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International Journal of Molecular... Dec 2023Hereditary spastic paraplegia (HSP) is a heterogeneous group of genetically determined diseases, characterised by progressive spastic paraparesis of the lower limbs,...
Hereditary spastic paraplegia (HSP) is a heterogeneous group of genetically determined diseases, characterised by progressive spastic paraparesis of the lower limbs, associated with degeneration of the corticospinal tract and the posterior column of the spinal cord. HSP occurs worldwide and the estimated prevalence is about 1-10/100,000, depending on the geographic localisation. More than 70 genes responsible for HSP have been identified to date, and reports of new potentially pathogenic variants appear regularly. All possible patterns of inheritance (autosomal dominant, autosomal recessive, X-linked and mitochondrial) have been described in families of HSP patients. Among the autosomal recessive forms of HSP (AR-HSP), hereditary spastic paraplegia type 11 is the most common one. We present a patient with diagnosed HSP 11, with a typical clinical picture and characteristic features in additional diagnostic tests.
Topics: Humans; Spastic Paraplegia, Hereditary; Pyramidal Tracts; Mitochondria; Neuroimaging; Mutation
PubMed: 38139357
DOI: 10.3390/ijms242417530 -
Asian Journal of Neurosurgery Sep 2023This article reports the management of a case of a 32-year-old male who presented with progressive weakness in the lower limbs and spastic paraparesis secondary to...
This article reports the management of a case of a 32-year-old male who presented with progressive weakness in the lower limbs and spastic paraparesis secondary to an intramedullary arachnoid cyst (IMAC). For literature review, the authors used the phrase "intramedullary arachnoid cyst" in PubMed search engine. 23 articles describing cases with IMAC were included in this review, with a total of 26 patients. We report a case with long term recurrant intramedullary arachnoid cyst and present a review on spinal intramedullary arachnoid cyst. IMAC is showing bimodal incidence and trending to occur below 10 years and after 30 years. However, rarely, it should be considered in the differential diagnosis of intramedullary cystic lesions. Authors suggest doing laminoplasty or fusion for the pediatric patients to prevent kyphoscoliosis deformity in the long run, but doing early surgery to gain better outcome. Resection of the cyst wall should be done as much as possible; if it could not be achieved, then marsupialization or cysto-subarachnoid shunt should be considered. Aspiration alone or fenestration is not enough to eradicate the cyst. Long-term and prospective studies are recommended to achieve the best treatment options. Review supports early surgical treatment of symptomatic IMACs with resection of the cyst wall as much as possible.
PubMed: 38152523
DOI: 10.1055/s-0043-1774380 -
Journal of Research in Medical Sciences... 2023Human T-cell lymph tropic virus type 1 (HTLV-I)-related myelopathy/tropical spastic paraparesis (TSP) is a progressive inflammatory process affecting the spinal cord... (Review)
Review
Nonpharmacological interventions and outcomes in the management of complications of human T-cell lymphotropic virus type 1-related myelopathy/tropical spastic paraparesis: A systematic review.
BACKGROUND
Human T-cell lymph tropic virus type 1 (HTLV-I)-related myelopathy/tropical spastic paraparesis (TSP) is a progressive inflammatory process affecting the spinal cord that occurs as a result of HTLV 1. The use of nonpharmacological approaches has always been one of the treatment strategies in these patients, but disagreement about these interventions and their results has led to their limited use. Therefore, this study aimed to identify nonpharmacological interventions and their consequences in these patients.
MATERIALS AND METHODS
We followed the Cochrane Handbook for systematic reviews of interventions. The present report is organized according to the preferred reporting items for systematic reviews and meta-analyses. This study was conducted at PubMed, Cochrane Library, Web of Science, and Scopus, among all published studies by December 30, 2021. Keywords were: HTLV-1, Human T-lymph tropic virus 1, HTLV-I-associated myelopathy, HAM/TSP, tropical spastic paraparesis, nonpharmacological intervention, nonpharmacological treatment, massage, physiotherapy, acupuncture, acupressure, and exercise. The quality of the studies was assessed using JADAD.
RESULTS
Of 288 articles, 11 were eligible for data extraction published between 2014 and 2021. 90/9% of studies were randomized clinical trials. 81/8% of articles were of high quality. The total sample size was 253 people, of which 137 (54/15%) were women. Approaches such as exercise and motion therapy, electrotherapy, behavioral therapy, and virtual reality can be used for these patients. With these interventions, results such as improved mobility and balance, physical condition, pain, quality of life, muscle spasticity, maximum inspiratory pressure, and urinary symptoms can be achieved.
CONCLUSION
The most common physical therapy method used in studies was active and passive body movements, which are associated with positive results for patients. Due to the small sample size in this group of studies, it is necessary to conduct more clinical trials for more accurate conclusions. Furthermore, due to the limited number of studies that have used electrical stimulation or combined intervention packages, it is not possible to say with certainty what effect these methods have on patients. It is necessary to conduct more clinical trials.
PubMed: 38510783
DOI: 10.4103/jrms.jrms_300_22 -
BMC Neurology Oct 2023Neurolathyrism is an upper motor neuron disorder characterized by spastic paraparesis, which is caused by the prolonged over-consumption of grass pea. It is a...
Prevalence of Neurolathyrism and its associated factors in Grass pea cultivation areas of Dawunt District, North-eastern Ethiopia; 2022: a community based multilevel analysis.
INTRODUCTION
Neurolathyrism is an upper motor neuron disorder characterized by spastic paraparesis, which is caused by the prolonged over-consumption of grass pea. It is a devastating disease with great impacts on physical, social, mental, and economical health.
OBJECTIVE
To determine the prevalence of neurolathyrism and its associated factors in grass pea cultivation areas of Dawunt wereda.
METHODS
Community based cross-sectional study design was conducted from February 01- March 30, 2021 on 631 Households with a total of 3,350 individuals. Two-stage random sampling technique was used to select participants. Multilevel binary logistic regression was used to identify factors associated with neurolathyrism. Statistical significance was declared at p < 0.05; and AOR with 95% CI was used to interpret the results.
RESULTS
The household and population level prevalence of neurolathyrism in Dawunt district were 9.2% (7.2-11.7%) and 2.4% (2.0-2.3.0%) respectively. Age (AOR = 7.4 ( 2.6-20.6)), male sex (AOR = 7.8 (3.9, 15.4)), and marital status (AOR = 4.0 (1.3-12.8)) were the individual level variables; family size (AOR = 12.6 (3.0-52.8)), annual grass pea production (AOR = 5.0 (2.3-11.0)), ever feeding only grass pea (AOR = 8.8(3.5-22.2)), ever feeding immature seeds of grass pea (AOR = 6.28 (2.80, 14.08)), high grass pea to other cereals mixing ratio (> 3:1) (AOR = 6.1 (1.1, 33.5)) were the household level variables found to have significant association with neurolathyrism.
CONCLUSION
The prevalence of neurolathyrism was found to be high. Ever feeding only grass pea, Grass pea to other cereals mixing ratio (using ratio of 1:1 or more), and Ever feeding immature grass pea seeds were the modifiable risk factors for neurolathyrism.
Topics: Humans; Male; Lathyrism; Lathyrus; Pisum sativum; Multilevel Analysis; Ethiopia; Cross-Sectional Studies; Prevalence
PubMed: 37798732
DOI: 10.1186/s12883-023-03379-0 -
Tomography (Ann Arbor, Mich.) Oct 2023Conus medullaris syndrome (CMS) is a distinctive spinal cord injury (SCI), which presents with varying degrees of upper motor neuron signs (UMNS) and lower motor neuron... (Review)
Review
INTRODUCTION
Conus medullaris syndrome (CMS) is a distinctive spinal cord injury (SCI), which presents with varying degrees of upper motor neuron signs (UMNS) and lower motor neuron signs (LMNS). Herein, we present a case with a burst fracture injury at the proximal Conus Medullaris (CM).
CASE PRESENTATION
A 48-year-old Taiwanese male presenting with lower back pain and paraparesis was having difficulty standing independently after a traumatic fall. An Imaging survey showed an incomplete D burst fracture of the T12 vertebra. Posterior decompression surgery was subsequently performed. However, spasticity and back pain persisted for four months after surgical intervention. Follow-up imaging with single photon emission computed tomography (SPECT) and a whole body bone scan both showed an increased uptake in the T12 vertebra.
CONCLUSION
The high-riding injury site for CMS is related to a more exclusive clinical representation of UMNS. Our case's persistent UMNS and scintigraphy findings during follow-up showcase the prolonged recovery period of a UMN injury. In conclusion, our study provides a different perspective on approaching follow-up for CM injuries, namely using scientigraphy techniques to confirm localization of persistent injury during the course of post-operative rehabilitation. Furthermore, we also offered a new technique for analyzing the location of lumbosacral injuries, and that is to measure the location of the injury relative to the tip of the CM. This, along with clinical neurological examination, assesses the extent to which the UMN is involved in patients with CMS, and is possibly a notable predictive tool for clinicians for the regeneration time frame and functional outcome of patients with lumbosacral injuries in the future.
Topics: Humans; Male; Middle Aged; Spinal Cord Compression; Cauda Equina Syndrome; Thoracic Vertebrae
PubMed: 37987342
DOI: 10.3390/tomography9060156 -
Iranian Journal of Medical Sciences May 2024Human T-cell Leukemia Virus type-1 (HTLV-1) -associated myelopathy causes sufferers to experience changes in several aspects of their lives. Gaining a deeper...
BACKGROUND
Human T-cell Leukemia Virus type-1 (HTLV-1) -associated myelopathy causes sufferers to experience changes in several aspects of their lives. Gaining a deeper understanding of these changes can help healthcare professionals improve care, enhance strategic decision-making, meet expectations, and manage patients effectively. However, there is no information about the experience and problems of patients with HTLV-1-associated myelopathy/tropical spastic paraparesis in Iran. Therefore, this study aimed to explain the lived experience of patients with HTLV-1-associated myelopathy/tropical spastic paraparesis.
METHODS
This qualitative study used hermeneutic phenomenology in 2022 in Mashhad, Iran. Participants were selected using purposeful sampling. Data were collected through 21 semi-structured in-depth interviews with 20 eligible patients with HTLV-1-associated myelopathy/tropical spastic paraparesis. The data were analyzed in MAXQDA/2020 using the six stages proposed by Van Manen.
RESULTS
The main concept of "Reduced self-sufficiency and social dignity" emerged from the narratives of the patients, which included three main categories "Disruption of desirable personal and social life", "reduced perception of role competencies", and "obligatory unpleasant lifestyle changes".
CONCLUSION
HTLV-1-associated myelopathy/tropical spastic paraparesis slowly makes patients feel insufficient and causes a sense of degradation in dignity. The disease can fundamentally change personal and social life. Thus, due to its incurability and progressiveness, palliative care should be provided to them to live with dignity.
Topics: Humans; Paraparesis, Tropical Spastic; Male; Female; Middle Aged; Adult; Qualitative Research; Iran; Human T-lymphotropic virus 1; Aged
PubMed: 38751877
DOI: 10.30476/IJMS.2023.97867.2973 -
Journal of Neurosurgery. Case Lessons Dec 2023Spinal cord diffuse midline glioma, H3K27-altered, is an extremely rare entity with a poor prognosis. However, its optimal treatment remains poorly defined. Although...
BACKGROUND
Spinal cord diffuse midline glioma, H3K27-altered, is an extremely rare entity with a poor prognosis. However, its optimal treatment remains poorly defined. Although cordectomy was introduced in the early 20th century, its efficacy has been questioned and shrouded behind the scenes.
OBSERVATIONS
A 76-year-old male with recent-onset paraparesis of the lower extremities and paresthesia presented to our outpatient clinic. Magnetic resonance imaging revealed an intra-axial spinal cord tumor extending from T12 to L2. The patient underwent laminectomy and partial tumor resection, and the surgical specimen was histologically diagnosed as a diffuse midline glioma, H3K27-altered. Although standard chemoradiotherapy was implemented, the patient experienced local tumor recurrence 2 years later and underwent cordectomy at T9. The patient was alive at the 4-year follow-up after cordectomy without tumor recurrence. According to the literature, patients with lesions in the lower thoracic cord below T8 achieved a longer survival than those with lesions in the upper thoracic cord above T5.
LESSONS
Cordectomy benefits selected cases of high-grade spinal cord gliomas. Maximal prevention of cerebrospinal fluid dissemination by tumor cells is indisputably important, and tumors located below the lower thoracic spine may be the key to success in establishing a long-term prognosis after cordectomy.
PubMed: 38109730
DOI: 10.3171/CASE23296 -
Journal of Veterinary Internal Medicine 2024Vertebral infections, including vertebral osteomyelitis, septic physitis, and discospondylitis, are rarely reported in goats, and when reported, have been largely...
BACKGROUND
Vertebral infections, including vertebral osteomyelitis, septic physitis, and discospondylitis, are rarely reported in goats, and when reported, have been largely limited to necropsy case reports.
OBJECTIVE
Describe clinical findings and outcome in goats with vertebral infections evaluated by computed tomography (CT).
ANIMALS
Five goats with vertebral osteomyelitis, septic physitis, and discospondylitis evaluated by CT.
METHODS
Retrospective case series.
RESULTS
The most common presenting complaints were progressive weakness, paresis and recumbency. Three goats were tetraparetic and 2 goats had pelvic limb paraparesis. Clinicopathologic findings included leukocytosis, mature neutrophilia, and hyperfibrinogenemia. The most common vertebrae affected were C7-T1. All 5 goats had discospondylitis with or without vertebral osteomyelitis and septic physitis. Computed tomographic evidence of spinal cord compression was present in 4/5 goats. Medical management (antimicrobials, physical therapy, analgesia, supportive care) was attempted in 4 goats, and 1 goat was euthanized at the time of diagnosis. All 4 goats that were treated regained ambulatory ability and survived to hospital discharge.
CONCLUSIONS AND CLINICAL IMPORTANCE
Despite severity of CT imaging findings, goats with discospondylitis, septic physitis, and vertebral osteomyelitis can successfully return to ambulatory function. Additional studies are required to determine ideal treatment regimens.
Topics: Animals; Goats; Goat Diseases; Osteomyelitis; Retrospective Studies; Female; Tomography, X-Ray Computed; Male; Discitis; Spondylitis; Spinal Diseases
PubMed: 38483064
DOI: 10.1111/jvim.17035 -
Journal of Medical Case Reports Jun 2024Hepatic myelopathy is a very rare neurological complication of chronic liver disease. Patients habitually present with progressive pure motor spastic paraparesis. This...
BACKGROUND
Hepatic myelopathy is a very rare neurological complication of chronic liver disease. Patients habitually present with progressive pure motor spastic paraparesis. This neurological dysfunction is almost always due to cirrhosis and portocaval shunt, either surgical or spontaneous.
CASES REPORT
We report two cases of a 57-year-old man and a 37-year-old woman with progressive spastic paraparesis linked to cirrhosis and portal hypertension. The two patients are of Tunisian origin (north Africa). Magnetic resonance imaging of the spinal cord of two patients was normal, while brain magnetic resonance imaging showed a T2 hypersignals of the pallidums. These signs, in favor of hepatic encephalopathy in the two patients with cirrhosis with isolated progressive spastic paraparesis without bladder or sensory disorders, help to retain the diagnosis of hepatic myelopathy.
CONCLUSION
Hepatic myelopathy is a severe and debilitating neurological complication of chronic liver disease. The pathogenesis is misunderstood and seems to be multifactorial, including the selective neurotoxic role both of ammonia and other pathogenic neurotoxins. Usually a pathological brain magnetic resonance imaging showing a hepatic encephalopathy was documented, contrasting with a normal spinal cord magnetic resonance imaging that contributed to diagnosis of hepatic myelopathy. Conservative therapies such as ammonia-lowering measures, diet supplementation, antispastic drugs, and endovascular shunt occlusion show little benefit in improving disease symptoms. Liver transplantation performed at early stage can prevent disease progression and could probably allow for recovery.
Topics: Humans; Female; Middle Aged; Male; Hepatic Encephalopathy; Adult; Magnetic Resonance Imaging; Spinal Cord Diseases; Liver Cirrhosis; Paraparesis, Spastic; Hypertension, Portal; Chronic Disease
PubMed: 38880918
DOI: 10.1186/s13256-024-04495-2 -
The Canadian Veterinary Journal = La... Nov 2023A 16-month-old neutered male domestic shorthair cat weighing 2.7 kg was referred for further evaluation of acute generalized muscle weakness and paraparesis after a...
A 16-month-old neutered male domestic shorthair cat weighing 2.7 kg was referred for further evaluation of acute generalized muscle weakness and paraparesis after a long-standing history of polyuria-polydipsia. The diagnosis of hypodipsic/adipsic hypernatremia relied on the key findings of absent spontaneous drinking despite hypernatremia and a hyperosmolar state (444.8 mOsm/kg, reference interval 280 to 310 mOsm/kg). Brain MRI revealed severe multifocal anatomic anomalies of the rostral calvarium and the forebrain, suggestive of encephaloclastic porencephaly. Involvement of the thalamic and hypothalamic regions could have been responsible for the cat's adipsic hypernatremia. The unique aspects of this case were the rare description of central nervous system disease leading to hypodipsia, and the history of chronic polydipsia before the acute onset of hypodipsia. Key clinical message: Multifocal abnormalities of the forebrain can present with polyuria-polydipsia syndrome, hypodipsia/adipsia, or both, depending on the stage of the disease. This likely happens when the hypothalamic and thalamic regions are affected, since they regulate antidiuretic hormone release and thirst, respectively.
Topics: Male; Cats; Animals; Hypernatremia; Polyuria; Thirst; Polydipsia; Craniocerebral Trauma; Cat Diseases
PubMed: 37915774
DOI: No ID Found