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PubMed1998

[A case of spastic paraparesis with mental deterioration and markedly thin corpus callosum--callosal dysfunction demonstrated by magnetic stimulation].

We have studied function of the corpus callosum in a patient with spastic paraparesis with mental deterioration and markedly thin corpus callosum using magnetic stimulation methods. In a 21-year-old woman with slowly progressive gait disturbance, neurological examination showed mental deterioration, euphoria, spastic paraparesis, bilateral Babinski's sign, and hyperesthesia caudal to the eighth thoracic level. No abnormalities were observed in electroencephalograms. Magnetic resonance imaging (MRI) studies of the brain showed cerebral cortical atrophy, markedly thin corpus callosum, and dilated cavum septum pellucidum and cavum Vergae, but spinal cord MRIs showed no abnormalities. The lysosomal enzyme activities, whose reduction was known to cause leukodystrophy, were all normal. Very long chain fatty acid was not increased in her blood, which is against adrenoleukodystrophy. She had no anti-HTLV-1 virus antibody. Based on these clinical features and the results of biochemical analyses, we diagnosed this patient as having spastic paraplegia associated with hypoplasia of the corpus callosum (Nojima and Iwabuchi). We performed three studies on the central motor pathways in this patient. The latencies of responses recorded from upper or lower limb muscles were all within the normal range, despite that the thresholds were slightly increased. This suggests that axonal degeneration occurs in the central motor pathways, which is consistent with the autopsy findings of a patient with hereditary spastic paraplegia associated with hypoplasia of the corpus callosum. Connection between the bilateral motor cortices was investigated by magnetic stimulation of both motor cortices. The suppression of the motor cortex evoked by stimulation of the contralateral motor cortex through the corpus callosum was absent in this patient. Intracortical inhibition within the motor cortex was demonstrated to be normal by a paired-magnetic stimulation technique. Based on the results of these results of these two experiments, we conclude that the function of the corpus callosum was disturbed in the present patient. This report first shows the functional abnormality of the extremely thin corpus callosum in a patient with hereditary spastic paraplegia associated with hypoplasia of the corpus callosum.

Adult↗

PubMed1992

Activated, HTLV-1-specific cytotoxic T-lymphocytes are found in healthy seropositives as well as in patients with tropical spastic paraparesis.

In human T cell lymphoma/leukemia virus (HTLV-1)-infected people with tropical spastic paraparesis (TSP), there are activated HTLV-1-specific cytotoxic T lymphocytes (CTL) in the circulation and lymphocytic infiltrates in spinal cord lesions that are rich in CD8+ T cells. These observations suggest a role for virus-specific CTL in the pathogenesis of TSP. We have examined the anti-HTLV-1 cytotoxic activity of freshly isolated CD8+ T cells from peripheral blood lymphocytes of eight subjects seropositive for HTLV-1. Four of five subjects with TSP had circulating activated anti-Tax CTL. However, two of three seropositive subjects without TSP also had activated anti-Tax CTL. These observations show that such activated CTL are not confined to patients with TSP and raise some uncertainty about their significance in the pathogenesis of the disease. In cultures of CD8+ T cells from two TSP subjects, we detected CTL with other HTLV-1 specificities, without exogenous antigenic stimulation. A CTL epitope in the middle region of Tax and one in the C terminus of Pol have been mapped at the peptide level and the HLA Class 1 molecules restricting their recognition have been defined.

Amino Acid Sequence↗

PubMed2004

Serum concentration and genetic polymorphism in the 5'-untraslated region of VEGF is not associated with susceptibility to HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) in HTLV-I infected individuals.

HTLV-I- associated myelopathy/tropical spastic paraparesis (HAM/TSP) is one outcome of human T-cell lymphotropic virus type I (HTLV-I) infection. It remains unknown why the majority of infected people remain healthy whereas only approximately 2-3% of infected individuals develop the disease. Recently, it has been reported that increased plasma concentrations of VEGF were significantly related to high ATL cell infiltration, and the viral transactivator Tax activates the VEGF promoter, linking the induction of angiogenesis to viral gene expression. To investigate whether VEGF promoter -634C/G single nucleotide polymorphism (SNP) and serum concentration of VEGF are associated with the development of HAM/TSP, we studied a group of 202 HAM/TSP patients, 202 asymptomatic HTLV-I seropositive carriers (HCs) and 108 seronegative healthy controls (NCs) in Kagoshima, Japan by using PCR-RFLP analysis. The serum concentration of VEGF was also compared among patients with HAM/TSP, ATL, HCs as well as with NCs. Our results indicate that both VEGF gene polymorphism and serum VEGF levels are not specifically associated with the risk of HAM/TSP in our cohort.

5' Untranslated Regions↗

PubMed1995

Clinical neurophysiological comparative study on HTLV-1 associated myelopathy/tropical spastic paraparesis.

In order to determine the neurophysiological characteristics of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) throughout the world, we analyzed and compared the most common clinical neurophysiological studies (CNPS) performed in this entity from those places with a high prevalence and interesting regional differences were noted. African patients showed a noteworthy involvement of the peripheral nervous system (PNS). Chilean patients displayed a more circumscribed abnormality around the spinal cord. The migrants from the West Indies to England showed important visual, and somatosensory evoked potential (SSEP) alterations in the upper limbs (UL). Japanese patients also presented some involvement of the PNS, but their illness duration was less protracted than that observed in other countries. The differences found in visual pathways and PNS involvement among these groups were statistically significant (p < 0.05). Thus, this study shows not only a widespread subclinical involvement in both central and PNS in HAM/TSP, but also strongly supports the idea that the lesion distribution and progression of this disease are different among countries. Such differences could likely be due to the action of the so-called environmental co-factors present in each of these areas which should be promptly investigated.

Human T-lymphotropic virus 1↗

PubMed2004

Severe resting clonus caused by thyrotoxicosis in a 16-year-old girl with hereditary spastic paraparesis: a case report.

A 16-year-old girl with a history of hereditary spastic paraparesis developed the subacute onset of severe clonus and new upper-extremity spasticity due to thyrotoxicosis. This case demonstrates the ability of hyperthyroid states to worsen symptoms of spasticity in a child with an underlying spastic disorder. It also demonstrates the importance of investigating for common systemic diseases as a cause of movement disorders even in children with underlying known genetic disorders.

Adolescent↗

PubMed2000

Variant Alzheimer's disease with spastic paraparesis and cotton wool plaques is caused by PS-1 mutations that lead to exceptionally high amyloid-beta concentrations.

We describe 3 new families affected by Alzheimer's disease with spastic paraparesis. In affected individuals, including the earliest known patient with this clinical syndrome, neuropathological examination revealed large "cotton wool" plaques similar to those we have previously described in a Finnish family. In the families in which DNA was available, presenilin-1 mutations were observed. Transfection of cells with these mutant genes caused exceptionally large increases in secreted Abeta42 levels. Furthermore, brain tissue from individuals with this syndrome had very high amyloid-beta concentrations. These findings define the molecular pathogenesis of an important subgroup of Alzheimer's disease and have implications for the pathogenesis of the disease in general.

Alzheimer Disease↗

PubMed1988

Seroprevalence of antibodies to HTLV-I in patients with chronic neurological disorders other than tropical spastic paraparesis.

Human T-lymphotropic virus type I (HTLV-I), the etiological agent of adult T-cell leukemia/lymphoma, also appears to be the cause of tropical spastic paraparesis, a chronic myelopathy reported in several different regions of the world. The prevalence of antibodies to HTLV-I in patients with chronic neurodegenerative disorders other than tropical spastic paraparesis and in patients with some muscle inflammatory disorders has been investigated. IgG antibodies to HTLV-I were measured in the sera and/or cerebrospinal fluid from 82 Guamanian patients with amyotrophic lateral sclerosis and parkinsonism-dementia, 164 Guamanian normal controls, 10 patients with kuru from the Eastern Highlands of Papua New Guinea, 4 patients with Viliuisk encephalomyelitis from the Iakut region of eastern Siberia, 45 Italian patients with multiple sclerosis, and 56 patients with polymyositis (49 from the United States and 7 from Jamaica). As determined by enzyme-linked immunosorbent assay, Western immunoblot, and gelatin particle agglutination techniques, serological evidence of HTLV-I infection was found in 1 patient with amyotrophic lateral sclerosis and 1 control subject from Guam, and in 1 patient from the United States and all 7 Jamaican patients with polymyositis. Except for the high seropositivity rate among the group of Jamaican patients with polymyositis, our data indicate that HTLV-I is an unlikely causative agent in the spectrum of the neurological diseases examined. The seropositivity of the 7 Jamaican patients with polymyositis requires further study.

Adult↗

PubMed2006

Intrathecal nicorandil and small-dose morphine can induce spastic paraparesis after a noninjurious interval of spinal cord ischemia in the rat.

We investigated the interaction between nicorandil, a K(+)ATP channel opener, and morphine on motor function after a noninjurious interval of spinal cord ischemia in the rat. Spinal ischemia was induced by aortic occlusion for 6 min with a balloon catheter in Sprague-Dawley rats. All animals received intrathecal (IT) injection of morphine (1-60 microg) 1 h after ischemia. In addition to IT injection of morphine, group M (control), group MN (combination of morphine and nicorandil), and group MNG (combination of morphine, nicorandil, and glibenclamide) received IT saline, nicorandil (10 microg), and both glibenclamide (10 microg) and nicorandil (10 microg) after 150 min of reperfusion, respectively. A quantal bioassay for the effect of IT morphine on neurological function after ischemia was performed to calculate 50% effective dose values (ED50) for inducing paraparesis at 3 h of reperfusion. The ED50 in group M and group MN was 15.1 +/- 4.9 microg and 2.9 +/- 1.0 microg of IT morphine, respectively (P < 0.05). In Group MNG, the dose-response curve shifted back to the right and the ED50 for inducing paraparesis was 11.6 +/- 4.7 microg of IT morphine. The present study demonstrates that IT small-dose morphine combined with nicorandil induces spastic paraparesis after noninjurious interval of spinal cord ischemia in the rat.

Animals↗

PubMed1986

Familial spastic paraparesis and deafness. A new x-linked neurodegenerative disorder.

We studied a large kindred with a chronic neurodegenerative disorder, affecting at least six male members in three generations. Spastic paraparesis, beginning at about 10 years of age, and hearing deficits were present in all affected members. Additionally, tremor ophthalmologic abnormalities, sensory deficits, short stature, hypogonadism, elevated cerebrospinal fluid protein, and absent or prolonged somatosensory evoked potentials were seen in some relatives. Although clinically similar to adrenomyeloneuropathy, the plasma and fibroblast levels of saturated very long-chain fatty acids were normal. This syndrome probably represents a new type of familial spastic paraparesis.

Adolescent↗

PubMed1991

[A case of spinal arteriovenous malformation associated with syringomyelia extended over the entire spinal cord that presented mainly as spastic paraparesis].

A 48-year-old housewife was hospitalized because of spastic paraparesis accompanied by dysesthesia and amyotrophy in her lower extremities, and by urinary disturbances. At the age of 35 years she noticed tingling sensations in her right foot and gradually her gait became spastic. In a few years lumbago and the spinal form of intermittent claudication developed. When she was 41 years old muscle wasting was noticed in her right leg, and in four years the same feature was also seen in her lower left limb. Plain X-ray films showed a wide cervical canal with no signs of basilar impression. On MRI we could see a polycyst-like syrinx that extended over the entire spinal cord. In addition the surfaces of the lower thoracic cord seemed irregular, but arteriovenous malformation itself was not visualized. Neither hydrocephalus nor Chiari malformation was accompanied. Needle EMG examination revealed some neurogenic changes in the muscles of both lower limbs. Cystometry proved uninhibited contractions of the detrusor muscle and the existence of detrusor-sphincter dyssynergia. Myelography showed an enlarged spinal cord above the D11-12 level with a shadow of worm-like appearance between the D7-12 level. CSF protein was elevated. Spinal angiography confirmed the presence of single coiled vessels with a nidus at D8-10 that were fed by bilateral D10 intercostal arteries and drained to D7, D9 or D12. Embolization of these abnormal vessels was conducted, and since then the patient's condition has remained stable. Vascular malformation is a rare cause of secondary syringomyelia. Clinical manifestations are determined mainly by the level of vascular malformation and are not reflective of the level of syrinx.(ABSTRACT TRUNCATED AT 250 WORDS)

Arteriovenous Malformations↗

PubMed1988

Bronchoalveolar lymphocytosis in patients with tropical spastic paraparesis associated with human T-cell lymphotropic virus type 1 (HTLV-1). Clinical, immunologic, and cytologic studies.

STUDY OBJECTIVE: To determine the features of pulmonary involvement in patients with tropical spastic paraparesis associated with human T-cell lymphotropic virus type 1. DESIGN: Nonrandomized prospective case series. SETTING: Tertiary care units in two university medical centers. PATIENTS: Consecutive sample of 21 patients with tropical spastic paraparesis associated with human T-cell lymphotropic virus type 1 infection. INTERVENTIONS: Chest roentgenogram and bronchoalveolar lavage were done in all patients. Fifteen patients had pulmonary function tests. Alveolar T-lymphocyte subsets were analysed in 10 patients and thoracic computed tomographic scans were done in 10 patients. MEASUREMENTS AND MAIN RESULTS: All patients were free of clinical pulmonary symptoms and had normal chest roentgenograms. Thoracic computed tomographic scans were normal in 9 of 10 patients and showed mild interstitial pneumonitis in 1. Pulmonary function tests were within the normal range in 13 patients and showed a mild restrictive syndrome in 2. Eighteen patients had increased absolute numbers of alveolar lymphocytes (mean, 77 +/- 39 X 10(3) lymphocytes/mL; range, 13.5 X 10(3) to 259 X 10(3) lymphocytes/mL). Sixteen patients had percentages of alveolar lymphocytes higher than 20% of all alveolar cells (mean, 33.5 +/- 12.7; range, 9 to 69). In all 10 patients tested, 64.2% +/- 13.2% of alveolar lymphocytes were CD8+ cells. CONCLUSIONS: Excessive absolute numbers and percentages of alveolar lymphocytes were observed in 18 and 16 patients, respectively. Most alveolar lymphocytes were CD8+ cells.

Adult↗

PubMed1990

HTLV-1 antibodies in serum and cerebrospinal fluid in tropical spastic paraparesis in Brazil.

HTLV-1 antibodies were investigated in serum and in CSF of 150 patients with neurologic disorders mainly myelopathies. The patients were considered into three groups according to the possible relationship of their disease to the presence of HTLV-1 antibodies: no relationship risk (control group), occasional risk group, and possible risk group. In this latter are 56 patients with crural spastic paraparesis or paraplegia of unknown etiology (SP). HTLV-1 antibodies were tested by the passive particle-agglutination method for anti-ATLA antibody detection. The search was negative in all patients of the control group, and positive (serum and/or CSF) in 16.5% of the patients from the second group and in 55.4% of the SP patients group. Clinical patterns in SP cases with HTLV-1 antibodies were those of tropical spastic paraparesis (TSP). CSF patterns considered (cytology, protein content and gamma-globulins rate) were different between TSP group with HTLV-1 antibodies in CSF and SP group with no HTLV-1 antibodies detection either in serum or in CSF. The difference was significant. Results of this investigation confirm the high incidence of TSP in Brazil, and bring additional indication for searching HTLV-1 antibodies in the CSF.

Adolescent↗

PubMed2006

Flow cytometry evaluation of the T-cell receptor Vbeta repertoire among human T-cell lymphotropic virus type-1 (HTLV-1) infected individuals: effect of interferon alpha therapy in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP).

Human T-cell lymphotropic virus type-1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is chronic inflammatory disease of the spinal cord characterized by perivascular lymphocytic cuffing and parenchymal lymphocytic infiltration. In this study using flow cytometry, we have investigated the T-cell receptor (TCR) Vbeta repertoire of peripheral blood T lymphocytes in 8 HAM/TSP patients, 10 HTLV-1 infected healthy carriers, and 11 uninfected healthy controls to determine if there is a biased usage of TCR Vbeta. We found that TCR Vbeta7.2 was under-utilized and Vbeta12 was over-utilized in CD4+ T cells of HTLV-1 infected individuals compared with healthy uninfected controls, whereas there were no such differences in CD8+ T cells. Comparison of Vbeta repertoire changes before and after interferon-alpha (IFN-alpha) treatment for HAM/TSP revealed that one out of five patients showed dramatic decrease of specific Vbeta in CD8+ T cells. Our results suggest that dominant Vbeta subpopulations in CD4+ T cells evolved associated with chronic HTLV-1 infection, and IFN-alpha treatment for HAM/TSP does not induce a specific pattern of TCR Vbeta changes.

Antiviral Agents↗

PubMed2003

Mediators of central nervous system damage during the progression of human T-cell leukemia type I-associated myelopathy/tropical spastic paraparesis.

Human T-cell leukemia virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) represents one of the most devastating diseases associated with HTLV-I infection. Despite the delineation of clinical features associated with this neurologic disease, more progress needs to be made with respect to understanding the molecular mechanisms relating to the genesis of HAM/TSP. Several factors have been hypothesized to contribute to whether an HTLV-I-infected individual remains asymptomatic, develops adult T-cell leukemia (ATL), or progresses to HAM/TSP. Among the most intriguing of these factors is the immune response mounted by the host against HTLV-I. Several cell populations are crucial with respect to generating an efficient immune response against the virus. This includes CD4(+) T cells, CD8(+) T cells, dendritic cells (DCs), monocytes/macrophages, and HTLV-I-infected cells that interact with immune cells to stimulate their effector functions. Although all of these cell types likely play important roles in the etiology of HAM/TSP, this review focuses specifically on the potential function of the CD8(+) T-cell population during the progression of HTLV-I-induced neurologic disease. The immune response in HAM/TSP patients may transition from a beneficial response aimed at controlling the viral infection, to a detrimental response that ultimately participates in mediating the pathology observed in HAM/TSP. In this respect, the generation of a hyperactive CD8(+) cytotoxic T lymphocyte (CTL) response primarily targeting the HTLV-I Tax protein likely plays a key role in the genesis of pathologic abnormalities associated with HAM/TSP. The efficiency and activity of Tax-specific CD8(+) CTLs may be regulated at a number of levels, and deregulation of Tax-specific CTL activation may contribute to HAM/TSP. This review focuses on potential mechanisms of central nervous system (CNS) damage associated with the genesis of HAM/TSP following HTLV-I infection, focusing on the role of the Tax-specific CTL compartment.

Blood-Brain Barrier↗

PubMed1993

Intravenous methylprednisolone in HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP).

HTLV-I (Human T-lymphotropic virus type I) associated myelopathy/tropical spastic paraparesis (HAM/TSP) is an immunomediated myelopathy induced by the HTLV-I. Some patients, specially those from Japan, seem to have a good response to steroid treatment. However, this has not been found in other regions of the world. High dose intravenous methylprednisolone has been used with success in patients with relapses of multiple sclerosis (MS), another autoimmune disease of the central nervous system. To test the effectiveness of methylprednisolone in patients with HAM/TSP, we devised an open trial in 23 patients. We found a very limited benefit of this form of treatment in these patients. Only one patient, who had the shortest disease duration (five months) in the whole group, showed a sustained benefit. We speculate that those patients with a shorter history, with presumably less demyelination and more inflammatory lesions, would show a better response to immunosuppressive treatments.

Female↗

PubMed1998

[HTLV-I-associated myelopathy, tropical spastic paraparesis: report of the first cases in Sergipe, Brazil].

HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) has been described in practically all regions of Brazil. The authors present eight clinically defined cases of HAM/TSP, as being the first reported in Sergipe (Northeastern Brazil). All of them were confirmed through ELISA in two examinations, although only two were confirmed by Western Blot. According to clinical/laboratorial investigation protocol, all patients presented involvement of the pyramidal tract with minimal sensory loss and sphincter alteration. The authors call the attention for the endemicity of HTLV-I in the region, whose prevalence amongst blood donors is significant (0.43%).

Adult↗

PubMed1999

[Subcortical dementia in HTLV-I tropical spastic paraparesis. Study of 43 cases].

BACKGROUND: Central nervous system damage associated to HTLV-I does not limit itself to the spinal cord, but also involves subcortical structures, producing cognitive impairment and behavioral changes which eventually could conform a new form of subcortical dementia. AIM: To study cognitive changes in patients with HTLV-I associated myelopathy. PATIENTS AND METHODS: Forty three patients (31 female) with Tropical Spastic Paraparesis, aged 52 years old as a mean and with a disease lasting a mean of 7.5 years, were studied. The diagnosis was based on clinical, radiological and neurophysiological changes. The virus was identified with ELISA, indirect immunofluorescence, Western Blot or proviral DNA identification. Cognitive assessment was done using the Wechler Adult Intelligence Scale (WAIS) and Benton Visual Retention Test (form D). Patients were grouped according to their motor disability in; 23 patients with independent spastic gait, 11 patients that needed support to walk and 9 patients unable to walk. RESULTS: WAIS test demonstrated cognitive impairment with special deficit in some subtests such as Digit Span, Digit Symbol, Picture Arrangement and Object Assembly. Benton Test also disclosed cognitive impairment. There was a positive relationship between cognitive and motor performance. CONCLUSIONS: At least 50% of patients with Tropical Spastic Paraparesis have certain degree of intellectual and affective impairment.

Adult↗

PubMed2006

Biological effects of four PSEN1 gene mutations causing Alzheimer disease with spastic paraparesis and cotton wool plaques.

We describe the biological consequences on PSEN1 exons 8 or 9 splicing and Abeta peptides production of four PSEN1 mutations associated with a phenotypic variant of Alzheimer disease, which includes cotton wool plaques and spastic paraparesis (CWP/SP). Two of these mutations (c.869-22_869-23ins18 and c.871A > C, p.T291P) are novel mutations located in intron 8 and exon 9, respectively. The c.869-22_869-23ins18 mutation caused exon 9 skipping whereas the c.871A > C (p.T291P) mutation showed only a modest effect on exon 9 skipping. The previously reported E280G and P264L mutations, located in exon 8, had no effect on mRNA splicing. Infection of cells with mutant T291P, E280G, or P264L cDNAs caused a variable increase in secreted Abeta42. We conclude that none of the previously proposed mechanisms, i.e. exceptionally large increases in secreted Abeta42 levels or loss of PSEN1 exons 8 or 9, provides complete explanation of the CWP/SP phenotype.

Alzheimer Disease↗