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1/186. Crusted scabies in association with human T-cell lymphotropic virus 1.

    BACKGROUND: Human T-cell lymphotropic virus I (HTLV- 1) infection can lead to myelopathy/tropical spastic paresis and adult T-cell leukemia/lymphoma (ATLL). infection with HTLV-1 has also been associated with clinically significant immunosuppression. Crusted scabies, also known as Norwegian scabies, is an uncommon presentation of scabies that may occur in conjunction with immunosuppression. Although crusted scabies has been reported in association with HTLV-1 infection, to our knowledge it has never been described in association with HTLV-1 associated myelopathy. OBJECTIVE: The aim is to describe a case of HTLV-1 associated myelopathy and concomitant crusted scabies. methods: This article includes a case report and a literature review. CONCLUSIONS: Crusted scabies is reported in association with HTLV-1 infection with or without concomitant ATLL. Crusted scabies should be considered in the differential diagnosis of a generalized cutaneous eruption in an HTLV-1 positive patient. patients with crusted scabies from an HTLV-1 endemic population should be rested for a possible HTLV-1 infection. These patients may be at increased risk of progressing to ATLL. ( info)

2/186. Idiopathic spinal cord herniation: case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: Idiopathic spinal cord herniation (ISCH) is a rare condition, reported in only 25 patients thus far, in which the thoracic cord is prolapsed through an anterior dural defect. It typically presents in middle age as either brown-sequard syndrome or spastic paraparesis. CLINICAL PRESENTATION: A 55-year-old woman initially presented at the age of 41 years with brown-sequard syndrome at the T8 disc space level on the left side. Investigations, including primitive magnetic resonance imaging, were deemed negative at that time. After a stepwise deterioration over 14 years, she presented again with spastic paraparesis and double incontinence, in addition to her previous spinothalamic dysfunction. magnetic resonance imaging at this stage suggested either ISCH or a dorsal arachnoid cyst. INTERVENTION: Through a T7-T8 laminectomy, a left-of-midline ISCH was identified and easily reduced by gentle cord traction. No dorsal arachnoid cyst was identified. The anterior dural defect was repaired with a XenoDerm patch (LifeCell Corp., Woodlands, TX). After surgery, there was improved motor and sphincter function. However, there was continued sensory disturbance. CONCLUSION: ISCH is rare cause of thoracic cord dysfunction. Despite prolonged diagnostic delay, significant clinical improvement may be obtained with ISCH reduction and anterior dural repair. ( info)

3/186. 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. ( info)

4/186. Spinal malignant melanotic schwannoma. Case report.

    The authors report a rare case of spinal cord compression syndrome due to a malignant melanotic schwannoma. Pathogenesis, diagnostic difficulty and therapeutical problems are discussed. The authors conclude that such tumours should be surgically treated due to the possibility of a benign clinical behaviour even in those cases showing malignant histological features. ( info)

5/186. Human T cell leukemia virus type I-associated myelopathy in a patient with systemic lupus erythematosus.

    A case of human T cell leukemia virus type I (HTLV-1) associated myelopathy (HAM)/tropical spastic paraparesis (TSP) with 14-year history of systemic lupus erythematosus (SLE) is reported. For 9 years, the numbness of the feet and sacral region progressed with occasional urinary incontinence and constipation. She was admitted to hospital due to gait disturbance and aggravation of SLE and the diagnosis of HAM/TSP was confirmed, indicating that HTLV-1 infection is associated with the development of not only HAM/TSP but also SLE. ( info)

6/186. Syringohydromyelia or HTLV-I-associated myelopathy/tropical spastic paraparesis. A diagnostic challenge. Case report.

    Human T-cell lymphotropic virus type I (HTLV-I) associated myelopathy/tropical spastic paraparesis (HAM/TSP) is the most common chronic myelopathy in brazil. We present the case of a 53 year old man that fulfilled the diagnostic criteria for HAM/TSP but had at the magnetic resonance imaging (MRI) of the spinal cord evidences of syringohydromyelia at the C6-C7 and D2-D7 levels along with Chiari type I malformation. The clinical picture was more typical of HAM/TSP than of syringohydromyelia, which was probably asymptomatic. The present case clearly demonstrates that serology and neuroimaging should be always used together. We conclude that, specially in places where HTLV-I is endemic, every patient with a spastic paraparesis, even with a radiological picture suggestive of a structural spinal cord lesion, should have a screening test for HTLV-I. The clinical picture must dictate the final direction of the diagnosis. ( info)

7/186. Ultrastructural pathology of a Chilean case of tropical spastic paraparesis/human T-cell lymphotropic type I-associated myelopathy (TSP/HAM).

    Human T-cell lymphotropic virus type I (HTLV-I), is the cause of endemic tropical spastic paraparesis (TSP) or HTLV-I-associated myelopathy (HAM). Because TSP/HAM is not a fatal disease, the neuropathology of this disease, albeit relatively well understood, is based on the examination of just a few incidental cases. Previously, we demonstrated peculiar lamellated structures, called "multilamellar bodies" (MLB). In this report, we present the ultrastructural neuropathology of a TSP/HAM case from chile, with further detailed descriptions of MLB. It is tempting to suggest that MLB may represent specific ultrastructural markers of TSP/HAM. The pathology of the anterior and posterior horns was similar and was comprised of axonal degeneration, accompanied by extensive astrocytic gliosis. Lymphocytic infiltration, particularly observed as "cuffs" around blood vessels, was scattered among other cellular elements. Ultrastructurally, myelin sheaths were relatively well preserved, and some demyelinated but not remyelinated fibers were observed. Moreover, axons with abnormal accumulations of neurofilaments, suggestive of axonal degeneration, were detected. Several axons contained Hirano bodies. In many samples, glial processes replaced most of the remaining neuropil. In a few specimens of the anterior and posterior horns of the spinal cord, MLB were observed. These structures consisted of stacks of 30 to 40 electron-dense lamellae, which were interrupted by narrow electron-lucent spaces. All of the lamellae were immersed within an amorphous substance of intermediate density. neurons of the dorsal root ganglia were basically normal except for increased lipofuscin accumulation. As in the spinal cord, myelinated axons were well preserved, but a few were demyelinated and surrounded by concentric arrays of Schwann cell membranes. Also, axons of the dorsal roots accumulated increased number of neurofilaments. mast cells and schwann cells were increased in number, the latter containing abundant pi granules and myelin fragments. ( info)

8/186. HTLV-I-associated myelopathy following allogeneic bone marrow transplantation.

    A 39-year-old polytransfused patient with aplastic anemia acquired transfusion-associated HTLV-I infection shortly before transplantation. The patient underwent allogeneic bone marrow transplantation and developed HTLV-I associated myelopathy 3 years later. Clinical abnormalities and a host of atypical findings are presented in the context of previous reports describing uncommon features of the disease. ( info)

9/186. Motor-evoked potentials: unusual findings.

    OBJECTIVE: The aims of this study were to present rare findings of motor evoked potentials (MEPs) in 3 patients with spastic paraparesis and to show that careful interpretation is indispensable in experiments done with very high intensity stimulation. methods: The conduction along several segments of the descending tracts was studied by our previously published method in 3 patients with spastic paraparesis. RESULTS: The threshold for activation of descending tracts was markedly increased in all the patients. In one patient, both transcranial electrical and magnetic cortical stimulation elicited responses with 4 different latencies. They were compatible with the latencies of I1-, D(D1)-, D2- or D3-waves. Very high intensity stimulation elicited D2 waves (activation around the cerebral peduncle) or D3 waves (activation at the foramen magnum level). In the other two patients, unexpectedly, the latency of responses to foramen magnum level stimulation was longer than the cortical latency. foramen magnum and spinal cord stimulation could not excite the corticospinal tract but activated other slowly conducting descending tracts (about 20 m/s), whereas cortical stimulation activated the corticospinal tract. CONCLUSIONS: The site of activation following cortical stimulation was variable when very high intensity stimulation is used. The descending tracts that contribute to the onset of electromyographic (EMG) responses may not be the same after cortical and spinal stimulation in patients with severely affected corticospinal tract, especially when using very high intensities of stimulation. Such factors complicate the interpretation of EMG responses obtained in patients with severely affected corticospinal tracts. ( info)

10/186. 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. ( info)
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