1/111. Wasting of the small hand muscles in upper and mid-cervical cord lesions.Four patients are described with destructive rheumatoid arthritis of the cervical spine and neurogenic wasting of forearm and hand muscles. The pathological connection is not immediately obvious, but a relationship between these two observations is described here with clinical, radiological, electrophysiological and necropsy findings. Compression of the anterior spinal artery at upper and mid-cervical levels is demonstrated to be the likely cause of changes lower in the spinal cord. These are shown to be due to the resulting ischaemia of the anterior part of the lower cervical spinal cord, with degeneration of the neurones innervating the forearm and hand muscles. These findings favour external compression of the anterior spinal artery leading to ischaemia in a watershed area as the likeliest explanation for this otherwise inappropriate and bizarre phenomenon.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/111. MRI of the spinal cord in myelopathy complicating vitamin B12 deficiency: two additional cases and a review of the literature.Focal spinal cord lesions have been present in all previously reported cases of MRI appearances in myelopathy complicating vitamin B12 deficiency. We describe two further cases showing mild atrophy only and review the salient features of the previous 11 publications. MRI findings reflect quite closely the known pathological changes in this condition.- - - - - - - - - - ranking = 10.748393612729keywords = atrophy (Clic here for more details about this article) |
3/111. ehlers-danlos syndrome associated with multiple spinal meningeal cysts--case report.A 40-year-old female with ehlers-danlos syndrome was admitted because of a large pelvic mass. Radiological examination revealed multiple spinal meningeal cysts. The first operation through a laminectomy revealed that the cysts originated from dilated dural sleeves containing nerve roots. Packing of dilated sleeves was inadequate. Finally the cysts were oversewed through a laparotomy. The cysts were reduced, but the postoperative course was complicated by poor wound healing and diffuse muscle atrophy. ehlers-danlos syndrome associated with spinal cysts may be best treated by endoscopic surgery.- - - - - - - - - - ranking = 10.915060279395keywords = atrophy, muscle (Clic here for more details about this article) |
4/111. spinal cord herniation into an extensive extradural meningeal cyst: postoperative analysis of intracystic flow by phase-contrast cine MRI.We report a patient with idiopathic spinal cord herniation who underwent postoperative myelography and phase-contrast cine magnetic resonance imaging (MRI) to clarify the condition of the ventral cystic lesion into which the spinal cord had been herniated. This 58-year-old man showed symptoms and signs compatible with brown-sequard syndrome. A myelogram and MRI showed that the spinal cord was incarcerated in a small space, and this finding led to the diagnosis of spinal cord herniation. In the operation, the herniated spinal cord was returned to the initial intradural space, and the dural defect was enlarged to prevent recurrence of the herniation. After the operation, leg muscle strength improved and bladder and bowel dysfunction resolved, but analgesia was unchanged. Postoperative myelography revealed a very large extradural cystic lesion, which extended from C2 to L1 vertebral level. A cerebrospinal fluid (CSF) flow study of the intradural space disclosed a near-normal pattern, but the intracystic CSF dynamics were abnormal. In the intracystic space at just the upper level of the lesion, cranial flow (reverse directional flow in comparison with the intradural flow) was seen in the systolic cycle after momentary fast caudal flow, and these CSF dynamics may have been related to the spinal cord herniation.- - - - - - - - - - ranking = 0.16666666666667keywords = muscle (Clic here for more details about this article) |
5/111. Progressive necrotic myelopathy: clinical course in 9 patients.OBJECTIVE: To review the clinical, laboratory, and radiological findings of 9 patients who had progressive idiopathic myelopathy with evidence of spinal cord necrosis. DESIGN AND methods: We reviewed personally examined cases of myelopathy that fulfilled the following criteria: (1) regional loss of reflexes, flaccidity, and muscle atrophy; (2) magnetic resonance imaging showing a shrunken or cavitated cord without evidence of arteriovenous malformation; (3) electromyogram showing denervation over several contiguous spinal cord sgements with preservation of sensory potentials in some cases; and (4) the absence of evidence of systemic disease or neoplasm. RESULTS: The illness began in these patients after the age of 40 years, with prominent burning or tingling limb pain, occasionally with radicular features or with less well-defined back, neck, or abdominal pain. Leg or infrequently arm weakness appeared concurrently or soon after the onset of pain. The most distinctive feature was a saltatory progression of symptoms, punctuated by both acute and subacute worsenings approximately every 3 to 9 months, culminating in paraplegia or tetraplegia. The distinguishing clinical findings, together indicative of destruction of gray matter elements of the cord, were limb atrophy, persistent areflexia, and flaccidity. The concentration of cerebrospinal fluid protein was typically elevated between 500 g/L and 1000 g/L, without oligoclonal bands, accompanied infrequently by pleocytosis. magnetic resonance imaging showed features suggesting cord necrosis, specifically swelling, T2-weighted hyperintensity, and gadolinium enhancement over several spinal cord segments, succeeded months later by atrophy in the same regions. necrosis of the cord was found in biopsy material from one patient and postmortem pathology in another case, but inflammation and blood vessel abnormalities were absent. Only 2 patients had prolonged visual evoked responses. The disease progressed despite immune-modulating treatments although several patients had brief epochs of limited improvement. CONCLUSIONS: The saltatory course, prolonged visual evoked responses in 2 patients, and a cranial abnormality on magnetic resonance imaging in another, raised the possibility of a link to multiple sclerosis. However, the normal cranial magnetic resonance imaging scans in 6 other patients, uniformly absent oligoclonal bands, and poor response to treatment were atypical for multiple sclerosis. On the basis of shared clinical and laboratory features, idiopathic progressive necrotic myelopathy is indistinguishable from a limited form of Devic disease.- - - - - - - - - - ranking = 32.411847504853keywords = atrophy, muscle (Clic here for more details about this article) |
6/111. Idiopathic spinal cord herniation: report of three cases and review of the literature.STUDY DESIGN: Three case reports and a literature review are presented. OBJECTIVE: To describe characteristic clinical and radiographic findings of idiopathic spinal cord herniation. SUMMARY OF BACKGROUND DATA: Idiopathic spinal cord herniation is a rare disease, with only 26 cases reported before the current study. methods: Three cases of idiopathic spinal cord herniation are reported, and previous reports on this subject are reviewed. RESULTS: The responsible regions were in the thoracic spine from T2 to T7. Symptoms were mainly unilateral muscle atrophy in the lower extremity and sensory disturbance below the thoracic level. These symptoms had been progressing gradually. magnetic resonance imaging demonstrated a unique feature: The spinal cord shifted anteriorly in a few segments. Computed tomographic myelogram showed another distinctive picture: There was no subarachnoid space anterior to the spinal cord. CONCLUSIONS: Because idiopathic spinal cord herniation is out of the concept of "compression myelopathy," this condition may be a pitfall in the diagnosis. Idiopathic spinal cord herniation should be recognized as one of the treatable causes for thoracic myelopathy.- - - - - - - - - - ranking = 10.915060279395keywords = atrophy, muscle (Clic here for more details about this article) |
7/111. An unusual form of spinal muscular atrophy with mental retardation occurring in an inbred population.Three sibs are described suffering from hereditary non-progressive spinal muscular atrophy with non-progressive mental retardation. One of them had in addition signs of pyramidal tract involvement. Muscular weakness was more pronounced proximally than distally and the neck muscles were severely involved. Th.ey all had small skulls and several associated congenital malformations were observed including syndactyly of the left hand in 1 patient. The patients belong to a small inbred community in the netherlands. Erythropoietic protoporphyria was also present in the family but segregated independently. This combination of "congenital" mental retardation with "congenital" non-progressive spinal muscular atrophy is believed to represent a new syndrome, caused by a rare recessive gene.- - - - - - - - - - ranking = 5015.4374617317keywords = muscular atrophy, atrophy, muscle (Clic here for more details about this article) |
8/111. tuberculoma of the conus medullaris: case report.OBJECTIVE AND IMPORTANCE: Intramedullary spinal tuberculoma is a rare form of central nervous system tuberculosis. This article describes an affected patient who presented with left leg paresis. CLINICAL PRESENTATION: A 46-year-old man presented with a 7-day history of left leg weakness. The patient's medical history included infection with pulmonary tuberculosis 15 years previously, at which time he had been treated with antituberculosis therapy. The neurological examination performed at admission revealed left leg paresis with Grade 2/5 power in all muscle groups. The patient reported no urinary or bowel problems. INTERVENTION: Surgery was performed with the patient in the prone position. The procedure involved laminectomies at T11, T12, and L1, followed by a midline myelotomy. The mass was excised completely. Histopathological examination revealed a granulomatous lesion that contained Langhans' giant cells, inflammatory cells, and evidence of caseating necrosis. The patient was prescribed a 6-month course of antituberculosis therapy with pyrazinamide, isoniazid, and rifampin. CONCLUSION: The outcome was favorable. Recently, a number of authors have reported success with medical management of intraspinal tuberculoma. Intraspinal tuberculoma produces a mass effect that can jeopardize spinal cord function. The optimal treatment is a combination of microsurgical resection and antituberculosis chemotherapy.- - - - - - - - - - ranking = 0.16666666666667keywords = muscle (Clic here for more details about this article) |
9/111. Sudden onset of cervical spondylotic myelopathy during sleep: a case report.Cervical spondylotic myelopathy is a common cause of compressive spinal cord dysfunction. The typical course involves either a gradual or an episodic increase in symptoms and neurologic deficits, with impairment evolving over a period of months to years. Acute neurologic deterioration in conjunction with cervical spondylosis has been described almost exclusively in traumatic situations such as disk herniation. We report a case of an acute, nontraumatic onset of tetraplegia in association with cervical spondylosis. A 56-year-old man developed tetraplegia during a 1-hour nap, with loss of volitional control of his extremities, impaired sensation below the C3 dermatome, and increased muscle tone. magnetic resonance imaging of the cervical spine revealed canal stenosis and increased T2 signal within the cord. This case report describes the rehabilitation course for this patient and reviews the clinical spectrum of onset and progression of cervical spondylotic myelopathy.- - - - - - - - - - ranking = 0.16666666666667keywords = muscle (Clic here for more details about this article) |
10/111. Progressive spinal muscular atrophy in a case of hypokalamic periodic paralysis.A 47-year-old man with well-documented hypokalemic periodic paralysis demons-rated permanent myopathy as shown by biopsy. In addition there was clinical and biopsy evidence of progressive spinal muscular atrophy. The feature of the combination of these diseases is reported in a case and discussed.- - - - - - - - - - ranking = 4179.3923292209keywords = muscular atrophy, atrophy (Clic here for more details about this article) |
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