1/194. Rigid spine syndrome. Case report. We describe a patient who had difficulty in walking since toddling stage and presented proximal upper and lower member weakness which have evolved to a progressive limitation of neck and trunk flexure, compatible with rigid spine syndrome. The serum muscle enzymes were somewhat elevated and the electromyography showed a myopatic change. The muscle biopsy demonstrated an active and chronic myopathy. The dna analysis through PCR did not display any abnormality for dystrophin gene. The dystrophin by immunofluorescence was present in all fibers, but some interruptions were found in the plasma membrane giving it the appearance of a rosary. The test for merosin was normal. ( info) |
2/194. Osmotic demyelination syndrome with two-phase movement disorders: case report. Osmotic demyelination syndrome (ODS) is characterized by regions of demyelination throughout the brain, which are most prominent in the pons. This demyelinating disease is associated with electrolyte disturbances and typically occurs in patients who are alcoholic or malnourished. movement disorders are not frequently recognized in patients with ODS. This report describes a 22-year-old woman with ODS after correction of profound hyponatremia. The main neurologic symptom was two-phase movement disorder. First, she had acute onset dystonia, then the movement disorder transformed to generalized rigidity and tremors in the delayed second phase. magnetic resonance imaging in the first phase revealed demyelinating lesions in the central pons, bilateral thalami and basal ganglia. In the second phase, the previous myelinolysis had been partially resolved. The clinical course of the two-phase movement disorder did not correlate with the resolving feature of neuroradiologic findings. During the second-phase movement disorder, the patient had a good response to propranolol and trihexyphenidyl. ( info) |
3/194. Spinal arachnoid cyst with weakness in the limbs and abdominal pain. A 7-year-old male admitted with neck rigidity, severe pain in the abdomen, and progressive weakness in the lower limbs was diagnosed as having a spinal intramedullary arachnoid cyst. There was a dramatic and immediate recovery after fenestration of the cyst. ( info) |
4/194. masseter muscle rigidity after vecuronium. masseter muscle rigidity after suxamethonium, usually occurring in children induced with halothane, is associated with malignant hyperthermia. A case is reported in which masseter muscle rigidity occurred in an adult following vecuronium. From the limited data available, this and two similar reported cases, it appears that non-depolarizing muscle relaxants can, very rarely, cause masseter muscle rigidity in adults. This masseter muscle rigidity may complicate airway management, but is unlikely to progress to generalized rigidity and malignant hyperthermia. ( info) |
5/194. 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. ( info) |
6/194. Improvement of depression and parkinsonism during ECT: a case study. Improvement in signs of parkinsonism and symptoms of depression was observed in a patient with Parkinson's disease who underwent a course of ECT for depression. Empirically this patient was observed by a blind rater to show a pattern of improvement in parkinsonian signs similar to that observed in parkinsonian patients treated with L-dopa. The time course of improvement of this patient's depression was also seen to parallel improvement in his Parkinson's disease. These results are consistent with the hypothesis that ECT increases catecholamine synthesis and more specifically would be evidence that ECT improves depression by increasing norepinephrine synthesis. ( info) |
7/194. A veteran with acute mental changes years after combat. A 49-year-old man presented with a five-week history of worsening confusion, agitation, and bizarre behavior. According to his mother and sister, who live with him, he had inexplicably jumped out of bed one day and complained of injuring his back. The pain apparently resolved within several days. Two weeks later, again just after awakening, he had experienced a period of confusion, lasting about 15 min. The latest episode occurred three days previously and included vivid hallucinations--at various times, he seemed to believe that he was talking to his brother on the telephone, drinking a glass of water, emptying the refrigerator, jumping into a foxhole, and stomping on rattlesnakes. He was disoriented to time as well as environment. ( info) |
8/194. Primary brainstem injury: benign course and improved survival. Primary brainstem injury following head injury is a rare event. The victims often have features of supratentorial injury, and a primary isolated injury to the brainstem occurring due to shearing stresses or to injury from the tentorial edge is extremely rare. In the presence of supratentorial injury, these patients may have altered sensorium. Isolated brainstem injury may manifest itself as internuclear ophthalmoplegia, anisocoria, rigidity and cerebellar tremor. Such injuries are now being diagnosed more often due to improved imaging techniques. We treated nine such cases who had sustained primary brainstem injury in road traffic accidents, all but one of whom were subsequently independent. Primary brainstem injuries need not be associated with poor prognosis and mortality and may run a benign course with good quality of survival. ( info) |
9/194. Rigid spine syndrome. Two case-reports. Rigid spine syndrome is characterized by massive spinal rigidity, usually most marked in the cervical region. Stiffness of the peripheral joints is sometimes present. We report two cases. Patient 1 was a 12-year-old boy diagnosed at three years of age with Duchenne's muscular dystrophy because of delayed onset of walking. contracture of the Achilles tendons, flexion contracture of the elbows, and loss of motion of the cervical spine were the main findings during the current evaluation. Radiographs of the affected joints were normal. An electrocardiogram showed an incomplete left bundle branch block. Muscle enzyme activities were moderately elevated. A myopathic pattern was seen on the electromyogram. A muscle biopsy showed muscle fiber atrophy with peri- and endomysial fibrosis. Patient 2 was a 39-year-old man with a five-year history of isolated rigidity of the cervical spine thought to be due to a spondylarthropathy. Extension was the only movement possible at the cervical spine. The peripheral joints showed no motion range limitation. Findings were normal from radiographs of the spine and sacroiliac joints, an erythrocyte sedimentation rate determination, an electromyogram, and muscle enzyme activity assays. A muscle biopsy showed muscle fiber atrophy with peri- and endomysial fibrosis. DISCUSSION: Rigid spine syndrome is rare in rheumatological practice and can simulate a number of other muscle and joint diseases. Peri- and endomysial fibrosis may be strongly suggestive, although nonpathognomonic. Involvement of the heart governs the prognosis. ( info) |
10/194. The dropped head sign in parkinsonism. We describe seven patients who exhibited the dropped head sign in parkinsonism. These included six females and one male between the ages of 53 and 74. Three patients were clinically diagnosed as probable Parkinson's disease and four were diagnosed with probable multiple system atrophy. None had weakness in the posterior neck muscles or spasms in the anterior neck muscles. When the patients attempted to extend the head voluntarily or passively muscle contraction that was not seen in the dropped-head condition appeared. Surface electromyography of the neck indicated that the anterior neck muscles had rigidity. A gamma-block of the SCM muscles reduced the muscle activity when the head was elevated and improved the dropped-head condition slightly. These findings seem to indicate that the dropped head sign in parkinsonism could be associated with anterior neck muscle rigidity. Although the severity of the dropped head condition was affected by medication or by the clinical course in three patients, there was no clear relationship between the severity of the dropped head condition and the parkinsonism. We suspected that unbalanced muscle rigidity between the anterior and the posterior neck muscles could cause the dropped head sign. ( info) |