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The Journal of the Association of... Oct 2023Statins are drugs for preventing cardiac events in the elderly population. Statins are well tolerated with a lower reported incidence of serious side effects (<0.15%)...
Statins are drugs for preventing cardiac events in the elderly population. Statins are well tolerated with a lower reported incidence of serious side effects (<0.15%) like myopathy and elevated transaminases [>3× upper limit of normal (ULN)]. Serious adverse effects of statins like statin-associated myopathy range from mild muscle pain to rhabdomyolysis. Drug-induced liver injury (DILI) is another adverse effect of statin use, typically presenting with an acute hepatocellular liver injury pattern as mixed or cholestatic injury. Symptoms usually disappear after 3 months of discontinuation of statins. Some patients require immunosuppression with steroids, intravenous immunoglobulin, or rituximab for management of rhabdomyolysis. DILI can be rapidly reversed by the stoppage of the statins if the enzyme elevation is more than twice the normal. Elderly patients are particularly at increased risk of such adverse effects, emphasizing a need for rational prescription of statins in older adults and close monitoring. We report a case of an elderly presenting with paraparesis and later diagnosed to be a case of statin-induced myositis that significantly improved with prompt management. : Kashyap K, Bisht K, Dhar M, Atorvastatin-induced Myositis and Drug-induced Liver Injury. J Assoc Physicians India 2023;71(10):96-98.
Topics: Humans; Atorvastatin; Chemical and Drug Induced Liver Injury; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Myositis; Aged; Male; Female
PubMed: 38716533
DOI: 10.59556/japi.71.0309 -
Neurosurgical Review May 2024Surgery and endovascular therapy are the primary treatment options for spinal dural arteriovenous fistula (SDAVF). Due to the absence of a consensus regarding which... (Comparative Study)
Comparative Study
Surgery and endovascular therapy are the primary treatment options for spinal dural arteriovenous fistula (SDAVF). Due to the absence of a consensus regarding which therapy yields a superior outcome, we conducted a comparative analysis of the surgical and endovascular treatment of SDAVF through a multicenter case series and a systematic literature review. Patients with SDAVF, surgically or endovascularly treated at four neurosurgical centers from January 2001 to December 2021, were included in this study. Level of SDAVF, primary treatment modality, baseline and post-procedural neurological status were collected. The primary outcomes were failure, complication rates, and a newly introduced parameter named as therapeutic delay. A systematic review of the literature was performed according to PRISMA-P guidelines. The systematic review identified 511 papers, of which 18 were eligible for analysis, for a total of 814 patients, predominantly male (72%) with a median age of 61 and mainly thoracic SDAVFs (65%). The failure rate was significantly higher for endovascular therapy (20%) compared to surgery (4%) (p < 0.01). Neurological complications were generally rare, with similar rates among the two groups (endovascular 2.9%; surgery 2.6%). Endovascular treatment showed a statistically significantly higher rate of persistent neurological complications than surgical treatment (2.9% versus 0.2%; p < 0.01). Both treatments showed similar rates of clinical improvement based on Aminoff Logue scale score. The multicenter, retrospective study involved 131 patients. The thoracic region was the most frequent location (58%), followed by lumbar (37%). Paraparesis (45%) and back pain (41%) were the most common presenting symptoms, followed by bladder dysfunction (34%) and sensory disturbances (21%). The mean clinical follow-up was 21 months, with all patients followed for at least 12 months. No statistically significant differences were found in demographic and clinical data, lesion characteristics, or outcomes between the two treatment groups. Median pre-treatment Aminoff-Logue score was 2.6, decreasing to 1.4 post-treatment with both treatments. The mean therapeutic delay for surgery and endovascular treatment showed no statistically significant difference. Surgical treatment demonstrated significantly lower failure rates (5% vs. 46%, p < 0.01). In the surgical group, 2 transient neurological (1 epidural hematoma, 1 CSF leak) and 3 non-neurological (3 wound infections) complications were recorded; while 2 permanent neurological (spinal infarcts), and 5 non-neurological (inguinal hematomas) were reported in the endovascular group. According to the literature review and this multicenter clinical series, surgical treatment has a significantly lower failure rate than endovascular treatment. Although the two treatments have similar complication rates, endovascular treatment seems to have a higher rate of persistent neurological complications.
Topics: Humans; Central Nervous System Vascular Malformations; Endovascular Procedures; Neurosurgical Procedures; Male; Female; Middle Aged; Treatment Outcome; Aged; Postoperative Complications; Embolization, Therapeutic
PubMed: 38713376
DOI: 10.1007/s10143-024-02443-8 -
Cureus Apr 2024Glioblastoma, a primary brain tumor known for its adverse prognosis and aggressive nature, presents a significant challenge when occurring in the spinal cord. We report...
Glioblastoma, a primary brain tumor known for its adverse prognosis and aggressive nature, presents a significant challenge when occurring in the spinal cord. We report a case of a 20-year-old female with no prior medical history who developed progressive paraparesis and urinary retention, symptoms indicative of an intramedullary glioblastoma in the spinal cord. This case study delves into the clinical presentation, diagnostic process, and therapeutic interventions, highlighting the complexities encountered during the patient's treatment course. Despite the typically poor prognosis associated with glioblastoma, with an average survival rate of approximately 15 months post-diagnosis, our patient's initial response to adjuvant chemotherapy and radiotherapy extended her survival to 34 months. However, tumor recurrence ultimately led to a decision against aggressive treatment, reflecting the challenges in managing this devastating condition. This case emphasizes the importance of a multidisciplinary approach in the care of spinal glioblastoma patients, ranging from neurosurgery, anesthesiology and intensive care, radiology, oncology, anatomic pathology and nuclear medicine, underscoring the complexity of the disease, while highlighting the urgent need for ongoing research and innovation in neuro-oncology to improve treatment outcomes. The use of modern treatment techniques, including the potential role of nanomaterials for drug delivery, suggests avenues for future research. This case report contributes to the scarce literature on spinal glioblastoma, advocating for detailed documentation of cases to enhance understanding and treatment strategies for this formidable disease.
PubMed: 38707088
DOI: 10.7759/cureus.57593 -
Multiple Sclerosis and Related Disorders Jul 2024The roles of endocannabinoids are described in immune modulation and neuroprotection. HTLV-1-associated myelopathy (HAM/TSP) is an inflammatory neurodegenerative...
BACKGROUND/AIM
The roles of endocannabinoids are described in immune modulation and neuroprotection. HTLV-1-associated myelopathy (HAM/TSP) is an inflammatory neurodegenerative disease. Therefore, in this study, the interactions of HTLV-1 regulatory factors and host cannabinoid receptors (CBRs) were evaluated in HAM/TSP.
METHODS
Nineteen HAM/TSPs, 22 asymptomatic carriers (ACs), and 18 healthy controls (HCs) were enrolled. RNA was extracted from PBMCs and then reverse-transcribed to cDNA. The gene expression of CB1R and CB2R, as well as HTLV-1 proviral load (PVL), Tax and HTLV-1 basic leucine zipper factor (HBZ) were assessed by RT-qPCR.
RESULTS
The mean expression of CB1R in ACs (8.51 ± 2.76) was significantly higher than HAMTSPs (1.593 ± 0.74, p = 0.05) and also HCs (0.10 ± 0.039, p = 0.001). The CB2R gene expression level in ACs (2.62±0.44) was significantly higher than HAM/TSPs (0.59 ± 0.15, p = 0.001) and HCs (1.00 ± 0.2, p = 0.006). Meanwhile there was a strong correlation between CB1R and CB2R gene expression levels in the HCs and HAM/TSPs (p = 0.001). HTLV-1-Tax expression in HAM/TSPs (386 ± 104) was higher than ACs (75 ± 32) and statistically significant (p = 0.003). While HTLV-1-HBZ was only expressed in three AC subjects and five HAM/TSPs, thus it cannot be analyzed.
CONCLUSION
The up-regulation of CB2R has immunomodulatory effects in inflammatory reactions. While CB1R as a neuroprotective agent may suppress inflammatory reactions in ACs, preventing HAM/TSP. It seems that, like multiple sclerosis (MS), cannabinoid medications are beneficial in HAM/TSP.
Topics: Humans; Male; Female; Receptor, Cannabinoid, CB1; Adult; Receptor, Cannabinoid, CB2; Paraparesis, Tropical Spastic; Middle Aged; Human T-lymphotropic virus 1; Gene Products, tax; Basic-Leucine Zipper Transcription Factors; Viral Load; Retroviridae Proteins
PubMed: 38704874
DOI: 10.1016/j.msard.2024.105659 -
Clinical Neurology and Neurosurgery Jun 2024Pyruvate dehydrogenase complex (PDC) deficiency is a genetic mitochondrial disease mostly associated with severe lactic acidosis, rapid progression of neurological...
Pyruvate dehydrogenase complex (PDC) deficiency is a genetic mitochondrial disease mostly associated with severe lactic acidosis, rapid progression of neurological symptoms and death during childhood. We present a 33-year-old male with PDC deficiency caused by a Val262Leu mutation in PDHA1gene. He demonstrated generalized dystonia affecting trunk and upper extremities and paraparesis as the most significant features, with onset of symptoms at age 8. Brain MRI showed bilaterally increased signal within the globus pallidus, typical of Leigh syndrome. A periodic lactate increase in serum and cerebrospinal fluid was detected. We describe a case of pyruvate dehydrogenase deficiency being diagnosed only 25 years after the onset of symptoms and highlight PDHC deficiency as a possible cause of treatable dystonia in childhood, which may respond well to thiamine and levodopa treatment.
Topics: Humans; Male; Adult; Pyruvate Dehydrogenase Complex Deficiency Disease; Pyruvate Dehydrogenase (Lipoamide); Dystonia; Levodopa; Dystonic Disorders; Magnetic Resonance Imaging; Mutation
PubMed: 38701546
DOI: 10.1016/j.clineuro.2024.108307 -
International Journal of Infectious... Aug 2024Infective dermatitis associated with human T-cell lymphotropic virus type-1 (HTLV-1) (IDH) is a severe form of chronically infected eczema occurring in early childhood,... (Review)
Review
Infective dermatitis associated with human T-cell lymphotropic virus type-1 (HTLV-1) (IDH) is a severe form of chronically infected eczema occurring in early childhood, although very rarely cases have been reported in adults. Most of the cases are from Jamaica and Brazil and occur in individuals with low socioeconomic status. IDH is always associated with refractory Staphylococcus aureus or beta-hemolytic Streptococcus infection of the skin and nasal vestibules. Patients with IDH may develop other even more severe HTLV-1-associated diseases, such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) of early or late appearance and adult T-cell leukemia/lymphoma. In the context of the Brazilian experience, it has been observed that 54% of IDH patients exhibit the juvenile form of HAM/TSP while the estimated incidence of adult HAM/TSP is 3%. As there are no curative treatments for HTLV-1 infection (or vaccines) or most of its associated diseases, prevention of infection is fundamental, mainly by vertical transmission, as it is responsible for the development of IDH, infantojuvenile HAM/TSP, and ATL. Public measures to reduce this transmission must be implemented urgently. Furthermore, it is recommended, mainly in HTLV-1 endemic areas, to search for HTLV-1 infection in all patients with infected eczema, even in adults.
Topics: Humans; HTLV-I Infections; Human T-lymphotropic virus 1; Brazil; Paraparesis, Tropical Spastic; Adult; Dermatitis
PubMed: 38697604
DOI: 10.1016/j.ijid.2024.107058 -
Interdisciplinary Cardiovascular and... Jun 2024Our goal was to evaluate postoperative patterns of collateral arteries to the spinal cord during occlusion of the segmental arteries supplying the artery of Adamkiewicz...
OBJECTIVES
Our goal was to evaluate postoperative patterns of collateral arteries to the spinal cord during occlusion of the segmental arteries supplying the artery of Adamkiewicz (AKA).
METHODS
Between April 2011 and December 2022, a total of 179 patients underwent thoraco-abdominal aortic aneurysm repair; 141 had an identifiable AKA on preoperative multidetector computed tomography scans, 40 underwent thoraco-abdominal aortic aneurysm replacement (TAAR) and 101 underwent thoracic endovascular aortic repair (TEVAR). New postoperative collateral blood pathways invisible on preoperative contrast-enhanced computed tomography scans were identified in 42 patients (10 patients who had TAAR vs 32 patients who had TEVAR) who underwent preoperative and postoperative multidetector computed tomography scanning for AKA identification.
RESULTS
The thoracodorsal and segmental arteries were the main collateral pathways in both groups. Th9-initiated collaterals were the most common. Collaterals from the internal thoracic artery were observed in the TEVAR group but not in the TAAR group. One patient in the TEVAR group experienced postoperative paraparesis, which was not observed in the TAAR group. Postoperative paraplegia was more common in the non-Th9-origin group, but this difference was not significant.
CONCLUSIONS
Thoracodorsal and segmental arteries may be important collateral pathways after TEVAR and TAAR. For thoracodorsal arteries, preserving the thoracodorsal muscle during the approach would be crucial; for segmental arteries, minimizing the area to be replaced or covered would be paramount. An AKA not initiated at the Th9 level poses a high risk of postoperative paraplegia.
PubMed: 38696750
DOI: 10.1093/icvts/ivae087 -
Movement Disorders Clinical Practice Jun 2024As the diagnosis of Parkinson's disease (PD) is fundamentally clinical, the usefulness of ioflupane (I) single-photon emission computed tomography (SPECT) or DaTSCAN as... (Review)
Review
BACKGROUND
As the diagnosis of Parkinson's disease (PD) is fundamentally clinical, the usefulness of ioflupane (I) single-photon emission computed tomography (SPECT) or DaTSCAN as a diagnostic tool has been a matter of debate for years. The performance of DaTSCAN is generally recommended in the follow-up of patients with a clinically uncertain diagnosis, especially in those with a suspected essential tremor, drug-induced parkinsonism, or vascular parkinsonism. However, there is a dearth of DaTSCAN findings regarding neurodegenerative parkinsonisms besides PD and atypical parkinsonisms. To date, a specific nigrostriatal dopamine uptake pattern that would help differentiate PD from the most frequent atypical parkinsonisms is yet to be described. This fact is further complicated by the possible visualization of abnormalities in the uptake pattern in patients with rarer neurodegenerative parkinsonisms.
OBJECTIVES
We aimed to summarize the current literature regarding DaTSCAN findings in patients with rare neurodegenerative parkinsonisms.
METHODS
The PubMed database was systematically screened for studies in English or Spanish up to October 15, 2023, using search terms "DaTSCAN", "ioflupane", "DaT-SPECT", "123I-FP-CIT SPECT", "dopamine transporter imaging", and "[123I] FP-CIT SPECT". Duplicated publications and studies regarding PD, atypical parkinsonisms, dystonia-parkinsonism, essential tremor, and parkinsonism due to non-degenerative causes were excluded.
RESULTS
The obtained results were reviewed and summarized, including DaTSCAN findings in fragile X-associated tremor/ataxia syndrome, prion diseases, Huntington's disease, spinocerebellar ataxia, hereditary spastic paraparesis, metabolic disorders, and other diseases (anti-IgLON5 disease, ring chromosome 20 syndrome, chorea-acanthocytosis, and neuronal ceroid lipofuscinosis).
CONCLUSIONS
This review highlights the need to determine in the future the utility and cost-effectiveness of DaTSCAN, both as a diagnostic and a prognostic tool, in patients with parkinsonian symptoms in rare neurodegenerative diseases.
Topics: Humans; Tomography, Emission-Computed, Single-Photon; Parkinsonian Disorders; Tropanes; Parkinson Disease
PubMed: 38693679
DOI: 10.1002/mdc3.14055 -
Cureus Mar 2024Raynaud's syndrome is characterized by paroxysmal vasospasm in the digital arterioles, following exposure to cold or stress. Pain, swelling, stiffness, and hypoesthesia...
Raynaud's syndrome is characterized by paroxysmal vasospasm in the digital arterioles, following exposure to cold or stress. Pain, swelling, stiffness, and hypoesthesia are observed as manifestations. The presence of a trophic ulcer is accompanied by a range of severe manifestations. The assaults occur in three distinct phases, namely vasospastic, plethoric, and erythema. Various approaches improve the overall well-being of a patient. It is possible to differentiate between primary and secondary Raynaud's syndrome, the latter being linked to systemic diseases. The application of botulin toxin is commonly indicated in several medical conditions including focal dystonia, spasticity with or without contractures, paraparesis in children with cerebral palsy, multiple sclerosis, brain injuries, involuntary muscle hyperactivity of a non-dystonic nature, pain management, strabismus, nystagmus, sialorrhea, and esthetic medicine. When treating Raynaud's a technique is used with injection at the base of each finger, from the palmar side, which helps with cooling and minimizing discomfort for patients. We present a clinical case of a 70-year-old female patient with Raynaud's syndrome in which we have placed 70E distributed to both hands botulin toxin type A. Improvement in the patient's symptomatology was noticed on day 3, with warming of the hands, lack of swelling, and pain with duration of the effect little over three months. The patient underwent a six-month follow-up following the therapy with botulinum toxin type A, and no indications of recurrence or advancement of Raynaud's syndrome (RS) were seen.
PubMed: 38690447
DOI: 10.7759/cureus.57327 -
Neurology. Genetics Jun 2024To report novel biallelic variants in a family presenting with pure hereditary spastic paraparesis.
OBJECTIVES
To report novel biallelic variants in a family presenting with pure hereditary spastic paraparesis.
METHODS
Two affected sisters presented with unsolved hereditary spastic paraparesis and underwent clinical and imaging assessments. This was followed by short-read next-generation sequencing.
RESULTS
Analysis of next-generation sequencing data uncovered compound heterozygous variants in (NM_058004.4: c.[3883C>A];[5785A>C]; p.[(His1295Asn);(Thr1929Pro)]. Using ACMG guidelines, both variants were classified as likely pathogenic.
DISCUSSION
Here, next-generation sequencing revealed 2 novel compound heterozygous variants in the phosphatidylinositol 4-kinase alpha gene () in 2 sisters presenting with progressive pure hereditary spastic paraparesis. Pathogenic variants in have previously been associated with a spectrum of disorders including autosomal recessive perisylvian polymicrogyria, with cerebellar hypoplasia, arthrogryposis, and pure spastic paraplegia. The cases presented in this study expand the phenotypic spectrum associated with variants and contribute new likely pathogenic variants for testing in patients with otherwise unsolved hereditary spastic paraparesis.
PubMed: 38685974
DOI: 10.1212/NXG.0000000000200152