1/39. 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 = ocular (Clic here for more details about this article) |
2/39. 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 = 4.942611988473keywords = dystrophy (Clic here for more details about this article) |
3/39. Bilateral blindness and lumbosacral myelopathy associated with high-dose carmustine and cisplatin therapy.PURPOSE: To report the early ocular pathologic findings associated with high-dose carmustine and cisplatin therapy. methods: A patient with metastatic breast carcinoma developed an acute onset of branch retinal artery occlusion, bilateral blindness, and a myelopathy involving the lower extremities after high-dose chemotherapy and bone marrow transplant. RESULTS: Histopathologic examination of the eye and optic nerves at autopsy disclosed nerve fiber layer infarction secondary to right inferior temporal retinal artery thrombosis. Patchy necrosis of both optic nerves, medulla oblongata, and spinal cord was associated with focal small-vessel thrombosis. CONCLUSIONS: The syndrome of retinal vascular occlusion, optic neuropathy, and myelopathy is associated with the high-dose chemotherapeutic agents carmustine and cisplatin. The distribution of necrosis suggests an ischemic event rather than direct neurotoxic effects.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
4/39. Transganglionic gracile response following limb amputation in man.Gracile neuroaxonal dystrophy (nad) is an distinctive morphological alteration of central projecting axon terminals of dorsal root ganglion neurons. Experimentally, lower limb amputation has been shown to accelerate the formation of gracile nad, suggesting that the transganglionic response to peripheral axotomy may play a role in its development. To determine if a similar response occurs in the human sensory nervous system following peripheral nerve injury, we have performed postmortem histopathological examinations of the dorsal column nuclei of three patients (aged 15, 55, and 77 years old); all of whom had undergone accidental or therapeutic unilateral limb amputation (1 year, 38 years, and 1 year 8 months prior to death, respectively). In a 15-year-old man who underwent therapeutic leg amputation, the gracile nuclei on the transected side revealed reactive gliosis and many small axonal spheroids. The spheroids and fine neurites were immunolabelled with antibodies for growth-associated protein-43, ubiquitin and neuropeptide y (NPY). Neither routine histological nor immunohistochemical methods demonstrated comparable changes in the contralateral gracile nucleus. In a 77-year-old man who underwent leg amputation, the gracile nucleus on the amputated side was gliotic and showed several NPY and ubiquitin-immunoreactive spheroids, which were not seen in the contralateral non-transected side. A 55-year-old man with a history of accidental arm amputation showed well-developed nad in the cuneate nucleus only on the transected side. This study clearly demonstrates the occurrence of transganglionic response to limb amputation in human dorsal column nuclei. The extent of the regenerative and/or degenerative responses may vary depending on the age of the patient and the time interval following the peripheral axotomy.- - - - - - - - - - ranking = 4.942611988473keywords = dystrophy (Clic here for more details about this article) |
5/39. Spontaneous thoracic spinal cord herniation--case report.A 54-year-old female presented with spontaneous thoracic spinal cord herniation manifesting as chronic progressive motor weakness in both legs. Spastic paraparesis (4/5) and pathological reflexes such as ankle clonus were noted. She also had mild bladder dysfunction but no bowel dysfunction. She had no sensory disturbance, including tactile and pinprick sense. Magnetic resonance (MR) imaging revealed that the atrophic spinal cord was displaced into the ventral extradural space at the T4-5 intervertebral level with markedly dilated dorsal subarachnoid space. Computed tomography obtained after myelography showed no evidence of intradural spinal arachnoid cyst. She underwent surgical repair of the spinal cord herniation via laminectomy, and spinal cord herniation through the ventral dural defect was confirmed. Postoperative MR imaging revealed improvement of the spinal cord herniation, but her symptoms were not improved. Spontaneous spinal cord herniation is a rare cause of chronic myelopathy, occurring in the upper and mid-thoracic levels, and the spinal cord is usually herniated into the ventral extradural space. Early differential diagnosis from intradural spinal arachnoid cysts is important for a satisfactory outcome.- - - - - - - - - - ranking = 3862.8737255259keywords = chronic progressive (Clic here for more details about this article) |
6/39. 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 = 2.2737883809353keywords = myopathy (Clic here for more details about this article) |
7/39. Human T cell leukaemia virus type I associated neuromuscular disease causing respiratory failure.polymyositis and inclusion body myositis have rarely been described in association with human T cell leukaemia virus type I (HTLV-I) infection. Most of such patients have coexisting HTLV-I associated myelopathy (HAM). Two patients with HTLV-I infection, myopathy, and respiratory failure are described. The muscle biopsy specimen of the first patient bore the histological features of inclusion body myositis and there was no evidence of concurrent myelopathy. The second patient had HAM, and her muscle biopsy showed non-specific myopathic and neuropathic changes. Both patients developed respiratory muscle weakness over eight years after diagnosis of myopathy, leading to hypercapnic respiratory failure requiring mechanical ventilatory support. Respiratory failure as a complication of HTLV-I associated myopathy has not previously been described.- - - - - - - - - - ranking = 6.821365142806keywords = myopathy (Clic here for more details about this article) |
8/39. 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 = 1keywords = ocular (Clic here for more details about this article) |
9/39. Encephalocraniocutaneous lipomatosis: case report and review of the literature.PURPOSE: Encephalocraniocutaneous lipomatosis is a congenital neurocutaneous syndrome characterized by lipomatous craniofacial hamartomas. The most common ocular manifestation is epibulbar choristoma, but many additional eye anomalies may be present. methods: To our knowledge, this is the first reported case with bilateral aniridia. We report a case of a boy affected by this syndrome and review the literature. CONCLUSION: Regarding this case, we suggest adding aniridia to the possible ocular anomalies in this syndrome.- - - - - - - - - - ranking = 2keywords = ocular (Clic here for more details about this article) |
10/39. Schistosomal myelopathy mimicking spinal cord neoplasm.We describe a 48-y-old male with chronic progressive myelopathy suggesting thoracic intramedullary neoplasm but in whom laboratory workup disclosed schistosoma mansoni myelopathy. The case illustrates the need for careful investigation of schistosomiasis in patients from endemic regions with myeloradiculopathy signs.- - - - - - - - - - ranking = 3862.8737255259keywords = chronic progressive (Clic here for more details about this article) |
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