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PubMed1993

Spastic paraparesis associated with human T-lymphotropic virus type I: a clinical, serological, and genomic study in Iranian-born Mashhadi Jews.

The Mashhadi-Jewish community originating in Iran is a closed and ethnically segregated population with a unique history and a high rate of intrafamilial marriage among its members. A high risk of infection by human T-lymphotropic virus type I (HTLV-I) and of adult T-cell leukemia associated with such infection was found in this population. HTLV-I is also associated with a syndrome of progressive spastic paraparesis. We therefore evaluated the occurrence of HTLV-I infection and spastic paraparesis in Mashhadi-born Iranian Jews who immigrated to Israel. We examined 83 Mashhadi-born subjects (52 women, 31 men; mean age, 61 +/- 15.5 years) and 73 age-matched non-Mashhadi Iranian-born Jews. Blood samples were tested for HTLV-I antibodies by particle agglutination test. The polymerase chain reaction (PCR) was used to detect HTLV-I proviral DNA sequences from peripheral blood mononuclear cells. Fifteen Mashhadi-born Jews (18%) were both seropositive and PCR-positive for HTLV-I. Four HTLV-I-seronegative subjects were found to be positive for HTLV-I proviral DNA by PCR. Of the 19 HTLV-I-infected subjects (11 women, 8 men; mean age, 59 +/- 16 years), 13 (68%) had spastic paraparesis of varying severity. There were no signs of myelopathy in the Mashhadi-born subjects who were negative for HTLV-I proviral DNA by PCR. None of the non-Mashhadi Iranian Jews was seropositive or PCR-positive for HTLV-I proviral DNA, or had clinical signs of spastic paraparesis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

Severe segmental narrowing of the spinal cord: an unusual finding in congenital spastic paraparesis.

A three-year-old boy is reported who had congenital spastic paraparesis caused by severe segmental narrowing of the spinal cord. X-rays of the spine showed thoracolumbar scoliosis and anomalies of the thoracic vertebrae from T5 to T11. Myelography, with CT scanning, demonstrated severe narrowing of the T5 to T12 segments of the spinal cord, measuring only 1.5 mm at T10. This is believed to be the first report of this unusual anatomical finding in congenital spastic paraparesis. An early embryonic vascular insult is thought to be the most likely cause.

Child, Preschool↗
PubMed2000

[Increased activity of metalloproteinases and their inhibitors in cerebrospinal fluid of patients with tropical spastic paraparesis].

BACKGROUND: Proteolytic modifications of neuronal surfaces and the surrounding extracellular matrix are very important in neuronal development and regeneration. Increased activity of matrix metalloproteinases (MMPs) and their tissue inhibitors, due to secretion by macrophages and lymphocytes, occur in inflammatory processes that disrupt the blood brain barrier. However, neurons and microglia can also secrete these enzymes. AIM: To identify the type of MMP present in the cerebrospinal fluid (CSF) and changes in the expression of tissue inhibitors of metalloproteinases (TIMPs) in patients with HTLV-1 associated tropical spastic paraparesis. PATIENTS AND METHODS: CSF samples from 12 patients with HTLV-1 associated tropical spastic paraparesis and 12 healthy controls were obtained by an atraumatic lumbar puncture. The presence of MMPs was measured by zymography and the relative amounts of TIMPs were measured by immunowestern blot. RESULTS: In the CSF of both controls and patients, a similar gelatinolytic band corresponding to proMMP-2 (latent form) was observed. In 83.3% of patients with HTLV 1 associated tropical spastic paraparesis, the MMP-9 was also present. TIMP-1, TIMP-2 and TIMP-3 were elevated 2.24 +/- 0.72, 3.85 +/- 1.38 and 5.89 +/- 3.4 fold, respectively, in the CSF of patients as compared to controls. CONCLUSIONS: Patients with HTLV-1 associated tropical spastic paraparesis have elevated activity of MMP-9 and levels of TIMPs in the CSF, when compared to healthy controls.

Adult↗
PubMed1997

A family with hereditary spastic paraparesis and epilepsy.

PURPOSE: We describe a family with hereditary spastic paraparesis (HSP) in which 4 of 6 affected members also have epilepsy. METHODS: All family members were examined by 2 neurologists. Four affected and 3 unaffected family members had EEG recordings. Four affected members were investigated for other causes of spastic paraparesis and epilepsy. RESULTS: Epileptic symptoms varied among family members: 1 had complex partial seizures, another had focal myoclonic epilepsy, and 2 had simple partial seizures secondarily generalized. All 4 had clinical or EEG evidence to support a focal origin for the epilepsy, and 2 had photoparoxysal responses on EEG. Symptoms were more severe and occurred earlier in the younger generation, suggesting genetic anticipation in this family. The onset of epilepsy developed simultaneously with, or < or = 18 years before, onset of gait disturbance. Three unaffected family members had normal EEGs. CONCLUSIONS: The association of HSP and epilepsy should no longer be assumed to be fortuitous.

Adolescent↗
PubMed1993

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.

Adult↗
PubMed1997

Human T-cell lymphotropic virus type 1 myositis, peripheral neuropathy, and cerebral white matter lesions in the absence of spastic paraparesis.

BACKGROUND: The human T-cell lymphotropic virus type 1 (HTLV-1) is associated with a chronic, progressive myelopathy termed tropical spastic paraparesis or HTLV-1-associate myelopathy. An increasing number of reports suggest that the spectrum of neurologic diseases associated with HTLV-1 is quite diverse. DESIGN: Case study. SETTING: A university teaching hospital (Ottawa General Hospital, Ottawa, Ontario). RESULTS: Serum creatine kinase levels were elevated (1091 U/L). Antibodies for HTLV-1 were detected by Western blot analysis and confirmed by polymerase chain reaction. Human immunodeficiency virus antibodies were not detected. Findings of nerve conduction studies revealed an axonal neuropathy, while results of needle electromyography were consistent with mixed neuropathic and myopathic changes. Findings of a muscle biopsy supported the presence of polymyositis. Magnetic resonance imaging scans of the brain showed chronic, extensive cerebral white matter involvement of more than 7 years' duration. Treatment with oral steroids resulted in an approximate 40% decrease in serum creatine kinase levels within 1 month and a marked improvement in strength. CONCLUSIONS: A broad spectrum of neurologic disorders is associated with HTLV-1, which may or may not include spastic paraparesis. Patients with myopathies and/or neuropathies of unknown origin who are from areas endemic for HTLV-1 should be screened for this retrovirus, even in the absence of spastic paraparesis.

Aged↗
PubMed1997

Association of late onset spastic paraparesis and dementia: probably an autosomal dominant form of complicated paraplegia.

The hereditary paraplegias are a heterogeneous group of genetic disorders characterized mainly by spastic paraparesis, which may be found as an isolated "pure form" known as Strümpell-Lorrain syndrome, or associated with a wide group of other manifestations [Harding, 1990; McKusick, 1994]. We studied two unrelated families, one with five members and the other with 11 members (over four generations), affected by a syndrome of late onset spastic paraparesis and dementia. Both pedigrees suggest an autosomal dominant pattern of inheritance. However, this cannot be concluded definitely because male-to-male transmission was not seen. Since this disorder has a late age of onset, we still do not know who will become affected in the second, third, and fourth generations. The association of late onset spastic paraparesis and dementia, without other pathological findings, has not been reported and probably represents a distinct entity.

Adult↗
PubMed2001

Variable phenotype of Alzheimer's disease with spastic paraparesis.

A variant form of Alzheimer's disease (AD), in which spastic paraparesis (SP) precedes dementia, is characterised by large, noncored, weakly neuritic Abeta-amyloid plaques resembling cotton wool balls and is caused by genomic deletion of presenilin 1 exon 9. A pedigree with a 5.9 kb exon 9 deletion shows a phenotypic spectrum including subjects with typical AD or with SP and numerous cotton wool plaques. In SP subjects, dementia onset is delayed and modified. This phenotypic variation suggests that modifying factors are associated with exon 9 deletions.

Adult↗
PubMed2005

Conventional magnetic resonance imaging features in patients with tropical spastic paraparesis.

Conventional brain and spinal cord magnetic resonance images were performed in 21 patients with human T-cell lymphotropic virus (HTLV)-1 associated myelopathy/tropical spastic paraparesis, to assess the role of conventional magnetic resonance imaging (MRI) in the disease diagnosis. These patients had no other central nervous system conditions or related risk factors at the time of tropical spastic paraparesis diagnosis. Eleven (52.4%) patients showed nonspecific brain abnormalities on T2-weighted images. The majority (77.2%) of brain abnormalities were located in the deep white matter. A transient contrast-enhancing lesion was identified in the brain of only one patient. In the brain of another patient, 9.0% of the T2-hyperintense lesion load was hypointense on the correspondent T1-weighted images. No differences in terms of demographic, biological, or clinical variables were present between patients with abnormal brain images and those with normal brain magnetic resonance images. Spinal cord T2-weighted images were abnormal in three (14.3%) patients. In one of these three patients, a diffuse but transient edema was found along the entire tract of the spinal cord. White matter lesions were present in the central nervous system of 60% of the cases in this study. However, no correlations between magnetic resonance imaging and clinical findings, and no specificity of lesions were observed. Hence, conventional magnetic resonance imaging is a sensitive but not highly specific tool for diagnosis of tropical spastic paraparesis.

Adult↗
PubMed1989

[A case of symptomatic adrenoleukodystrophy heterozygote manifested by spastic paraparesis of late onset].

A case of symptomatic adrenoleukodystrophy (ALD) heterozygote, manifested by spastic paraparesis, was reported. The patient's 9 year-old grandson had ALD, and her 34 year-old daughter, who was the mother of the case of ALD, was asymptomatic but accompanied by elevation of serum very long chain fatty acid (VLCFA). The patient's gait disturbance appeared at age 62 and that gradually worsened. On admission, she showed spasticity on the lower limbs with bilateral Babinski's reflexes, muscle weakness of the lower limbs and interossei muscles of the hand, and mild sensory disturbance on the distal part of all limbs. Serum VLCFA was markedly elevated. There was no abnormal adrenal function. Sensory and motor nerve conduction velocities were within normal limits. A needle EMG examination disclosed long-duration and high-amplitude potentials. Auditory brainstem response (ABR) revealed elongation of III-V wave intervals bilaterally, and somatosensory evoked potential (SEP) showed delayed N20. Brain CT scan revealed no abnormality but MRI (T2 weighted image) showed mild high intensity areas in the capsula interna and cerebral peduncles. The abnormality of MRI suspected that spastic paraparesis may result from involvement of the corticospinal tract of the cerebrum or brainstem though the symptom has been considered due to the lesion of the spinal cord. Examinations of ABR, SEP and MRI were useful methods to make a diagnosis of symptomatic ALD heterozygote.

Adrenoleukodystrophy↗
PubMed2004

Neuropsychological functions in variant Alzheimer's disease with spastic paraparesis.

Few data exist on the effects of specific Alzheimer's disease (AD)-related mutations on cognitive function. We present neuropsychological test results in eight members of a large kindred with variant Alzheimer's disease (VarAD) due to a deletion of the presenilin 1 (PS-1) gene, encompassing exon 9. The disease was neuropathologically characterized by the presence of large, unusual, "cotton wool" plaques (CWP). Four surviving patients were prospectively tested, and retrospective neuropsychological data were collected from additional four deceased patients. The neuropsychological evaluation was based on tests of verbal and visual memory, abstract thinking, and visuoconstructive and spatial functions. In addition, psychiatric symptoms were evaluated. In four patients, brain glucose metabolism was examined by positron emission tomography (PET). PET showed temporoparietal hypometabolism typical of AD. In addition, variable patterns of hypometabolism (hemispherical asymmetry and occipital accentuation) were related to individual deficits of cognitive performance. However, all these early-onset patients (age range 43-63 years) with a deletion mutation of PS-1 gene showed prominent memory impairment and deficits in visuoconstructive and intellectual functions.

Alzheimer Disease↗