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1/10. depression of evoked electromyographic (EEMG) responses by propofol in a patient with human T-cell lymphotropic virus type I-associated myelopathy (HAM).

    IMPLICATIONS: We report a patient with human T-cell lymphotropic virus type I-associated myelopathy. Although muscle strength in both of the upper extremities was normal in this patient, evoked electromyogram of the adductor pollicis was depressed by propofol at the induction of anesthesia.
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2/10. Low HTLV-1 seroprevalence in endemic tropical spastic paraparesis in ethiopia.

    Tropical spastic paraparesis (TSP), a chronic progressive myelopathy, occurs in ethiopia in epidemic form as neurolathyrism, while the endemic form has remained obscure. We describe the clinical features of 22 patients with TSP and the results of screening for HTLV-1 in these patients, 26 patients with other neurological disorders, 14 patients with leukaemia and 66 blood donors. The major manifestations in the patients with TSP were weakness and spasticity of the lower limbs with upper motor neurone signs and minimal sensory loss and bladder dysfunction. Two patients with TSP (9%), 2 patients with other neurological disorders (7.7%) and one patient with leukaemia and deafness were seropositive for HTLV-1. All the 66 blood donors were seronegative. Our results suggest that HTLV-1 may not play a major role in the pathogenesis of TSP in ethiopia.
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3/10. Skeletal muscle involvement in tropical spastic paraparesis/HTLV-1-associated myelopathy.

    The frequency of muscle involvement in TSP/HAM is not known, nor is the precise role that HTLV-1 and the diverse cytokines play in the genesis of HTLV-1-associated diseases. In order to better define the frequency and characteristics of the skeletal muscle involvement in TSP/HAM, we studied 11 affected patients. EMG was performed in 9 patients and muscle biopsy was performed in all 11. Muscle tissue was analyzed using: reverse transcriptase PCR for interleukin-1 in 8; PCR for HTLV-1 proviral dna in 5; and electron microscopy for viral particles in 3. We found pathologic alterations in all 11 patients. Four patients (36%) had a neurogenic process, while a primary muscle involvement was observed in the rest (64%). Four patients (36%) had polymyositis, and 3 (27%) had a noninflammatory myopathy. muscle weakness in the upper limbs was significantly associated with inflammation in the muscle biopsy. EMG was abnormal in only 2 of 9 patients. Reverse transcriptase PCR did not demonstrate message for interleukin-1 in any sample examined. PCR did identify HTLV-1 proviral dna in the muscle of 3 patients. Retroviral-like particles were found, by EM, in only one biopsy. HTLV-1 may play an important role in the pathogenesis of the frequent myopathies associated with HAM/TSP.
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4/10. Tropical spastic paraparesis in an aborigine.

    OBJECTIVE: To present the first documented case of human T-lymphotropic virus type I (HTLV-I) associated myelopathy/tropical spastic paraparesis in the Australian population. CLINICAL FEATURES: A 31-year-old Aboriginal man with an 18-month history of progressive weakness of the legs was found to have an upper motor neurone weakness of all limbs associated with sphincteric disturbance and impotence. htlv-i antibodies were detected in his serum and no other cause for the patient's myelopathy could be found. INTERVENTION AND OUTCOME: He was counselled regarding HTLV-I associated myelopathy/tropical spastic paraparesis. CONCLUSION: This is the first description of HTLV-I associated myelopathy/tropical spastic paraparesis in an Australian. In cases of spinal cord disorder without evidence of compression we recommend serological testing for HTLV-I, especially in Aboriginal patients. Additionally, testing of blood donors for this retrovirus needs consideration.
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5/10. Primary xanthoma of thoracic spine presenting with myelopathy.

    STUDY DESIGN. This retrospective case study reports on a patient with an isolated primary xanthoma arising in the second thoracic vertebra with paravertebral and spinal canal extension. OBJECTIVE. The possibility of this lesion's occurrence in the spine is presented with radiologic and pathologic findings appropriate for the diagnosis of spinal xanthoma. SUMMARY OF BACKGROUND DATA. Primary xanthoma of bone is an extremely rare but benign entity. It has not been previously described in this location, and has been described only once in the spine at all. methods. The mass was resected by curettage, and posterior instrumentation was successfully performed. Preoperative radiographs and magnetic resonance imaging of the thoracic spine were performed, as was histologic examination of the lesion. RESULTS. Radiographs of the upper thoracic spine revealed a lytic defect. magnetic resonance imaging revealed a heterogeneous lesion that was enhanced upon the administration of gadolinium-diethylenetriaminetetraacetic acid. Histologic examination revealed a cellular lesion consisting of lipid-laden macrophages, fibroblasts, and scattered Touton giant cells. These features correspond to descriptions of primary xanthoma of bone. Two years after surgery, the patient was neurologically intact with no evidence the lesion would recur. CONCLUSIONS. Primary xanthoma of bone is considered a benign lesion and can be successfully treated by local resection without adjuvant therapy. It should be considered part of the differential diagnosis of a mass lesion, with appropriate signal characteristics presenting in a thoracic vertebra.
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6/10. HTLV-I associated uveitis, myelopathy, rheumatoid arthritis and sjogren's syndrome.

    A 62 year-old white female presented with a 10-year-history of slowly progressive spastic paraparesis, pain and dysesthesia in the lower limbs and sphincter disturbance. A few years after the onset of the neurologic symptoms she developped migratory arthritis with swelling of the knees and pain on palpation of knees and fingers, dry eyes, mouth and skin. Two months before admission she presented bilateral nongranulomatous anterior uveitis. Examination revealed spastic paraparesis with bilateral Babinski sign, a decreased sensation level below L3, decreased vibration sense in the lower extremities, and a postural tremor of the upper limbs. Laboratory work-up disclosed HTLV-I positive tests in the blood and cerebrospinal fluid (CSF), and a mild pleocytosis in the CSF with a normal protein content. Nerve conduction velocity studies were normal. The present case shows the association of uveitis, arthritis and sjogren's syndrome in a patient with tropical spastic paraparesis/human T-cell lymphotropic virus type I (HTLV-I) associated myelopathy (TSP/HAM), and illustrates the wide spectrum of clinical manifestations which may accompany this infection with this virus.
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7/10. Thoracic spondylosis presenting with spastic paraparesis.

    Spondylotic change of the spine is common in the cervical and lumbar regions and may present with compression of the spinal cord and nerve roots. Myelopathy due to degenerative disease in the thoracic spine is exceptional. Only a few cases have been reported in the literature and these reports have described disease in the lower four thoracic segments. We report a case of paraparesis caused by degenerative disease in the upper thoracic spine which has not previously been described.
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8/10. Idiopathic dural herniation of the thoracic spinal cord.

    Symptomatic anterior or anterolateral dural herniation of the spinal cord is rare, and not uncommonly misdiagnosed, both clinically and radiologically. We present four patients with a radiological diagnosis of herniation of the thoracic spinal cord, and review the current literature. All affected patients have been adults, typically presenting with long-standing, unexplained sensory symptoms and eventually developing a brown-sequard syndrome, with or without motor changes. Herniation occurs in the upper or midthoracic region, between the T2 and T8 levels.
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9/10. CD8 and CD4 T cell-mediated polymyositis complicating the HTLV-1 associated myelopathy. Quantitative evaluation of corticosteroid treatment.

    INTRODUCTION: Inflammatory myopathy is a treatable cause of worsening in the spectrum of neurological conditions that may develop during the course of HTLV-1 infection. MATERIAL AND methods: To investigate the cause of subacute worsening in the strength of a 46-y-old black male with HTLV-1 associated myelopathy we performed electrodiagnostic examination and a muscle biopsy which was studied with histochemistry, immunocytochemistry and electron microscopy. Serial measurements of isometric muscle strength were performed during the course of corticosteroid treatment. RESULTS: The muscle biopsy showed evidence of denervation atrophy and prominent inflammatory changes with autoaggressive features. Lymphocyte typing showed a predominance of CD8 T cells. The patient had sustained, marked improvement in strength, especially of the upper extremities, with oral, high single-dose, alternate-day prednisone therapy. CONCLUSION: A muscle biopsy should be considered in all patients with HTLV-1 associated weakness, especially when electromyography indicates possible coexisting primary muscle involvement and/or serum creatine kinase levels are elevated. HTLV-1-associated polymyositis can be successfully treated with corticosteroids.
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10/10. Thoracic spinal Pantopaque cyst and associated syrinx resulting in progressive spastic paraparesis: case report.

    OBJECTIVE AND IMPORTANCE: We describe a unique presentation of a thoracic spinal Pantopaque cyst. Although Pantopaque is no longer used, sequelae of its long-term use may continue to surface. CLINICAL PRESENTATION: Our patient presented to the emergency room with 4 months of progressive lower extremity numbness, spasticity, and incontinence and a 2-day history of left upper extremity dysesthesias. magnetic resonance imaging at admission revealed a C7-T10 syrinx and an intrathecal extramedullary mass to the right of the spinal cord at T10-T11. INTERVENTION: Radiographic evidence led to the initial diagnosis of hemangioblastoma. The results of a critical analysis of the preoperative studies caused us to suspect a Pantopaque cyst. This suspicion was confirmed at the time of surgery, when a cyst filled with contrast medium was revealed. T9-T11 laminectomies were performed, allowing for a 5-cm dural opening. The cyst was aspirated and excised, and then the syrinx was decompressed. CONCLUSION: Our patient experienced significant sensory and motor improvement postoperatively. We present a previously undescribed complication of one of the most commonly used contrast media, Pantopaque. Despite its replacement with newer agents, it may continue to play a significant role in the pathological presentation of patients in whom it was previously used.
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