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Clinical Infectious Diseases : An... Sep 2023Human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic neurological condition characterized by progressive...
BACKGROUND
Human T-lymphotropic virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic neurological condition characterized by progressive myelopathic symptoms including spasticity, pain, weakness, and urinary symptoms, without proven treatments. Mogamulizumab (MOG) is a monoclonal antibody that binds CCR4 and leads to the clearance of HTLV-1-infected CCR4+ cells. A phase 1-2a study in Japan evaluated MOG for the treatment of HAM/TSP and reported decreases in HTLV-1 proviral load and neuroinflammatory markers, with clinical improvement in some participants.
METHODS
We administered MOG 0.1 mg/kg every 8 weeks to individuals with HAM/TSP as a compassionate and palliative treatment. Patients who received MOG had (1) a positive peripheral HTLV-1 antibody, (2) progressive myelopathic symptoms, and (3) a diagnosis of HAM/TSP.
RESULTS
Four female patients, ages 45-68, received MOG (range, 2-6 infusions) between 1 November 2019 and 30 November 2022. Two patients with <3 years of symptoms had milder disease, with Osame scores <4. The other 2, with >7 years of symptoms, had Osame scores >5. One patient, with 6 total treatments, received dose-reduced MOG after she developed a rash at the initial dose. The 2 patients with milder baseline disease reported symptomatic improvement and saw reductions in Osame and/or modified Ashworth scale scores during follow-up. The other 2 patients showed no improvement. All 4 developed rashes after receiving MOG-a treatment-limiting event in some cases.
CONCLUSIONS
Clinical trials are needed including diverse patient populations to assess the potential role of MOG for HAM/TSP. Our findings may help inform the development of these trials.
Topics: Humans; Female; Paraparesis, Tropical Spastic; Human T-lymphotropic virus 1; Antibodies, Monoclonal, Humanized; Exanthema; Viral Load
PubMed: 37157862
DOI: 10.1093/cid/ciad281 -
Journal of the American Veterinary... Jul 2023
Topics: Cats; Animals; Urination; Defecation; Behavior, Animal; Paraparesis; Cat Diseases
PubMed: 36735502
DOI: 10.2460/javma.22.12.0564 -
Molecular Neurobiology Jul 2023Human T lymphotropic virus-associated myelopathy/tropical spastic paraparesis (HTLV/TSP), also known as HTLV-associated myelopathy/tropical spastic paraparesis... (Review)
Review
Human T lymphotropic virus-associated myelopathy/tropical spastic paraparesis (HTLV/TSP), also known as HTLV-associated myelopathy/tropical spastic paraparesis (HAM/TSP), and multiple sclerosis (MS) are chronic debilitating diseases of the central nervous system; although the etiology of which is different, similarities have been observed between these two demyelinating diseases, especially in clinical manifestation and immunopathogenesis. Exorbitant response of the immune system to the virus and neurons in CNS is the causative agent of HAM/TSP and MS, respectively. Helper T lymphocyte-17 cells (Th17s), a component of the immune system, which have a proven role in immunity and autoimmunity, mediate protection against bacterial/fungal infections. The role of these cells has been reviewed in several CNS diseases. A pivotal role for Th17s is presented in demyelination, even more axial than Th1s, during MS. The effect of Th17s is not well determined in HTLV-1-associated infections; however, the evidence that we have supplied in this review illustrates the attendance, also the role of Th17 cells during HAM/TSP. Furthermore, for better conception concerning the trace of these cells in HAM/TSP, a comparative characterization with MS, the resembling disease, has been applied here.
Topics: Humans; Paraparesis, Tropical Spastic; Human T-lymphotropic virus 1; Multiple Sclerosis; Th17 Cells; Interleukin-17
PubMed: 36947318
DOI: 10.1007/s12035-023-03303-0 -
Journal of Neurosurgery. Case Lessons Sep 2023Syringomyelia is a neurological disorder that is caused by abnormal cerebrospinal fluid flow or circulation. It is an incidental finding in most cases, predominantly...
BACKGROUND
Syringomyelia is a neurological disorder that is caused by abnormal cerebrospinal fluid flow or circulation. It is an incidental finding in most cases, predominantly presenting with sensory symptoms of insensitivity to pain and temperature. Spinal ossified ligamentum flavum (OLF) leading to syringomyelia is one of the rare causes. The authors report an unusual case of syringomyelia due to a thoracic OLF.
OBSERVATIONS
A 54-year-old female presented with backache, difficulty walking, spasticity in the bilateral lower limbs, tingling sensation in the bilateral lower limbs, and paraparesis for 5 years. Her radiological investigations were suggestive of an OLF causing a syrinx. She underwent laminectomy, and her syrinx resolved on subsequent follow-up.
LESSONS
A syrinx due to a single-level OLF is rare, and this uncommon cause should be kept in mind while formulating treatment plans.
PubMed: 37728291
DOI: 10.3171/CASE23340 -
Journal of Craniovertebral Junction &... 2024Lymphangiomas are benign hamartomas in the spectrum of lymphatic malformations, exhibiting multifaceted clinical features. Spinal involvement is exceedingly rare, with... (Review)
Review
PURPOSE
Lymphangiomas are benign hamartomas in the spectrum of lymphatic malformations, exhibiting multifaceted clinical features. Spinal involvement is exceedingly rare, with only 35 cases reported to date. Both due to their rarity and chameleonic radiologic features, spinal lymphangiomas (SLs) are usually misdiagnosed; postoperatively, surgeons are thus confronted with an unexpected histopathological diagnosis with sparse pertinent literature and no treatment guidelines available.
METHODS
Here, we report the case of a 67-year-old female who underwent surgery for a T6-T7 epidural SL with transforaminal extension, manifesting with spastic paraparesis. Then, we present the results of the first systematic review of the literature on this subject, delineating the clinical and imaging features and the therapeutic implications of this rare disease entity.
RESULTS
Our patient was treated with T6-T7 hemilaminectomy and resection of the epidural mass, with complete recovery of her neurological picture. No recurrence was evident at 18 months. In the literature, 35 cases of SL were reported that can be classified as vertebral SL (n = 18), epidural SL (n = 10), intradural SL (n = 3), or intrathoracic lymphangiomas with secondary spinal involvement (n = 4). Specific treatment strategies (both surgical and nonsurgical) were adopted in relation to each of these categories.
CONCLUSION
Gathering knowledge about SL is fundamental to promote both correct preoperative identification and appropriate perioperative management of this rare disease entity. By reviewing the literature and discussing an exemplary case, we delineate a framework that can guide surgeons facing such an unfamiliar diagnosis.
PubMed: 38644908
DOI: 10.4103/jcvjs.jcvjs_125_23 -
Annals of Surgery Aug 2023To assess outcomes of fenestrated-branched endovascular aortic repair (FB-EVAR) of Extent I-III thoracoabdominal aortic aneurysms (TAAAs) without prophylactic... (Review)
Review
OBJECTIVE
To assess outcomes of fenestrated-branched endovascular aortic repair (FB-EVAR) of Extent I-III thoracoabdominal aortic aneurysms (TAAAs) without prophylactic cerebrospinal fluid drainage (CSFD).
BACKGROUND
Prophylactic CSFD has been routinely used during endovascular TAAA repair, but concerns about major drain-related complications have led to revising this paradigm.
METHODS
We reviewed a multicenter cohort of 541 patients treated for Extent I-III TAAAs by FB-EVAR without prophylactic CSFD. Spinal cord injury (SCI) was graded as ambulatory (paraparesis) or nonambulatory (paraplegia). Endpoints were any SCI, permanent paraplegia, response to rescue treatment, major drain-related complications, mortality, and patient survival.
RESULTS
There were 22 Extent I, 240 Extent II and 279 Extent III TAAAs. Thirty-day mortality was 3%. SCI occurred in 45 patients (8%), paraparesis occurring in 23 (4%) and paraplegia in 22 patients (4%). SCI was more common in patients with Extent I-II compared with Extent III TAAAs (12% vs. 5%, P =0.01). Rescue treatment included permissive hypertension in all patients, with CSFD in 22 (4%). Symptom improvement was noted in 73%. Twelve patients (2%) had permanent paraplegia. Two patients (0.4%) had major drain-related complications. Independent predictors for SCI by multivariate logistic regression were sustained perioperative hypotension [odds ratio (OR): 4.4, 95% confidence interval (95% CI): 1.7-11.1], patent collateral network (OR: 0.3, 95% CI: 0.1-0.6), and total length of aortic coverage (OR: 1.05, 95% CI: 1.01-1.10). Patient survival at 3 years was 72%±3%.
CONCLUSION
FB-EVAR of Extent I-III TAAAs without CSFD has low mortality and low rates of permanent paraplegia (2%). SCI occurred in 8% of patients, and rescue treatment improved symptoms in 73% of them.
Topics: Humans; Aortic Aneurysm, Thoracic; Endovascular Procedures; Risk Factors; Aortic Aneurysm, Abdominal; Spinal Cord Injuries; Blood Vessel Prosthesis Implantation; Paraplegia; Cerebrospinal Fluid Leak; Aortic Aneurysm, Thoracoabdominal; Drainage; Treatment Outcome; Retrospective Studies; Multicenter Studies as Topic
PubMed: 35925761
DOI: 10.1097/SLA.0000000000005653 -
Infectious Disease Reports Aug 2023To achieve the objective of this study, we conducted a narrative review on physical therapeutic modalities applied to prevent functional losses associated with human... (Review)
Review
To achieve the objective of this study, we conducted a narrative review on physical therapeutic modalities applied to prevent functional losses associated with human T-lymphotropic virus 1 (HTLV-1) infections to promote health education and viable and accessible alternatives in the development of health education technology adapted to the home environment. This study comprised a qualitative stage of theoretical development to construct a digital booklet with an observational basis based on studies that reiterate themes about educational technologies as tools to conduct a home protocol of guided exercises without the direct supervision of professional physical therapists. Results indicate a lack of research on the development of health education technologies to assist patients with HTLV-1 without tropical spastic paraparesis or HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). We believe that this narrative review can initiate a theoretical framework to conduct a home exercise program aimed at people with HTLV-1 who have subtle symptoms, and also at people without the clinical definition of HAM/TSP, helping to train human resources for care and research on the subject and increase scientific production in physical therapy.
PubMed: 37736995
DOI: 10.3390/idr15050048 -
The Journal of Thoracic and... Oct 2023Many patients undergoing thoracoabdominal aortic aneurysm (TAAA) repair have had a previous myocardial infarction (MI). To address the paucity of data regarding outcomes...
OBJECTIVE
Many patients undergoing thoracoabdominal aortic aneurysm (TAAA) repair have had a previous myocardial infarction (MI). To address the paucity of data regarding outcomes in such patients, we aimed to compare outcomes after open TAAA repair in patients with and without previous MI.
METHODS
From 1986 to 2022, we performed 3737 consecutive open TAAA repairs. Of these, 706 (18.9%) were in patients with previous MI. We used multivariable logistic regression to identify predictors of operative death. Propensity score matching analyzed preoperative and select operative variables to create matched groups of patients with or without a previous MI (n = 704 pairs). Late survival was determined by Kaplan-Meier analysis and compared by log rank test.
RESULTS
Overall, operative mortality was 8.5% and the adverse event rate was 15.2%; these were elevated in patients with MI (11.0% vs 7.9% [P = .01] and 18.0% vs 14.6% [P = .02], respectively). In the propensity score-matching cohort, the MI group had a greater rate of cardiac complications (32.4% vs 25.4%; P = .005) and delayed paraparesis (5.1% vs 2.4%; P = .1); however, there was no difference in operative mortality (11.1% vs 10.9%; P = 1) or adverse event rate (18.0% vs 16.8%; P = .6). Overall, previous MI was not independently associated with operative mortality in multivariable analysis (P = .1). The matched MI group trended toward poorer 10-year survival (29.8% ± 1.9% non-MI vs 25.0% ± 1.8% MI; P = .051).
CONCLUSIONS
Although previous MI was not associated with early mortality after TAAA repair, patients with a previous MI had greater rates of cardiac complications and delayed paraparesis. Patients with a previous MI also trended toward poorer survival.
PubMed: 37802329
DOI: 10.1016/j.jtcvs.2023.09.071 -
Asian Journal of Neurosurgery Sep 2023A spinal arachnoid cyst is a rare entity representing only 1 to 3% of spinal canal lesions. Very few of them are reported to be symptomatic. Moreover, occurrence in...
A spinal arachnoid cyst is a rare entity representing only 1 to 3% of spinal canal lesions. Very few of them are reported to be symptomatic. Moreover, occurrence in multiples is even rarer. Extradural type is more common than intradural. In the spine, it is rare in the sacral region. Other common pathologies, such as a dermoid or epidermoid cyst, are often considered at presentation. Even magnetic resonance imaging can miss the diagnosis sometimes. We report a case with symptoms of paraparesis and incontinence at presentation, initially misdiagnosed as a dermoid cyst, later found to be multiple intradural arachnoid cysts located in the sacral region. Spinal arachnoid cysts may cause debilitating symptoms. The disease is completely curable if the detection and classification are early as in our case.
PubMed: 38152537
DOI: 10.1055/s-0043-1774376 -
Free Neuropathology Jan 2023The history of adrenoleukodystrophy (ALD), adrenomyeloneuropathy (AMN) and other peroxisomal diseases is exemplary for the stunning progress of scientific medicine... (Review)
Review
The history of adrenoleukodystrophy (ALD), adrenomyeloneuropathy (AMN) and other peroxisomal diseases is exemplary for the stunning progress of scientific medicine within the past 50 years. Like many breakthroughs in medicine, the detailed analysis of patients' pathologically affected tissues was instrumental, resulting in stepwise systematic clarification of what had remained enigmatic until the 1970s. This flashback paper is a recollection of the first neuropathological description of a slowly evolving clinical phenotype, spastic paraparesis with adrenal insufficiency, in a young adult by Budka et al. 1976 [3], using virtual microscopy of the original histologic slides. The clinico-pathological presentation derives from the classical cerebral ALD phenotype in boys, where electron microscopy demonstrated the underlying pathological hallmark of characteristic lipid inclusions shared by both phenotypes. Our report allowed the delineation of a new disease type almost simultaneously described in more cases as AMN by Griffin et al. 1977 [4] and Schaumburg et al. 1977 [11]. Moreover, our report indicated clinical heterogeneity in the ALD disease group that, as shown later, extends further to females, to Addison-only, and even to asymptomatic subjects. The gene underlying ALD was discovered in 1993 as a defect in the gene. Yet, it has hitherto remained unclear how the gene defect causes the strikingly broad and unpredictable phenotypic spectrum of ALD/AMN.
PubMed: 37915358
DOI: 10.17879/freeneuropathology-2023-5115