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Medicine Feb 2022Here, we describe a series of 7 patients who presented with acute paraparesis due to anterior communicating artery aneurysm rupture. This study aimed to assess the...
Here, we describe a series of 7 patients who presented with acute paraparesis due to anterior communicating artery aneurysm rupture. This study aimed to assess the clinical and radiological factors associated with acute paraparesis syndrome caused by subarachnoid hemorrhage (SAH).Between June 2005 and December 2012, our institution consecutively treated 210 patients with anterior communicating aneurysm rupture within 24 hours after ictus. We divided the patients into 2 groups based on the presence (n = 7) and absence (n = 203) of acute paraparesis after anterior communicating aneurysm rupture.Diffusion-weighted magnetic resonance imaging revealed high intensity in the medial aspects of the bilateral frontal lobes in 3 patients. The mean third ventricular distance at the time of admission was 9.2 mm (range, 8-12.5 mm), and the mean bicaudate distance was 33.9 mm (range, 24-39 mm). There was a significant difference in the bicaudate distance (P = .001) and third ventricle distance (P = .001) between the 2 groups. Acute hydrocephalus and global cerebral edema (GCE) were confirmed radiologically in all patients in the acute paraparesis group. The presence of acute hydrocephalus (P = .001) and GCE (P = .003) were significantly different between the groups.Acute paraparesis syndrome after SAH is transient and gradually improves if the patient does not develop severe vasospasm. The present study demonstrates that acute paraparesis after SAH is associated with acute hydrocephalus and GCE.
Topics: Aneurysm, Ruptured; Humans; Hydrocephalus; Intracranial Aneurysm; Paraparesis; Stroke; Subarachnoid Hemorrhage; Syndrome
PubMed: 35119048
DOI: 10.1097/MD.0000000000028792 -
Frontiers in Microbiology 2020HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP) is a neuropathological disorder in 1-3% of individuals infected with Human T-lymphotropic virus 1... (Review)
Review
HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP) is a neuropathological disorder in 1-3% of individuals infected with Human T-lymphotropic virus 1 (HTLV-1). This condition is characterized by progressive spastic lower limb weakness and paralysis, lower back pain, bladder incontinence, and mild sensory disturbances resembling spinal forms of multiple sclerosis. This disease also causes chronic disability and is therefore associated with high health burden in areas where HTLV-1 infection is endemic. Despite various efforts in understanding the virus and discovery of novel diagnostic markers, and cellular and viral interactions, HAM/TSP management is still unsatisfactory and mainly focused on symptomatic alleviation, and it hasn't been explained why only a minority of the virus carriers develop HAM/TSP. This comprehensive review focuses on host and viral factors in association with immunopathology of the disease in hope of providing new insights for drug therapies or other forms of intervention.
PubMed: 33414779
DOI: 10.3389/fmicb.2020.614940 -
Gene Aug 2022The recent coronavirus disease 2019 (COVID-19) significantly affected many people worldwide, especially those with underlying diseases. While some people with underlying... (Review)
Review
The recent coronavirus disease 2019 (COVID-19) significantly affected many people worldwide, especially those with underlying diseases. While some people with underlying illnesses, including cardiovascular diseases, are more vulnerable to develop severe COVID-19, other populations, including people who have autoimmune diseases, may develop severe diseases similar to the general population. The severity and outcome of COVID-19 are reviewed in individuals with underlying viral diseases, including acquired immune deficiency syndrome and hepatitis, however, some infectious diseases, including human T-lymphotropic virus type 1 (HTLV-1) diseases, is under-reported in the literature. HTLV-1 is a sexually transmitted disease that is endemic in some parts of the world. Infected patients may develop clinical symptoms of HTLV-1 associated myelopathy / tropical spastic paraparesis (HAM/TSP) and adult T cell leukemia (ATL) or may remain asymptomatic during their life. To the best of our knowledge, no clinical studies evaluate the severity and outcomes of SARS-CoV-2 infection in HTLV-1 infected patients. We aimed to review the pathogenesis of both of these viral infections and discuss their similarities in provoking immune responses. Although HTLV-1 infected patients may have had variable degrees of inflammation and immune system dysregulation, the available data is limited to conclude that HTLV-1 infected patients may be more vulnerable to developing severe COVID-19 in contrast to the general population.
Topics: Adult; COVID-19; Coinfection; Human T-lymphotropic virus 1; Humans; Paraparesis, Tropical Spastic; SARS-CoV-2
PubMed: 35569772
DOI: 10.1016/j.gene.2022.146550 -
Viruses Sep 2022Human T-cell Leukemia virus type-1 (HTLV-1) causes adult T-cell leukemia/lymphoma (ATLL), HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and other... (Review)
Review
Human T-cell Leukemia virus type-1 (HTLV-1) causes adult T-cell leukemia/lymphoma (ATLL), HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and other inflammatory diseases. High viral DNA burden (VL) in peripheral blood mononuclear cells is a documented risk factor for ATLL and HAM/TSP, and patients with HAM/TSP have a higher VL in cerebrospinal fluid than in peripheral blood. VL alone is not sufficient to differentiate symptomatic patients from healthy carriers, suggesting the importance of other factors, including host immune response. HTLV-1 infection is life-long; CD4-infected cells are not eradicated by the immune response because HTLV-1 inhibits the function of dendritic cells, monocytes, Natural Killer cells, and adaptive cytotoxic CD8 responses. Although the majority of infected CD4 T-cells adopt a resting phenotype, antigen stimulation may result in bursts of viral expression. The antigen-dependent "on-off" viral expression creates "conditional latency" that when combined with ineffective host responses precludes virus eradication. Epidemiological and clinical data suggest that the continuous attempt of the host immunity to eliminate infected cells results in chronic immune activation that can be further exacerbated by co-morbidities, resulting in the development of severe disease. We review cell and animal model studies that uncovered mechanisms used by HTLV-1 to usurp and/or counteract host immunity.
Topics: Adult; Humans; Human T-lymphotropic virus 1; Leukemia-Lymphoma, Adult T-Cell; Leukocytes, Mononuclear; DNA, Viral; Paraparesis, Tropical Spastic; Vaccines
PubMed: 36298639
DOI: 10.3390/v14102084 -
Acta Neurologica Belgica Feb 2021Spinal adhesive arachnoiditis is a rare pathology involving pia mater of the spinal cord and nerve roots. It can potentially lead to disability-many patients end up... (Review)
Review
Spinal adhesive arachnoiditis is a rare pathology involving pia mater of the spinal cord and nerve roots. It can potentially lead to disability-many patients end up wheelchair-bound due to subsequent paraparesis. It is an infrequent but possible cause of lower extremities weakness in patients with a history of spinal surgery, epidural anaesthesia, myelography or spinal tumors. Three patients, one male and two females, admitted to our unit due to paraparesis presented at least one of the above mentioned risk factors. Each of them had a severe course of illness-progressive paresis of lower extremities. All above cases were diagnosed with spinal adhesive arachnoiditis confirmed with Magnetic Resonance Imaging (MRI) scan-the most sensitive and specific diagnostic tool. Despite conservative treatment and intensive rehabilitation none of the presented patients preserved the ability to mobilise independently. Considering spinal adhesive arachnoiditis in patients with paraparesis and history of typical risk factors should be included in clinical diagnostic procedure.
Topics: Adult; Arachnoiditis; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Muscle Weakness; Thoracic Vertebrae; Tissue Adhesions
PubMed: 32833147
DOI: 10.1007/s13760-020-01431-1 -
BMJ Case Reports Feb 2019Spinal arachnoid cysts (SAC) are rare in isolation and the exact aetiology is still debated. Primary (congenital) cysts are caused by structural abnormalities in the...
Spinal arachnoid cysts (SAC) are rare in isolation and the exact aetiology is still debated. Primary (congenital) cysts are caused by structural abnormalities in the arachnoid layer and largely affect the thoracic region. Secondary cysts are induced by a multitude of factors, infection, trauma or iatrogenic response, and can affect any level of the spinal cord. While subarachnoid haemorrhage (SAH) is a relatively common condition with significant repercussions, it is extremely uncommonly associated with SAC. When present, it may develop in the months and years after the original bleed, giving rise to new neurological symptoms. Prompt treatment is needed to halt or reverse the worsening of symptoms and questions are still being asked about how best to approach this condition. A 42-year-old man presented with chronic back pain, severe worsening ataxia and numbness below the umbilicus, 7 months after treatment for a World Federation of Neurosurgical Societies grade five (WFNS V) SAH. Imaging revealed a SAC extending from T12 to L4 and causing thecal compression. This was treated with a L3 laminectomy andmarsupialisation. An improvement in neurological function was observed at 6 months. Aetiology of the SAC and its association with SAH are discussed and a review of the relevant literature is provided.
Topics: Adult; Arachnoid Cysts; Back Pain; Humans; Laminectomy; Magnetic Resonance Imaging; Male; Paraparesis; Spinal Cord; Spinal Cord Compression; Spinal Cord Diseases; Subarachnoid Hemorrhage; Treatment Outcome
PubMed: 30819681
DOI: 10.1136/bcr-2018-227666 -
JCI Insight Feb 2021In this study, we examined and characterized disease-specific TCR signatures in cerebrospinal fluid (CSF) of patients with HTLV-1-associated myelopathy/tropical spastic...
In this study, we examined and characterized disease-specific TCR signatures in cerebrospinal fluid (CSF) of patients with HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). TCR β libraries using unique molecular identifier-based methodologies were sequenced in paired peripheral blood mononuclear cells (PBMCs) and CSF cells from HAM/TSP patients and normal healthy donors (NDs). The sequence analysis demonstrated that TCR β repertoires in CSF of HAM/TSP patients were highly expanded and contained both TCR clonotypes shared with PBMCs and uniquely enriched within the CSF. In addition, we analyzed TCR β repertoires of highly expanded and potentially immunopathologic HTLV-1 Tax11-19-specific CD8+ T cells from PBMCs of HLA-A*0201+ HAM/TSP and identified a conserved motif (PGLAG) in the CDR3 region. Importantly, TCR β clonotypes of expanded clones in HTLV-1 Tax11-19-specific CD8+ T cells were also expanded and enriched in the CSF of the same patient. These results suggest that exploring TCR repertoires of CSF and antigen-specific T cells may provide a TCR repertoire signature in virus-associated neurologic disorders.
Topics: CD8-Positive T-Lymphocytes; Clone Cells; HTLV-I Infections; Human T-lymphotropic virus 1; Humans; Leukocytes, Mononuclear; Nervous System Diseases; Paraparesis, Tropical Spastic; Receptors, Antigen, T-Cell; Receptors, Antigen, T-Cell, alpha-beta
PubMed: 33616082
DOI: 10.1172/jci.insight.144869 -
PloS One 2019Vestibular Myogenic Evoked Potential (VEMP) evaluates vestibulo-ocular and vestibulo-collic reflexes involved in the function of the otolithic organs and their afferent...
PURPOSE
Vestibular Myogenic Evoked Potential (VEMP) evaluates vestibulo-ocular and vestibulo-collic reflexes involved in the function of the otolithic organs and their afferent pathways. We compared the results of cervical and ocular VEMP in HTLV-1 associated myelopathy (HAM) and HTLV-1-asymptomatic infection.
PARTICIPANTS AND METHODS
This cross-sectional study included 52 HTLV-1-infected individuals (26 HAM and 26 asymptomatic carriers) and 26 seronegative controls. The groups were similar regarding age and gender. Participants underwent simultaneous ocular and cervical VEMP. The stimulus to generate VEMP was a low-frequency tone burst sound tone burst, with an intensity of 120 decibels normalized hearing level, bandpass filter from 10 to 1,500 Hertz (Hz), with 100 stimuli at 500 Hz and 50 milliseconds recording time. The latencies of the electrophysiological waves P13 and N23 for cervical VEMP and N10 and P15 waves for ocular VEMP were compared among the groups. The absence or delay of the electrophysiological waves were considered abnormal results.
RESULTS
Ocular VEMP was similar among the groups for N10 (p = 0.375) and different for P15 (p≤0.001). Cervical VEMP was different for P13 (p = 0.001) and N23 (p = 0.003). About ocular VEMP, in the HTLV-1-asymptomatic group, normal waves were found in 23(88.5%) individuals; in HAM group, normal waves were found in 7(26.9%). About cervical VEMP, 18(69.2%) asymptomatic carriers presented normal waves and only 3(11.5%) patients with HAM presented normal waves. Abnormalities in both VEMPs were found in 1(3.8%) asymptomatic carrier and in 16(61.5%) patients with HAM.
CONCLUSION
Neurological impairment in HAM was not restricted to the spinal cord. The mesencephalic connections, tested by ocular VEMP, have been also altered. Damage of the oculomotor system, responsible for eye stabilization during head and body movements, may explain why dizziness is such a frequent complaint in HAM.
Topics: Acoustic Stimulation; Adult; Cross-Sectional Studies; Eye; Female; HTLV-I Infections; Human T-lymphotropic virus 1; Humans; Male; Middle Aged; Muscle Development; Nervous System Diseases; Paraparesis, Tropical Spastic; Vestibular Evoked Myogenic Potentials; Vestibule, Labyrinth
PubMed: 31881022
DOI: 10.1371/journal.pone.0217327 -
Neurology Apr 2020To identify the etiology of an outbreak of spastic paraparesis among women and children in the Western Province of Zambia suspected to be konzo.
OBJECTIVE
To identify the etiology of an outbreak of spastic paraparesis among women and children in the Western Province of Zambia suspected to be konzo.
METHODS
We conducted an outbreak investigation of individuals from Mongu District, Western Province, Zambia, who previously developed lower extremity weakness. Cases were classified with the World Health Organization definition of konzo. Active case finding was conducted through door-to-door evaluation in affected villages and sensitization at local health clinics. Demographic, medical, and dietary history was used to identify common exposures in all cases. Urine and blood specimens were taken to evaluate for konzo and alternative etiologies.
RESULTS
We identified 32 cases of konzo exclusively affecting children 6 to 14 years of age and predominantly females >14 years of age. Fourteen of 15 (93%) cases ≥15 years of age were female, 11 (73%) of whom were breastfeeding at the time of symptom onset. Cassava was the most commonly consumed food (median [range] 14 [4-21] times per week), while protein-rich foods were consumed <1 time per week for all cases. Of the 30 patients providing urine specimens, median thiocyanate level was 281 (interquartile range 149-522) μmol/L, and 73% of urine samples had thiocyanate levels >136 μmol/L, the 95th percentile of the US population in 2013 to 2014.
CONCLUSION
This investigation revealed the first documented cases of konzo in Zambia, occurring in poor communities with diets high in cassava and low in protein, consistent with previous descriptions from neighboring countries.
Topics: Adolescent; Age Factors; Breast Feeding; Child; Cyanides; Diet; Disease Outbreaks; Female; Humans; Male; Manihot; Muscle Weakness; Paraparesis, Spastic; Protein Deficiency; Rain; Seasons; Thiocyanates; Young Adult; Zambia
PubMed: 32127386
DOI: 10.1212/WNL.0000000000009017 -
Viruses Jan 2022Corticosteroids are most commonly used to treat HTLV-1-associated myelopathy (HAM); however, their clinical efficacy has not been tested in randomized clinical trials.... (Randomized Controlled Trial)
Randomized Controlled Trial
Corticosteroids are most commonly used to treat HTLV-1-associated myelopathy (HAM); however, their clinical efficacy has not been tested in randomized clinical trials. This randomized controlled trial included 8 and 30 HAM patients with rapidly and slowly progressing walking disabilities, respectively. Rapid progressors were assigned (1:1) to receive or not receive a 3-day course of intravenous methylprednisolone in addition to oral prednisolone therapy. Meanwhile, slow progressors were assigned (1:1) to receive oral prednisolone or placebo. The primary outcomes were a composite of ≥1-grade improvement in the Osame Motor Disability Score or ≥30% improvement in the 10 m walking time (10 mWT) at week 2 for rapid progressors and changes from baseline in 10 mWT at week 24 for slow progressors. In the rapid progressor trial, all four patients with but only one of four without intravenous methylprednisolone achieved the primary outcome ( = 0.14). In the slow progressor trial, the median changes in 10 mWT were -13.8% (95% CI: -20.1--7.1; < 0.001) and -6.0% (95% CI: -12.8-1.3; = 0.10) with prednisolone and placebo, respectively ( for between-group difference = 0.12). Whereas statistical significance was not reached for the primary endpoints, the overall data indicated the benefit of corticosteroid therapy. (Registration number: UMIN000023798, UMIN000024085).
Topics: Adrenal Cortex Hormones; Aged; Disabled Persons; Female; Human T-lymphotropic virus 1; Humans; Male; Methylprednisolone; Middle Aged; Motor Disorders; Paraparesis, Tropical Spastic; Prednisolone; Prospective Studies; Treatment Outcome
PubMed: 35062340
DOI: 10.3390/v14010136