1/17. Neuromyotonia: an unusual presentation of intrathoracic malignancy.A 48 year old woman is described who presented with increasing muscular rigidity and who was found to have a mediastinal tumour. Electrophysiological studies revealed that the muscular stiffness resulted from very high frequency motor unit activity which outlasted voluntary effort, and which was abolished by nerve block. The abnormal activity may have arisen at the anterior horn cell level. Marked improvement followed the administration of diphenylhydantoin.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/17. Progressive encephalomyelitis with rigidity: a paraneoplastic presentation of oat cell carcinoma of the lung. Case report.Progressive encephalomyelitis with rigidity and myoclonus (PEWR) is a rare neurological disorder, characterised by muscular rigidity, painful spasms, myoclonus, and evidence of brain stem and spinal cord involvement. A 73-year-old white man was admitted with a 10-day history of painful muscle spasms and continuous muscle rigidity on his left lower limb. He had involuntary spasms on his legs and developed encephalopathy with cranial nerves signs and long tract spinal cord symptomatology. Brain CT scan and spinal MRI were normal. The CSF showed lymphocytic pleocytosis and no other abnormalities. EMG showed involuntary muscle activity with 2-6 seconds of duration, interval of 30-50 ms and a frequency of 2/second in the left lower limb. Anti-GAD antibodies were detected in the blood. We detected radiological signs of lung cancer during the follow-up, which proved to be an oat cell carcinoma. The patient died two weeks after the diagnosis of the cancer.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
3/17. stiff-person syndrome: an autoimmune disease.stiff-person syndrome (SPS) is characterized by progressive, usually symmetric rigidity of the axial muscles with superimposed painful spasms precipitated by tactile stimuli, passive stretch, volitional movement of affected or unaffected muscles, startling noises, and emotional stimuli. electromyography demonstrates continuous normal motor unit potentials in the affected muscles. Both the rigidity and the spasms are relieved by sleep, general anesthesia, myoneural blockade, peripheral nerve blockade, and partially by diazepam. Evidence for an autoimmune etiology of SPS includes its association with other autoimmune diseases and autoantibodies and the presence of antibodies against glutamic acid decarboxylase (GAD) in the cerebrospinal fluid (CSF) of many affected patients. We describe two patients with this syndrome who had GAD antibodies in both CSF and serum. Partial relief of the symptoms in these patients by corticosteroid therapy provides additional evidence of an autoimmune etiology of SPS and of the role of immunotherapy in its treatment.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/17. Familial autosomal recessive rigid spine syndrome with neurogenic facio-scapulo-peroneal muscle atrophy.Two sisters and a first cousin presented with rigid spine and facio-scapulo-peroneal muscle atrophy. The patients belonged to a family with two first-cousin marriages. electromyography, muscle and nerve biopsy showed neurogenic muscle atrophy without peripheral nerve involvement. Follow up did not show progression of the disease. This is the first observation of an association of neurogenic facio-scapulo-peroneal and rigid spine syndrome. The double first-cousin marriage suggests autosomal recessive inheritance.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
5/17. An autopsy case of peroneal muscular atrophy with rigidity and tremor. Ultrastructural and systematic morphometrical studies on peripheral nerves.An autopsy case of hereditary peroneal muscular atrophy (PMA) with rigidity and static tremor is presented. The patient developed slowly progressive distal muscular atrophy of the legs at the age of 15 years. By the age of 52 years, PMA became marked associated with pes cavus, and tremor and rigidity of the extremities were noted. Motor and sensory conduction velocities gradually depressed and lost near the end of his life. At autopsy, the major neuropathological abnormalities involved the peripheral nervous systems, and were characterized by axonal atrophy and loss of myelinated fibers. These changes involved both the proximal and distal nerves, being more severely affected in the distal. The pathological changes in other regions of the nervous systems were mainly confined to the spinal cord, dorsal ganglia and spinal nerve roots, and pigmented neurons in the brain stem. Morphometrically, the total fascicular area was much smaller than in control, but the total number of myelinated fibers greatly outnumbered that of control 75,200 to 48,200 at the proximal sciatic nerve and then gradually decreased towards the periphery; however, even in the distal sural nerve, the total number of myelinated fibers exceeded that of control (6820 to 5469). Thus, the density of myelinated fibers were much higher, being 1.5 to 2 times greater, than in control. Its abrupt decline at the distal nerve might account for neurogenic atrophy of the distal musculature. Unmyelinated fibers were slightly increased in density and not atrophic. This case is unique in its clinicopathology and does not belong to any subtypes of PMA including "neuronal plus".- - - - - - - - - - ranking = 9keywords = nerve (Clic here for more details about this article) |
6/17. Autoimmune myastenia gravis with thymoma following the spontaneous remission of stiff-man syndrome.A patient who developed generalized autoimmune myasthenia gravis six years after the spontaneous remission of a stiff-man syndrome is described. He also suffered from chronic active hepatitis and had radiological evidence of a thymoma. He did not have diabetes mellitus. Besides anti-nicotinic acetylcholine receptor antibodies, anti-nuclear, anti-dna, anti-mitochondrial and anti-skeletal muscle antibodies were found in his serum, while islet-cell antibodies were absent. Immunocytochemistry studies failed to demonstrate autoantibodies to GABA-ergic nerve terminals, although an aspecific neuronal immunostaining was observed. The clinical and immunological features of this case support the hypothesis of a dysimmune pathogenesis of SMS, also in cases not associated with autoimmunity to GABA-ergic nerve terminals. Furthermore, a relationship between thymoma and the neurological syndromes discussed could be considered.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
7/17. A new syndrome of axial muscle rigidity associated with etretinate therapy.We report on three cases of a novel syndrome associated with treatment for psoriasis with the retinoid etretinate. The distinctive features of this syndrome include rigidity that is restricted to the axial muscles, impairment of neck extension greater than flexion, impairment of anteflexion at the waist, severe impairment of lateral flexion at both the waist and the neck, and, in one case, rigidity of proximal lower extremity muscles. Peripheral nerve block extinguished the lower extremity rigidity in one patient, and two patients had a favorable response to Sinemet. One patient had persistence of symptoms and findings 2 years after discontinuation of etretinate. The clinical features of this syndrome are consistent with an effect of etretinate on central, possible catecholaminergic neurons.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/17. Stiff-man syndrome with spinal cord lesion.An autopsy case of stiff-man syndrome is presented. The patient was a 54-year-old man who had suffered from fluctuating rigidity and tonic spasm of the trunk and leg muscles for three years. These symptoms were induced by emotional stress, strain at voiding and other external stimuli. physical examination revealed stiffness of the neck, trunk and legs without obvious muscle weakness or atrophy. Deep tendon reflexes were hyperactive, while sensation was normal. EMG showed the stiffened muscles to have continuous motor unit activity which was decreased by myoneural block, spinal anesthesia, diazepam or sleep. Administration of diazepam (60 mg daily) gave some relief of the stiffness for several days. However, respiratory arrest suddenly occurred. Although the patient was resuscitated, he died nine days after this episode. Examination of the spinal cord revealed loss and degeneration of nerve cells with marked gliosis in the medial motor nuclei of the anterior horns. A symmetric degeneration in the bilateral anterior columns which has never been described as a spinal cord lesion in the stiff-man syndrome was also seen.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
9/17. Motor neuron rigidity. An electrophysiological, pharmacological and pathological study.A 28-year-old male with generalized muscle stiffness and widespread muscle twitching is described. Continuous electrical activity was present at rest and could be abolished only by succinylcholine, curare, and block of the distal part of the peripheral nerve. Muscle biopsy revealed mild myopathic changes. In addition glycogen depletion was found in muscle fibres which were type IIb in one region of the biopsy and type I in another. With carbamazepine treatment marked improvement occurred both clinically and electromyographically. It is concluded that the abnormal electrical activity originated in the terminal branches of the peripheral motor nerve and that this activity was confined to muscle fibres belonging to single motor unit territories.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
10/17. Huntington's disease: survival of large striatal neurons in the rigid variant.A morphometric method was employed to investigate the relationship of rigidity and hyperkinesia to the degree of striatal and nigral nerve cell loss in one patient with the rigid variant of Huntington's disease and four patients with hyperkinetic chorea. Both striatal neuron populations, small and medium-sized neurons and large neurons, were affected in the patients with hyperkinetic chorea, whereas the large neurons were preserved in the patient with the rigid variant. The substantia nigra was slightly involved in each patient. The findings suggest that the rigid variant of Huntington's disease may reflect the unbalanced activity of residual, mostly large, striatal neurons, inhibiting the substantia nigra.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
| | Next -> |