1/111. MR imaging of Dejerine-Sottas disease.We report the MR findings in two patients with clinically and histologically proved Dejerine-Sottas disease. One patient had spinal involvement with multiple thickened and clumped nerve roots of the cauda equina; the second had multiple enlarged and enhancing cranial nerves. Although these findings are not specific for Dejerine-Sottas disease, they are suggestive of the diagnosis, which is further corroborated with history and confirmed with sural nerve biopsy and laboratory studies.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/111. 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 = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
3/111. 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 = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
4/111. Myopathy, myasthenic syndrome, and epidermolysis bullosa simplex due to plectin deficiency.plectin, an intermediate filament linking protein, is normally associated with the sarcolemma, nuclear membrane, and intermyofibrillar network in muscle, and with hemisdesmosomes in skin. A 20-year-old female with epidermolysis bullosa simplex since birth had progressive ocular, facial, limb, and trunkal weakness and fatigability since age 9, fivefold CK elevation, a 25% decrement with myopathic motor unit potentials and increased electrical irritability on electromyography, and no anti-acetylcholine receptor (AChR) antibodies. plectin expression was absent in muscle and severe plectin deficiency was noted in skin. Morphologic studies revealed necrotic and regenerating fibers and a wide spectrum of ultrastructural abnormalities: large accumulations of heterochromatic and lobulated nuclei, rare apoptotic nuclei, numerous cytoplasmic and few intranuclear nemaline rods, disarrayed myofibrils, thick-filament loss, vacuolar change, and pathologic alterations in membranous organelles. Many endplates (EPs) had an abnormal configuration with chains of small regions over the fiber surface and a few displayed focal degeneration of the junctional folds. The EP AChR content was normal. in vitro electrophysiologic studies showed normal quantal release by nerve impulse, small miniature EP potentials, and fetal as well as adult AChR channels at the EP. Our findings support the notion that plectin is essential for the structural integrity of muscle and skin, and for normal neuromuscular transmission.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
5/111. electrodiagnosis in spinal cord injured persons with new weakness or sensory loss: central and peripheral etiologies.OBJECTIVE: To assess the prevalence and causes of late neurologic decline of persons with spinal cord injury (SCI). DESIGN: Retrospective review of persons with SCI over a 9-year period. Those with complaints of new weakness or sensory loss were grouped into three categories based on clinical examination, electrodiagnosis, and imaging: (1) central pathology (ie, brain, spinal cord, or nerve root); (2) peripheral pathology (plexus or peripheral nerve); or (3) no identifiable etiology. The specific diagnoses of late neurologic decline were identified. SETTING: Regional veterans Affairs spinal cord Injury Service. patients: Five hundred two inpatient and outpatient adults with SCI. RESULTS: Nineteen percent of the study population complained of new weakness and/or sensory loss. Neurologic abnormalities were noted in 13.5%, 7.2% with central and 6.4% with peripheral causes. The most common pathologies were posttraumatic syringomyelia (2.4%) and cervical (1.6%) and lumbosacral (1.2%) myelopathy/radiculopathy. A specific etiology was not determined in 6 cases (1.6%). Peripheral involvement was mostly from ulnar nerve entrapment (3.4%) and carpal tunnel syndrome (3.0%). CONCLUSIONS: Late-onset neurologic decline is common after SCI and can result from central or peripheral pathology. Regular neurologic monitoring of SCI patients is recommended, since many with neurologic decline respond favorably if diagnosed and treated early.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
6/111. Proximal diabetic neuropathy presenting with respiratory weakness.A patient is described with proximal diabetic neuropathy presenting with respiratory weakness. A 50 year old man developed progressive shortness of breath over 2 months. He also had weakness of hip flexion. phrenic nerve responses were absent, and spontaneous activity was seen in the intercostal and lumbar paraspinal muscles with long duration neurogenic MUPs and reduced recruitment in the diaphragm. Without treatment, the patient began to improve with resolution of his proximal leg weakness and breathing difficulties. Proximal diabetic neuropathy is another cause of neuromuscular respiratory weakness.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
7/111. Extensor pollicis longus paralysis following thoracoscopic sympathectomy.Thoracoscopic sympathectomy is an acceptable form of treatment for palmar hyperhidrosis. Many authors have reported favourable results. Complications range from pneumo-haemothorax, Horner's syndrome, compensatory hyperhidrosis and bleeding. Plas et al reported 2.7% of the procedures had complications requiring intervention and 9.7% had non-interventive complications. There have been isolated reports of other rare complications including false aneurysm of intercostal artery, inferior brachial plexus injury and abnormal suntanning. We report an unusual case of isolated extensor pollicis longus paralysis after a thoracoscopic sympathectomy for palmar hyperhidrosis, in a fit young male. Such complications have not been previously reported. We recognise that such isolated nerve injury is uncommon.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
8/111. Ipsilateral facial weakness in upper medullary infarction-supranuclear or infranuclear origin?We describe two patients with upper medullary infarctions showing ipsilateral facial weakness and relative sparing of the upper facial muscles. Electrophysiological follow-up using transcranial magnetic stimulation of the motor cortex in combination with stimulation of the peripheral facial nerve disclosed a supranuclear (corticofacial) tract lesion in one patient and a partial nuclear/infranuclear intra-axial facial nerve lesion in another.- - - - - - - - - - ranking = 0.66666666666667keywords = nerve (Clic here for more details about this article) |
9/111. Distinguishing clinical and electrodiagnostic features of X-linked bulbospinal neuronopathy.X-linked bulbospinal neuronopathy (XLBSN) or Kennedys disease is a rare inherited neuromuscular disease characterized by adult-onset muscle weakness, usually in a limb-girdle distribution. It is frequently misdiagnosed despite a distinctive clinical presentation, usually due to the absence of a clear family history, and perhaps also due to failure of recognition. Accurate diagnosis is crucial for genetic counseling purposes and because alternative diagnoses usually carry a poorer prognosis. We evaluated 4 patients with XLBSN and one symptomatic female heterozygote patient. Based on our clinical observations in these patients and a systematic review of previously reported cases, the following clinical and electrophysiologic features when present in the setting of adult-onset muscle weakness, are strongly suggestive of the disorder: 1) facial weakness, 2) facial twitching or fasciculations, 3) tongue weakness and atrophy, 4) postural hand tremor, 5) hypo- or areflexia, and 6) absent or low-amplitude sensory nerve action potentials despite clinically normal sensation. We also hypothesize regarding the possibility of partial expression of the abnormal XLBSN gene in a symptomatic heterozygote female patient.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
10/111. Adhesive capsulitis of the glenohumeral joint with an unusual neuropathic presentation: a case report.A 37-yr-old woman presented with a 7-mo history of unilateral shoulder girdle stiffness, pain, and weakness and had already been diagnosed with frozen shoulder. physical examination revealed scapular winging and suspicious focal paralysis of shoulder girdle muscles. Subsequently, electrodiagnostic studies reported denervation of deltoid, infraspinatus, serratus anterior, and lower cervical paraspinal muscles, in addition to a prolonged long thoracic nerve latency. The history, physical examination, and cervical magnetic resonance imaging scan seemed most consistent with neuralgic amyotrophy, although the electrodiagnostic examination could be interpreted as cervical radiculopathy. Some of the difficulties in identifying neuralgic amyotrophy and distinguishing it from cervical radiculopathy are discussed herein. Historically, frozen shoulder has seemed to develop as a complication of the neuropathic process. Both neuralgic amyotrophy and frozen shoulder have a poorly understood pathogenesis, and their combined presence is presumed to be rare. Because of difficulties inherent in the physical examination of frozen shoulder, a coexistent neuropathic process may go undetected.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
| | Next -> |