1/49. Ankylosing spondylitis and multiple sclerosis.Ankylosing spondylitis can be associated with extra-articular involvement. Besides internal and ocular complications, neurological manifestations such as single root lesions, compression of the myelum or the cauda equina syndrome have also been described. We present a patient with ankylosing spondylitis who developed a monophasic myelopathy resembling multiple sclerosis. literature data show no conclusive evidence for an increased association of ankylosing spondylitis and multiple sclerosis. However, a monophasic myelopathy may be a separate neurological manifestation associated with ankylosing spondylitis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/49. Optic-spinal form of multiple sclerosis and anti-thyroid autoantibodies.The optic-spinal form of multiple sclerosis (OSMS), characterized by recurrent involvement of optic nerve and spinal cord with rare brain magnetic resonance imaging lesions, is relatively common among Asians. While individual cases of OSMS with anti-thyroid autoantibodies (ATABs) have been reported, the frequency of ATAbs in OSMS and classical multiple sclerosis has not been studied. We studied serum ATAbs and anti-nuclear antibodies (ANA) in 46 Japanese patients with multiple sclerosis: 14 with OSMS, and 32 with non-OSMS. Six patients were positive for ATAbs: five women with OSMS and one man with non-OSMS. The frequency of ATAbs in OSMS (5/14) was significantly higher than that in non-OSMS (1/32; P = 0.007), but the frequency of ANA did not differ between OSMS (3/14) and non-OSMS (6/32; P = 0.99). There may be a pathogenetic link between anti-thyroid autoimmunity and a subgroup of OSMS in Japanese.- - - - - - - - - - ranking = 1.1666666666667keywords = sclerosis (Clic here for more details about this article) |
3/49. syringomyelia and complex regional pain syndrome as complications of multiple sclerosis.OBJECTIVE: To describe a patient from Southeast Asia with the optic-spinal phenotype of multiple sclerosis who developed syringomyelia and resultant complex regional pain syndrome (formerly named reflex sympathetic dystrophy). DESIGN: Case report. SETTING: Department of neurology at a tertiary care hospital in the Republic of singapore. PATIENT: A 53-year-old Chinese woman with a history of optic neuritis developed an episode of left hemiparesis leading to a diagnosis of multiple sclerosis. Serial neuroimaging studies revealed an active demyelinating plaque in the cervical area that later progressed into a syrinx. Over a period of 1 year she also developed signs of sympathetic dysfunction including horner syndrome of the left eye and complex regional pain syndrome in the left hand. CONCLUSIONS: A case of the optic-spinal phenotype of multiple sclerosis that is commonly observed in Southeast Asia is described. This characteristically tissue-destructive form of multiple sclerosis resulted in syringomyelia complicated by a complex regional pain syndrome. Possible pathogenic mechanisms for these associations are discussed.- - - - - - - - - - ranking = 1.3333333333333keywords = sclerosis (Clic here for more details about this article) |
4/49. 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 = 0.33333333333333keywords = sclerosis (Clic here for more details about this article) |
5/49. Cervical epidural pseudotumor and multifocal fibrosclerosis. Case report and review of the literature.The authors present the case of a 45-year-old man suffering from progressive quadriplegia due to an expansive C3-T2 epidural mass. Neuropathological examination demonstrated pseudotumor tissue. The patient had had an orbital pseudotumor 5 years before admission, and other systemic manifestations of an idiopathic inflammatory disease were discovered. This case is extremely rare. Nine cases of multifocal fibrosclerosis with central nervous system involvement are described in the literature. To the authors' knowledge, this is the first description of a cervical epidural pseudotumor. Modern imaging has made the diagnosis of such an entity possible, and it is important for the neurosurgeon to consider this syndrome because the combination of surgery and systemic medical therapy can ensure a long-term survival with good quality of life.- - - - - - - - - - ranking = 0.83333333333333keywords = sclerosis (Clic here for more details about this article) |
6/49. Orbital, adnexal, and unusual systemic involvement in Rosai-Dorfman disease.PURPOSE: To describe the unusual clinical course of a patient with Rosai-Dorfman disease (RDD) affecting the eyelid and orbital tissues and involving the spinal cord. methods: Case report. RESULTS: A 68-year-old Indian man first presented in 1994 with a right lower eyelid lump for 1 year. An en bloc excisional biopsy was reported to show "reactive lymphoid hyperplasia with sclerosis." The patient subsequently defaulted follow-up and presented again in 1999 with bilateral lower eyelid masses and proptosis. Computerized tomography showed bilateral orbital, ethmoidal sinus, and frontal sinus soft tissue masses. Bilateral excisional biopsies of the orbital and eyelid masses showed histologic features of RDD. The patient had a history of paraplegia with decompression laminectomy and excision of an epidural mass in 1994. In addition, he underwent excision of soft tissue masses from the abdominal wall in 1993. Retrospective review of the histologic specimens from these two areas showed a histologic picture similar to that of eyelid specimens (in 1994 and 1999). CONCLUSIONS: It is important to consider RDD in addition to lymphoproliferative disorders in a patient with orbital and ocular adnexal masses. The initial histologic presentation may not be pathognomonic.- - - - - - - - - - ranking = 0.16666666666667keywords = sclerosis (Clic here for more details about this article) |
7/49. Recurrent encephalopathy and seizures in a US native with HTLV-I-associated myelopathy/tropical spastic paraparesis: a clinicopathologic study.A patient with progressive spastic paraparesis originally ascribed to multiple sclerosis developed recurrent encephalopathy and seizures. A diagnosis of HTLV-I-associated myelopathy/tropical spastic paraparesis was established prior to death. autopsy confirmed chronic inflammatory myelopathy and active inflammation in the white matter of the temporal lobes.- - - - - - - - - - ranking = 0.16666666666667keywords = sclerosis (Clic here for more details about this article) |
8/49. neurologic manifestations of progressive systemic sclerosis.Neurologic involvement in progressive systemic sclerosis is considered uncommon. We retrospectively examined the prevalence and nature of neurologic complications in 50 patients with progressive systemic sclerosis. In 20 (40%), neurologic abnormalities were detected, with a total of 28 neurologic manifestations. All levels of the central and peripheral nervous system were affected: muscle (22%), peripheral nerve (18%), spinal cord (8%), and brain (6%). Of note were the presence of myelopathy in four patients and inclusion-body myositis in two. In 10 patients (20%), no definable cause of the neurologic dysfunction could be identified, apart from progressive systemic sclerosis. Thus, neurologic presentations of progressive systemic sclerosis are much more common than previously reported and may be due to direct involvement of the nervous system by a primary pathologic process in a significant number of patients.- - - - - - - - - - ranking = 1.3333333333333keywords = sclerosis (Clic here for more details about this article) |
9/49. Pseudoathetosis as a presenting symptom of spinal multiple sclerosis.We report on a 38-year-old female patient with acute proprioceptive sensory impairment and pseudoathetosis in the four limbs, particularly in the fingers of both hands. She had great difficulty in buttoning, unbuttoning, using chopsticks and writing, because she was no longer able to feel her fingers in space. There was a profound loss of position and vibration sensation in all limbs, especially in both hands. Pseudoathetoid movement, a rare presentation of multiple sclerosis, was observed in the outstretched hands and extended fingers. T1- and T2-weighted magnetic resonance imaging (MRI) of the spinal cord revealed an inactive intramedullary lesion in the C2-C5 segments. gadolinium-enhanced MRI revealed an active lesion in the posterior columns of the cervical cord at the C3 vertebral level, which is very likely responsible for pseudoathetoid movement. Based on the disseminated lesions in the spinal cord verified by MRI the four neurologic manifestations, and the abnormal somatosensory-evoked potentials, we made a definitive diagnosis of multiple sclerosis of the spinal cord.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
10/49. Neurological complications of Werner's syndrome.patients with Werner's syndrome have the appearance of premature ageing. Neurological complications are usually regarded as uncommon. The neurological manifestations in three patients with cardinal features of Werner's syndrome, including short stature, premature greying and baldness, thin arms and legs, cataracts and scleroderma-like skin changes, are presented. The neurological features included transient ischaemic attacks secondary to atherosclerosis in the common carotid arteries (one patient), sensory peripheral neuropathy (one patient) and peripheral neuropathy with a possible myelopathy (one patient). In one of these patients the diagnosis of Werner's syndrome was not recognised prior to neurological referral. Although neurological disease in patients with Werner's syndrome is uncommon, it may be under-recognised. Some of the neurological complications are secondary to premature cerebrovascular disease, but the pathogenesis of peripheral neuropathy and myelopathy in patients with Werner's syndrome is uncertain.- - - - - - - - - - ranking = 0.16666666666667keywords = sclerosis (Clic here for more details about this article) |
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