sugita pins for head fixation. The head was turned 45 degrees to the right side and the neck was slightly flexed for access to the left retroauricular area, with both arms tucked by the side of the body. Postoperatively, she developed weakness in the right upper extremity comparable with palsy of the upper trunk of the brachial plexus. hematoma at the right internal jugular vein cannulation site was ruled out by CAT scan and MRI. The only remarkable finding was considerable swelling of the right sternocleidomastoid and scalene muscle group, with some retropharyngeal edema. An EMG confirmed neuropraxia of the upper trunk of brachial plexus. She made a complete recovery of sensory and motor power in the affected limb over the next three months with conservative treatment and physiotherapy. CONCLUSIONS: brachial plexus injury is still seen during anesthesia despite the awareness about its etiology. Malpositioning of the neck during prolonged surgery could lead to compression of scalene muscles and venous drainage impedance. The resultant swelling in the structures surrounding the brachial plexus may result in a severe compression.- - - - - - - - - - ranking = 0.072990412448436keywords = motor, upper, lateral (Clic here for more details about this article) |
3/204. Ankylosing spondylitis and multiple sclerosis.Ankylosing spondylitis can be associated with extra-articular involvement. Besides internal and ocular complications, neurological manifestations such as single root lesions, compression of the myelum or the cauda equina syndrome have also been described. We present a patient with ankylosing spondylitis who developed a monophasic myelopathy resembling multiple sclerosis. literature data show no conclusive evidence for an increased association of ankylosing spondylitis and multiple sclerosis. However, a monophasic myelopathy may be a separate neurological manifestation associated with ankylosing spondylitis.- - - - - - - - - - ranking = 0.0050846943832566keywords = sclerosis (Clic here for more details about this article) |
4/204. Autosomal dominant distal myopathy not linked to the known distal myopathy loci.The distal myopathies are clinically, pathologically and genetically heterogenous. Thus far, seven types of distal myopathy have been linked to four chromosome loci. We recently examined four affected members from three generations of an autosomal dominant distal myopathy kindred. A muscle biopsy was performed on the index case. Muscle histopathology showed non-specific myopathic findings including increased variation in fiber size and increased internalized nuclei. No abnormal inclusions or vacuoles were present. Microsatellite markers for the four distal myopathy loci on chromosomes 2, 9 and 14 were studied on affected and several unaffected family members. Affected patients developed distal weakness in anterior foreleg muscles followed by progressive distal upper and proximal lower extremity involvement. Chromosome 2, 9 and 14 regional markers were informative and demonstrated recombinations with affected individuals in the pedigree. The resulting LOD scores obtained from the multipoint analyses gave no evidence of positive linkage to any of the regions and positively excluded (lod score less than -2) all, or virtually all, of the candidate regions examined. This autosomal dominant distal myopathy family does not show evidence of linkage to any of the known distal myopathy loci, suggesting the existence of at least one more distal myopathy locus. Furthermore, the clinical and pathological features appear distinct from other previously described but genetically-undetermined autosomal dominant distal myopathies.- - - - - - - - - - ranking = 0.00031750984993107keywords = upper (Clic here for more details about this article) |
5/204. Application of a rheolytic thrombectomy device in the treatment of dural sinus thrombosis: a new technique.We present a novel application of a transvascular rheolytic thrombectomy system in the treatment of symptomatic dural sinus thrombosis in a 54-year-old woman with somnolence and left-sided weakness. The diagnosis of bilateral transverse and superior sagittal sinus thrombosis was made and the patient was treated with anticoagulant therapy. After an initial period of improvement, she became comatose and hemiplegic 8 days after presentation. After excluding intracerebral hemorrhage by MR imaging, we performed angiography and transfemoral venous thrombolysis with a hydrodynamic thrombectomy catheter, followed by intrasinus urokinase thrombolytic therapy over the course of 2 days. This technique resulted in dramatic sinus thrombolysis and near total neurologic recovery. Six months after treatment, the patient showed mild cognitive impairment and no focal neurologic deficit. Our preliminary experience suggests that this technique may play a significant role in the endovascular treatment of this potentially devastating disease.- - - - - - - - - - ranking = 0.000191527212737keywords = lateral (Clic here for more details about this article) |
6/204. Lessons to be learned: a case study approach: prolonged methaemoglobinaemia due to inadvertent dapsone poisoning; treatment with methylene blue and exchange transfusion.The authors present a case of methaemoglobinaemia of acute onset, with an unusually protracted course. The long persistence of this disorder led to a search for the cause which was eventually traced to medication with dapsone. The latter was found to be inappropriately being taken by the patient instead of an antispasmodic that had been prescribed for a spinal condition; this was because the tablets had been incorrectly labelled and dispensed in a pharmacy. The patient took increasing doses of the presumed 'antispasmodic' tablets as they seemed to lack clinical effect, thus further exacerbating the toxic consequences. Moreover, the patient brought his wrongly labelled tablets into hospital and was allowed to use them there, contrary to normal hospital policy. As treatment for the methaemoglobinaemia both bolus and continuous infusions of methylene blue were used, which probably contributed to the severe haemolysis which followed. Furthermore, the development of a rare side effect of dapsone toxicity, namely that of a sensorimotor neuropathy, is reported.- - - - - - - - - - ranking = 0.071846355685906keywords = motor (Clic here for more details about this article) |
7/204. An unusual manifestation of diabetes mellitus.MEDICAL history: Type 2 diabetes mellitus for five years; unexplained 35-lb weight loss three years ago; Bell's palsy on right side many years ago. MEDICATIONS: glipizide, 10 mg/day. family history: Father died of leukemia at age 65; mother has kidney stones; no diabetes or neuromuscular disease. SOCIAL history: insurance salesman; heterosexual, promiscuous, uses condoms; smokes (25 pack years); does not drink. physical examination: Well-nourished, well developed, not in acute distress; had difficulty rising from a sitting position because of right lower extremity weakness. blood pressure, 154/74; pulse, 88; temperature, 36.6 degrees C; respiratory rate, 16. head, eyes, ears, nose, and throat: normal. neck: normal. heart: S4. Lungs: clear. abdomen: mildly obese. extremities: no cyanosis, clubbing, or edema; atrophy and weakness of right thigh and both calves; wide-based gait; able to walk on toes but not heels. Neurologic responses: cranial nerves intact; deep tendon reflexes, 1 symmetrically; plantar reflexes, flexor bilaterally. skin: macular rash in sun-exposed areas. LABORATORY FINDINGS: Hemoglobin, 13.2 gm/dL; mean corpuscular volume, 80 micron 3; white blood cell count, 7,200/mm3 (normal differential); platelet count, 137,000/mm3. serum: electrolytes, normal; blood urea nitrogen, 18 mg/dL; creatinine, 0.8 mg/dL; glucose, 308 mg/dL; total protein, albumin, liver enzymes, and creatine kinase, normal. urine: 1 glucose. Venereal disease test: nonreactive; hiv test: negative. DIFFERENTIAL diagnosis: dermatomyositis; heavy-metal poisoning; diabetic amyotrophy. HOSPITAL COURSE: The patient was given 50 mg/day of oral amitriptyline to alleviate the painful paresthesias and was switched to 20 U/day of subcutaneously injected neutral protamine Hagedorn (NPH) insulin to normalize the blood glucose level. Histologic studies of skin and muscle showed sun damage and neuropathic changes, respectively. There was no evidence of vasculitis. Screening for heavy-metal toxins produced negative results.- - - - - - - - - - ranking = 0.000191527212737keywords = lateral (Clic here for more details about this article) |
8/204. Distal neuralgic amyotrophy.Neuralgic amyotrophy consists of severe pain around the shoulder and arm followed by weakness in one or several muscles of the same area. We describe four patients with distal neuralgic amyotrophy in whom acute, severe, and transient pain around the shoulder or arm was followed by weakness of the forearm and hand muscles only. Minor sensory symptoms were present in only one patient. The presence of structural lesions causing the extent of the forearm and hand motor deficit was excluded by ancillary examinations. Electrophysiological studies showed a motor axonopathy and minimal sensory axonopathy. A follow-up of 2 years or longer showed either spontaneous improvement or residual motor deficit. Unfamiliarity with a clinically distal localization of neuralgic amyotrophy may result in misdiagnosis of lower cervical (poly)radiculopathy in view of the distal localization of the motor deficit and the high prevalence of coincidental abnormalities of the lower cervical spine on plain radiography, computed tomography, or magnetic resonance imaging.- - - - - - - - - - ranking = 0.28738542274362keywords = motor (Clic here for more details about this article) |
9/204. adult-onset nemaline myopathy: Another cause of dropped head.A 59-year-old man with severe neck extensor weakness had findings diagnostic of nemaline myopathy on muscle biopsy. review of the literature shows that dropped head occurs in nearly half of the patients with adult-onset nemaline myopathy. Other leading causes of dropped head syndrome are amyotrophic lateral sclerosis, myasthenia gravis, and isolated neck extensor myopathy.- - - - - - - - - - ranking = 0.13995432287915keywords = lateral sclerosis, sclerosis, lateral (Clic here for more details about this article) |
10/204. Cervical cord tethering mimicking focal muscular atrophy.spinal cord tethering rarely occurs in the cervical region. In adults, it usually results from previous operations. However, congenital origin is always diagnosed and treated early in the infant period. We report a 12-year-old boy with cervical spinal dysraphism which was erroneously diagnosed as focal muscular atrophy, a benign form of motor neuron disease. The patient was brought to our hospital because of rapid deterioration of symptoms. Careful evaluation disclosed a hairy dimple at the nuchal area, which led to the correct diagnosis. X-ray of the cervical spine showed spina bifida from C(4) to C(6) levels and fusion of the laminae of C(4) and C(5). spine MRI studies disclosed that the cervical cord was tethered caudally and dorsally, and the ventral nerve roots were markedly stretched, especially over the left side. Surgical intervention was undertaken and the patient's muscle power improved after untethering. The purpose of this report is to acquaint the reader with a surgically treatable condition that may appear to be benign focal amyotrophy. skin lesion at the nuchal area should be carefully looked for.- - - - - - - - - - ranking = 3.6201016983368keywords = motor neuron disease, neuron disease, motor neuron, motor, neuron (Clic here for more details about this article) |
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