1/359. Acute sensorimotor polyneuropathy with tonic pupils and an abduction deficit: an unusual presentation of polyarteritis nodosa.A tonic pupil may occur in isolation or as part of a systemic disorder. We report a patient who developed tonic pupils and an abduction deficit in the setting of polyarteritis nodosa. The combination of a tonic pupil and an abduction deficit should suggest the possibility of a vasculopathic disorder, because the ciliary ganglion and lateral rectus muscle are both supplied by the lateral muscular artery. Widespread small artery and arteriolar narrowing and occlusion are the hallmarks of polyarteritis nodosa. Unusual ischemic syndromes may occur, such as this rare combination of neuro-ophthalmic signs, by involvement of both the nutrient artery and its collaterals. We are unaware of other reports of neuropathic tonic pupils in association with polyarteritis nodosa.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/359. peroneal nerve palsy caused by intraneural ganglion.A case of peroneal nerve palsy caused by an intraneural ganglion is presented. The cystic mass was located posterolateral to the lateral femoral condyle and extended along the common peroneal nerve distal to the origin of the peroneus longus muscle. The nerve was compressed in the narrow fibro-osseous tunnel against the fibula neck and the tight origin of the peroneus longus muscle. The nerve was decompressed by complete tumor excision and transection of the origin of the peroneus longus muscle. Full recovery of nerve function was obtained in 6 months.- - - - - - - - - - ranking = 3keywords = muscle (Clic here for more details about this article) |
3/359. occupational exposure to methyl methacrylate monomer induces generalised neuropathy in a dental technician.A 36-year-old dental technician for 14 years developed paraesthesia and numbness in her legs. Neurophysiological studies revealed absent sensory nerve action potentials (SNAPs) from her lower limbs and normal upper limb SNAPs on presentation. Motor nerve studies were normal. Repeat studies 2 months after leaving her job showed some improvement in the lower limb SNAPs. It is suggested that her symptoms were caused by occupational exposure to methyl methacrylate monomer.- - - - - - - - - - ranking = 0.50493706350974keywords = limb (Clic here for more details about this article) |
4/359. hypertrophy and pseudohypertrophy of the lower leg following chronic radiculopathy and neuropathy: imaging findings in two patients.Enlargement of the ipsilateral muscle compartment is an exceptional finding in patients with chronic radiculopathy, peripheral nerve injury, anterior horn cell diseases, or acquired peripheral neuropathy. We report radiographic, ultrasonographic, CT and MRI findings in a patient with chronic S1 radiculopathy and another with chronic neuropathy of the common fibular nerve (L4-S2), both presenting with painless enlargement of the calf muscles.- - - - - - - - - - ranking = 2keywords = muscle (Clic here for more details about this article) |
5/359. Congenital muscular dystrophy with central and peripheral nervous system involvement in a Belgian patient.We report a patient with congenital muscular dystrophy (CMD), developmental brain defects, and peripheral neuropathy. Marked hypotonia and plagiocephaly were noted at birth. failure to thrive, generalized muscle weakness and wasting, absent deep tendon reflexes, partial seizures, and secondary microcephaly developed. brain MRI showed a large area of cortical dysplasia, a thin but complete corpus callosum, and diffuse ventriculomegaly. Nerve conduction velocities were slow and creatine kinase levels only mildly elevated. Muscle biopsy showed dystrophic features with normal merosin, sarcoglycan, and dystrophin immunostaining. The Japanese Fukuyama CMD founder mutation was not detected. This is the first report of a patient with merosin-positive CMD, cobblestone lissencephaly, and demyelinating peripheral neuropathy.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
6/359. Laryngeal electromyographic findings in charcot-marie-tooth disease type II.charcot-marie-tooth disease is a hereditary motor and sensory neuropathy that exhibits progressive muscular atrophy in the limbs, beginning with the lower extremities. It is now understood to be a heterogeneous group of disorders that can be differentiated both clinically and genetically. In charcot-marie-tooth disease type II C, axonal neuropathy, diaphragm weakness, and vocal cord paralysis are described within kindreds. We used laryngeal electromyography to study a patient with this disorder. This technique has potential in the diagnosis of charcot-marie-tooth disease type II.- - - - - - - - - - ranking = 0.16831235450325keywords = limb (Clic here for more details about this article) |
7/359. Idiopathic small-fiber sensory neuropathy in childhood: A diagnosis based on objective findings on punch skin biopsy specimens.Idiopathic small-fiber sensory neuropathy (SFSN) has not previously been reported in children. Although affected patients complain of neuropathic pain, this condition is often difficult to diagnose because of the few objective physical signs and normal nerve conduction studies. We report a girl with idiopathic SFSN in whom the results of a sural nerve biopsy were normal, but punch skin biopsy revealed reduced intraepidermal nerve fiber density and established the diagnosis. Idiopathic SFSN should be considered in the differential diagnosis of children who have burning limb pain with no routine electrophysiologic or pathologic abnormalities.- - - - - - - - - - ranking = 0.16831235450325keywords = limb (Clic here for more details about this article) |
8/359. Neurological manifestations of chronic hepatitis c.hepatitis c virus (HCV) infection is often associated with abnormal immunological responses. We describe four patients with vasculitic neurological signs and symptoms following HCV infection. A 56-year-old woman with HCV infection developed peripheral neuropathy characterized by asymmetric distal painful hypesthesia, dysesthesia and moderate motor weakness of the lower limbs. Serological examinations revealed cryoglobulinemia and low levels of complement c4. A biopsy of the sural nerve revealed vasculitic neuropathy. HCV infection associated immunomediated vasculitis was diagnosed. While steroid therapy was ineffective, treatment with interferon-alpha improved the neuropathy considerably without, however, eliminating HCV infection. A 62-year-old man with HCV infection developed peripheral sensory neuropathy. complement c3 was slightly diminished. Nerve biopsy revealed vasculitic neuropathy. A 71-year-old woman developed chronic symmetric sensomotor polyneuropathy. HCV hepatitis followed blood transfusions. cryoglobulins tested positive, consistent with type II cryoglobulinemia. Complements C3 and C4 were diminished. Inflammatory infiltrates in the sural nerve biopsy specimen led to the diagnosis of chronic vasculitic disorder. A 55-year-old woman with HCV infection developed vasculitis of the skin, connective tissue, visceral organs, and kidney, leading to hemodialysis. Neurologically she developed severe apathy and drowsiness, myoclonic jerks, exaggerated deep tendon reflexes, and positive pyramidal signs. magnetic resonance imaging of the brain showed diffuse increased signal abnormalities involving supra- and infratentorial white matter suggesting cerebral vasculitis. cryoglobulins were positive, complements C3 and C4 slightly diminished (54 mg/dl, 4.3 mg/dl). Supportive therapy resulted in neurological improvement. Treatment with interferon-alpha was discontinued because of agranulocytosis. In patients with peripheral neuropathy or signs of leucencephalopathy, a hepatitis c associated vasculitis should be considered in the differential diagnosis.- - - - - - - - - - ranking = 0.16831235450325keywords = limb (Clic here for more details about this article) |
9/359. Bilateral lumbosacral plexopathy after mesenteric thrombosis.OBJECTIVE: A case of lumbosacral plexopathy (LSP) following operation for mesenteric thrombosis. DESIGN: Case report of a 64-year-old man who developed weakness and numbness of the distal legs after an operation for mesenteric thrombosis. SETTING: Department of Physical medicine and rehabilitation, University Hospital La Fe, Valencia, spain. SUBJECT: Single patient case report. MAIN OUTCOME MEASURE: Clinical and electromyography follow-up of the patient between October 1996 and August 1997. RESULTS: physical examination revealed marked lower extremity weakness, hypotonia, hyporreflexia and normal bowel and bladder function. electromyography demonstrated marked denervation of all major muscle groups, and sensory nerve conduction showed absence of responses in all peripheral nerves, in both legs. CONCLUSION: To our knowledge, bilateral LSP following an intervention of mesenteric thrombosis, has never been reported in the literature. diagnosis of LSP might be based on electromyography and nerve conduction studies that demonstrate electrodiagnostic criteria for LSP, including denervation in muscles innervated by at least two lumbosacral segmental levels and involving at least two different peripheral nerves, without paraspinal involvement.- - - - - - - - - - ranking = 2keywords = muscle (Clic here for more details about this article) |
10/359. Restoring hand function in patients with severe polyneuropathy: the role of electromyography before tendon transfer surgery.electromyography (EMG) was evaluated as a supplement to clinical examination and biomechanical considerations to optimize forearm donor muscle selection before tendon transfers to 4 functionless hands in 3 patients with slowly progressive polyneuropathies. Two patients had unusually severe charcot-marie-tooth disease; the third patient had idiopathic mononeuropathy multiplex. Standard EMG parameters were used to devise an intuitive muscle grading system, including most importantly interference patterns and motor control, plus motor unit morphology and stability. Given our objective of restoring survivable function despite ongoing polyneuropathy, we found that EMG reveals prognostically important differences among partially denervated candidate muscles that cannot be detected by experienced clinical examiners. Opposition transfer was performed on one hand of each patient. After 39-, 39-, and 51-month follow-up durations, restored opposition was graded as good in these 3 hands. We conclude that EMG provides meaningful guidance in selecting optimal forearm muscles for tendon transfers to hands in the setting of slowly progressive polyneuropathies.- - - - - - - - - - ranking = 4keywords = muscle (Clic here for more details about this article) |
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