Autoimmune Hemolytic Anemia Due to Spondyloenchondrodysplasia with Spastic Paraparesis and Intracranial Calcification due to Mutation in ACP5.

Spondyloenchondrodysplasia (SPENCD) is a rare spondylometaphyseal skeletal dysplasia with characteristic lesions mimicking enchondromatosis and resulting in short stature. A large spectrum of immunologic abnormalities may be seen in SPENCD, including immune deficiencies and autoimmune disorders. SPENCD is caused by loss of tartrate-resistant acid phosphatase activity, due to homozygous mutations in ACP5 , playing a role in nonnucleic-acid-related stimulation/regulation of the type I interferon pathway. In this article, we presented a 19-year-old boy with SPENCD, presenting with recurrent autoimmune hemolytic anemia episodes since he was 5 years old. He had short stature, platyspondyly, metaphyseal changes, intracranial calcification, spastic paraparesis, and mild intellectual disability. He also had recurrent pneumonia attacks. The clinical diagnosis of SPENCD was confirmed by sequencing of the ACP5 gene, and a homozygous c.155A > C (p.K52T) variation was found, which was reported before as pathogenic. In conclusion, in early onset chronic autoimmune cytopenias an immune dysregulation may often have a role in the etiology. Associating findings and immunologic functions should be carefully evaluated in such patients in the light of the literature. The present case shows the importance of multisystemic evaluation for the detection of SPENCD that has a monogenic etiology.

Liver Transplantation Reverses Hepatic Myelopathy in the Decompensated Phase of Cirrhosis: Case Report and Literature Review.

Hepatic myelopathy (HM) is a rare neurological complication in the end stage of many liver diseases and is characterized by bilateral spastic paraparesis without sensory and sphincter dysfunction. It occurs owing to metabolic disorders and central nervous system dysfunction associated with cirrhosis. Without timely and effective clinical intervention, the prognosis of these patients is devastating. Although liver transplantation (LT) is an effective treatment for HM, the prognosis of these patients remains unsatisfactory. Early recognition and diagnosis of this disease are essential for improving patient prognosis. Here, we report a case of hepatitis B virus-associated decompensated cirrhosis with HM. The patient recovered well after LT. We also summarize the clinical characteristics and post-transplant outcomes of 25 patients with HM treated by LT through 2023, including this case.

[Medullary infarction secondary to endovascular treatment of thoracoabdominal aortic aneurysm] / Infarto medular secundario a tratamiento endovascular de aneurisma de aorta toraco-abdominal.

Medullary infarction is a severe and infrequent pathology, which represents 1% of all ischemic strokes, and is also a rare complication of different surgical procedures. It is caused by the acute interruption of the blood flow of the spinal cord, manifesting itself with clinical neurological deficits related to the affected vascular territory. Methods: We present the case of an 80-year-old patient, with cardiovascular risk factors, who is present on post-surgical day 13, after placement of a vascular endoprosthesis for a thoracoabdominal aneurysm, sudden appearance of paraparesis with progression to paraplegia and hypoesthesia in both lower extremities. CT angiography of the aorta rules out local complications in the endoprosthesis. Medullary MRI showed images compatible with acute dorsal medullary infarction from level D9. Results: On discharge, the patient presented paraplegia and hypoesthesia of both lower extremities with fecal and urinary incontinence. Conclusion: Spinal cord infarction may be limited to a vascular territory or be more extensive according to its pathogenesis. The affectation of the anterior spinal artery is the most common and is characterized by bilateral motor deficits and loss of thermoalgesic sensitivity, which could have a great impact on the quality of life of patients. Its etiology is varied, including aortic surgery within its causes. MRI is very useful for its diagnosis and currently there are no clinical guides for the optimal treatment.

Introducción: El infarto medular es una patología severa e infrecuente, que representa el 1% del total de ictus isquémicos, siendo además una complicación rara de distintos procedimientos quirúrgicos. Es causado por la interrupción aguda del flujo sanguíneo de la médula espinal, manifestándose con déficits neurológicos clínicos relacionados con el territorio vascular afectado. Métodos: Presentamos el caso de un paciente de 80 años, con factores de riesgo cardiovascular, quien presenta en día postquirúrgico 13, tras colocación de endoprótesis vascular por aneurisma toraco-abdominal aparición brusca de paraparesia con progresión a paraplejía e hipoestesia en ambas extremidades inferiores.  Angio-TC de aorta descarta complicación local en la endoprótesis. RM medular mostró imágenes compatibles con Infarto agudo de médula dorsal desde el nivel D9. El paciente no fue subsidiario de tratamiento revascularizador. El tratamiento consistió en medidas de soporte. Resultados: Al alta el paciente presentaba paraplejia e hipoestesia de ambas extremidades inferiores con incontinencia fecal y urinaria. Conclusión: El infarto de la médula espinal puede estar limitado a un territorio vascular o estar más extendido según su patogenia. La afectación de la arteria espinal anterior es la más común y se caracteriza por déficits motores bilaterales y pérdida de la sensibilidad termoalgésica, pudiendo llegar a producir un gran impacto en la calidad de vida de los pacientes. Su etiología es variada, incluyéndose la cirugía aórtica dentro de sus causas. La RM es muy útil para su diagnóstico y actualmente no existen guías clínicas para el tratamiento óptimo.

HTLV in Sweden.

Sweden is a country with a low prevalence of human lymphotropic T-cell virus (HTLV) infection, estimated at < 0.005%, but the infection rate is notably higher in specific risk groups such as HTLV-2 among intravenous drug users (IVDU) and people originating from HTLV-1 highly endemic areas. Thus, in the most recent study from 2012, the prevalence of HTLV-2 among IVDU in Stockholm was 3.2%. However, much of the epidemiological data on HTLV in Sweden stems from studies conducted primarily between the 1990s and 2007, and the impact of migration to Sweden during the past 15 years has not been evaluated. Despite Sweden's status as a country with generally low prevalence of HTLV, it is prudent to anticipate and prepare for several potential challenges associated with HTLV infection in the future. Proactive measures to enhance awareness, alongside strategies to curtail transmission and mitigate complications, are crucial for addressing this relatively rare, but significant health issue. In this work, we review the current epidemiological knowledge about HTLV in Sweden and discuss future Swedish perspectives.

Hereditary spastic paraparesis type 18 (SPG18): new ERLIN2 variants in a series of Italian patients, shedding light upon genetic and phenotypic variability.

INTRODUCTION: Hereditary spastic paraparesis (HSP) is a group of central nervous system diseases primarily affecting the spinal upper motor neurons, with different inheritance patterns and phenotypes. SPG18 is a rare, early-onset, complicated HSP, first reported as linked to biallelic ERLIN2 mutations. Recent cases of late-onset, pure HSP with monoallelic ERLIN2 variants prompt inquiries into the zygosity of such genetic conditions. The observed relationship between phenotype and mode of inheritance suggests a potential dominant negative effect of mutated ERLIN2 protein, potentially resulting in a milder phenotype. This speculation suggests that a wider range of HSP genes could be linked to various inheritance patterns. PURPOSE AND BACKGROUND: With documented cases of HSP loci exhibiting both dominant and recessive patterns, this study emphasizes that the concept of zygosity is no longer a limiting factor in the establishment of molecular diagnoses for HSP. Recent cases have demonstrated phenoconversion in SPG18, from HSP to an amyotrophic lateral sclerosis (ALS)-like syndrome. METHODS AND RESULTS: This report highlights two cases out of five exhibiting HSP-ALS phenoconversion, discussing an observed prevalence in autosomal dominant SPG18. Additionally, the study emphasizes the relatively high incidence of the c.502G>A variant in monoallelic SPG18 cases. This mutation appears to be particularly common in cases of HSPALS phenoconversion, indicating its potential role as a hotspot for a distinctive SPG18 phenotype with an ALS-like syndrome. CONCLUSIONS: Clinicians need to be aware that patients with HSP may show ALS signs and symptoms. On the other hand, HSP panels must be included in genetic testing methods for instances of familial ALS.

Multicenter, randomized, double-blind, placebo-controlled phase 3 study of mogamulizumab with open-label extension study in a minimum number of patients with human T-cell leukemia virus type-1-associated myelopathy.

Human T-cell leukemia virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic neurodegenerative disease. This multicenter, randomized phase 3 study evaluated the efficacy and safety of 0.3 mg/kg intravenous mogamulizumab, a monoclonal antibody targeting-CC chemokine receptor 4, every 12 weeks in HAM/TSP patients. This study comprised a 24-week double-blind, placebo-controlled period, 24-week open-label period, and extension treatment period. The primary endpoint was the proportion of patients with a ≥ 1-grade improvement in the Osame motor disability score (OMDS). Secondary endpoints were changes in HTLV-1 proviral load, 10-m timed walk, cerebrospinal fluid (CSF) neopterin levels, and safety. The exploratory endpoint was CSF chemokine C-X-C motif ligand 10 (CXCL10) levels. Thirty-four and 33 patients were randomized to mogamulizumab and placebo arms, respectively. At the end of the double-blind period, no significant difference was found in the OMDS improvement rate or other secondary efficacy endpoints assessing motor activities. However, the mogamulizumab arm showed a significant decrease in HTLV-1 proviral load (- 59.39 ± 29.91% vs. placebo 2.32 ± 36.31%) and CSF neopterin (p < 0.001)/CXCL10 levels (p = 0.004). The baseline OMDS pattern and the 60-80% HTLV-1 proviral load reduction were sustained through the open-label and extension treatment periods. Although a higher incidence of rash (69.2%) was reported, the safety profile was similar compared with a previous phase 1/2a study. We found no significant difference in clinical benefit; however, mogamulizumab may provide long-term clinical benefit by preventing disease progression, as CSF neopterin/CXCL10 levels are associated with long-term prognosis in HAM/TSP.Clinical Trial Registration Number: NCT03191526 (registered date: 6-June-2017).

Hereditary spastic paraparesis type 46 (SPG46): new GBA2 variants in a large Italian case series and review of the literature.

Hereditary spastic paraparesis (HSP) is a group of central nervous system diseases primarily affecting the spinal upper motor neurons, with different inheritance patterns and phenotypes. SPG46 is a rare, early-onset and autosomal recessive HSP, linked to biallelic GBA2 mutations. About thirty families have been described worldwide, with different phenotypes like complicated HSP, recessive cerebellar ataxia or Marinesco-Sjögren Syndrome. Herein, we report five SPG46 patients harbouring five novel GBA2 mutations, the largest series described in Italy so far. Probands were enrolled in five different centres and underwent neurological examination, clinical cognitive assessment, column imaging for scoliosis assessment, ophthalmologic examination, brain imaging, GBA2 activity in peripheral blood cells and genetic testing. Their phenotype was consistent with HSP, with notable features like upper gaze palsy and movement disorders. We review demographic, genetic, biochemical and clinical information from all documented cases in the existing literature, focusing on the global distribution of cases, the features of the syndrome, its variable presentation, new potential identifying features and the significance of measuring GBA2 enzyme activity.

Mycophenolate mofetil for the long-term treatment of HTLV-1 associated myelopathy: A case report.

Human T-cell leukemia virus type 1 (HTLV-1) can cause HTLV-1 Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP). Current treatment options for HAM/TSP are limited. We present a woman with rapidly-progressive HAM/TSP with significant, sustained clinical improvement following initiation of mycophenolate mofetil (MMA). Peripheral blood mononuclear cells from the patient, her asymptomatic carrier husband and eight healthy controls were isolated. Frequencies of T-cell populations upon exposure to low and high MMA concentrations and differences in proliferation were analyzed using flow cytometry and a CSFE-proliferation assay. Characterization of T-cell function and proliferation showed higher levels of GranzymeB in HTLV-1+ donors. The improvement and stability of symptoms in this patient with HAM/TSP following MMA initiation requires further study as a potential treatment for HAM/TSP.

Paraparesis due to angio-neurotropic Gurltia paralysans in a domestic cat (Felis catus) and retrospective study on feline gurltiosis cases in South America.

Introduction: The nematode Gurltia paralysans is a neglected angio-neurotropic parasite causing chronic meningomyelitis in domestic cats (Felis catus) as well as wild felids of the genus Leopardus in South America. Adult G. paralysans nematodes parasitize the leptomeningeal veins of the subarachnoid space and/or meningeal veins of the spinal cord parenchyma. The geographic range of G. paralysans encompasses rural and peri-urban regions of Chile, Argentina, Uruguay, Colombia and Brazil. Methods: This case report presents clinical and pathological findings of a G. paralysans-infected cat suffering from severe thrombophlebitis and meningomyelitis resulting in ambulatory paraparesis. Neurological examination of affected cat localized the lesions at the thoracolumbar (T3-L3) and lumbosacral (L4-Cd4) segments. Molecular and morphological characteristics of extracted nematodes from parasitized spinal cord veins confirmed G. paralysans. Additionally, data obtained from a questionnaire answered by cat owners of 12 past feline gurltiosis cases (2014-2015) were here analyzed. Questionnaire collected data on age, gender, geographic location, type of food, hunting behavior, type of prey, and other epidemiological features of G. paralysans-infected cats. Results and Discussion: Data revealed that the majority of cats originated from rural settlements thereby showing outdoor life styles with hunting/predatory behaviors, being in close contact to wild life [i.e. gastropods, amphibians, reptiles, rodents, birds, and wild felids (Leopardus guinia)] and with minimal veterinary assistance. Overall, this neglected angio-neurotropic G. paralysans nematode still represents an important etiology of severe thrombophlebitis and meningomyelitis of domestic cats living in endemic rural areas with high biodiversity of definitive hosts (DH), intermediary (IH), and paratenic hosts (PH). The intention of this study is to generate awareness among veterinary surgeons as well as biologists on this neglected feline neuroparasitosis not only affecting domestic cats but also endangered wild felid species of the genus Leopardus within the South American continent.

Arterial thromboembolism in a cat with transient myocardial thickening.

Feline arterial thromboembolism has been reported to be secondary to various feline cardiomyopathies; however, it has not been described in cats with transient myocardial thickening. A previously healthy, one-year-old, castrated male cat presented with acute paraparesis and congestive heart failure. Echocardiography revealed asymmetric left ventricular free wall thickening and left atrial enlargement. Antithrombotic treatment and cardiac medication resulted in reperfusion and mobility on day seven in one limb and on day 10 in the other. Different complications were managed successfully, including worsening acute kidney injury, inflammation, pleural effusion, and anemia. After three weeks, the cat was discharged and prescribed oral antithrombotic drugs (clopidogrel and rivaroxaban) and cardiac medication. Within five months, echocardiographic findings normalized, and medical treatment was gradually discontinued. To date, the cat remains healthy at 1735 days after the initial diagnosis and 1494 days after the last antithrombotic medication. To the best of our knowledge, this is the first case report on feline arterial thromboembolism combined with transient myocardial thickening, with favorable long-term survival.

Physiotherapeutic Approach for Paraparesis Resulting From L2-S1 Disc Bulge Along With Diabetic Neuropathy: A Case Report.

A degenerative disc disease is characterized by osteophytes, disc space reduction, nerve compression and discomfort are all symptoms of disc bulging. Diabetic neuropathy, a condition leading to significant health issues, involves a sensory dysfunction initiating in the lower extremities and progressing to pain. In the present case, a 61-year-old retired physical training teacher came to the hospital with complaints of difficulty in walking, sitting, and standing in the past two years. The patient also complained of a tingling sensation in the upper and lower limbs, low back pain, and body stiffness. The patient has a known case of intervertebral disc prolapse at L2-S1 level, two years back. Patient had a history of diabetes mellitus type 2, hypertension, and hypothyroidism for the past three years. The study delves into the detailed evaluation, customized treatment plan, and rehabilitation strategies employed to meet the patient's condition. It emphasizes the value of a multidisciplinary approach, including physical therapy, in the treatment of complicated musculoskeletal and neurological illnesses, intending to improve the patient's mobility, functionality, and overall quality of life.

Spontaneous Spinal Hematoma in Patients Using Antiplatelets and Anticoagulants: A Systematic Review.

BACKGROUND: Spontaneous spinal hematoma (SSH) is a debilitating complication in patients taking either antiplatelet (AP) or anticoagulation (AC) medications. SSH is rare and, therefore, a systematic review is warranted to re-examine and outline trends, clinical characteristics, and outcomes associated with SSH formation. METHODS: PubMed, EMBASE, Scopus, and Web-of-Science were searched. Studies reporting clinical data of patients with SSH using AC medications were included. In addition, clinical studies meeting our a priori inclusion criteria limited to SSH were further defined in quality through risk bias assessment. RESULTS: We included 10 studies with 259 patients' pooled data post-screening 3083 abstracts. Within the cohort (n = 259), the prevalence of idiopathic, nontraumatic SSH with concomitant treatment with AC medications was greater 191 (73.75%) compared with AP treatment (27%). The lumbar spine was the most common site of hematoma (41.70%), followed by the cervical (22.01%) and thoracic (8.49%) spine. Most patients had surgical intervention (70.27%), and 29.73% had conservative management. The pooled data suggest that immediate diagnosis and intervention are the best prognostic factors in clinical outcomes. American Spinal Injury Association grading at initial symptom onset and post-treatment showed the greatest efficacy in symptomatic relief (87.64%) and return of motor and sensory symptoms (39.19%). CONCLUSIONS: Our review suggested that AC medications were related to SSH in most patients (74%), followed by APs (27%) and combined ACs + APs (1.9%). We recommend prompt intervention, a high suspicion for patients with neurologic deficits and diagnostic imaging before intervention to determine a case-specific treatment plan.

A rare case of adult-onset spastic paraparesis associated with Klinefelter syndrome.

REPORT: The rare association of Klinefelter syndrome and the clinical presentation of a late onset chronic progressive spastic paresis. CLINICAL PRESENTATION AND GENETICS: An infertile, 61-year-old man, presented with late adult onset of gait problems, deep muscle pain, and bladder problems. He presented for the first time, years after onset with a spastic paraparesis with high arched feet. His parents had already died, but the patient described high arched feet with his mother. There is no further certain information about the parents. After thorough investigation, an additional X chromosome was found, whereafter the diagnosis of Klinefelter syndrome could be made. Other acquired and genetic causes for spastic paraparesis or hereditary motor neuropathy are excluded. CONCLUSION: This rare case, together with three other literature reports by Sasaki (Intern Med 58(3):437-440, 2019), Sajra (Med Arh 61(1):52-53, 2007) and Matsubara et al., (J Neurol Neurosurg Psychiatry 57(5):640-642, 1994). suggests that Klinefelter syndrome can be associated with spastic paraparesis, besides the other various neuropsychiatric symptoms that are more commonly described.

Descriptive spectrum of thiamine deficiency in pregnancy: A potentially preventable condition.

OBJECTIVE: Pregnancy, a nutritionally demanding situation in terms of macro- and micronutrient supply owing to heightened maternal, placental, and fetal needs, significantly affects thiamine reserves. Thiamine deficiency during pregnancy and the postpartum period, presenting with varied manifestations and outcomes, is a relatively common condition in our population. The study aimed to understand the various manifestations and outcomes of acute thiamine deficiency in pregnant and postpartum women, emphasizing the significance of early recognition and thiamine therapy to prevent serious complications during pregnancy and after childbirth. METHODS: This prospective study conducted in a tertiary care center in North India enrolled consecutive pregnant and postpartum women presenting with clinical features consistent with thiamine deficiency disorders, such as thiamine deficiency-related neuropathy, high-output heart failure, heart failure with reduced ejection fraction, Wernicke's encephalopathy, gastric beriberi, and thiamine-responsive acute pulmonary hypertension. In addition to capturing medical history including drug intake, dietary consumption, and comorbidities, women underwent brief relevant clinical examinations and laboratory assessments, including whole-blood thiamine levels. Response to intravenous thiamine supplementation was also monitored. RESULTS: Data of 31 women (12 pregnant, 19 postpartum) with a diagnosis of acute thiamine deficiency and a mean age of 28.88 ± 2.69 years were analyzed. The mean thiamine level was 1.28 ± 0.44 µg/dL with mean blood lactate of 3.46 ± 3.33. The most common presentation was gastric beriberi (n = 10), followed by paraparesis (n = 6), high-output heart failure (n = 6), acute pulmonary hypertension, heart failure with reduced ejection fraction (n = 3 each), and an acute confusional state (n = 2). All patients responded to thiamine challenge. CONCLUSION: In the context of borderline thiamine status, particularly in our population with endemic thiamine deficiency and heightened demand for thiamine during pregnancy and the peripartum period, the deficiency can have varied and serious manifestations of dry and wet beriberi. Early recognition of the clinical features and thiamine therapy can be life-saving. There is a need for validated clinical criteria owing to the non-availability of thiamine testing in resource-limited settings.

Neisseria mucosa: A not so Benign Culprit of urinary tract infection: A case report.

Neisseria mucosa is saprophytic human commensal but reported as a causative agent in a couple of urinary tract infections [UTI] in susceptible individuals. In the present case, a young girl with long standing neurological problems presented with bladder outlet obstruction and fever. Her urine culture yielded Neisseria mucosa which was susceptible to broad spectrum penicillins, aminoglycosides, cephalosporins, ciprofloxacin, and azithromycin. She recovered with suitable dosage of amoxicillin clavulanic acid and was discharged. Isolation of N. mucosa here becomes clinically significant as this girl had various ureteric and lower limb weaknesses in past and was symptomatic for UTI with this infection.

[Early-onset familial Alzheimer's disease with spastic paraparesis associated with PSEN1 gene]. / Rannyaya semeinaya bolezn' Al'tsgeimera so spasticheskim paraparezom, svyazannaya s genom PSEN1.

A familial case of a rare autosomal dominant Alzheimer's disease (AD), related to PSEN1 gene (AD3, OMIM 607822), differing from common multifactorial form by earlier onset and, in part of cases, by accompanying neurological signs, spastic paraparesis particularly, is presented. The first sign in a female proband and in her son was paraparesis manifested at the age of 29 and 21 years, respectively. Cognitive disturbances developed soon; the former diagnosis was hereditary spastic paraplegia with cognitive impairment, In the proband examined in 2008 at 33 years old the diagnosis was not established. In the son examined in 2022 at 27 years old whole-exome sequencing detected a novel PSEN1 missense mutation p.Thr421Ala. The mutation was confirmed by Sanger sequencing in him, found out in the proband (who was severely disabled by that time) and excluded in her unaffected mother. Except for different age of onset, AD3 in two patients was similar, though in whole it is variable, also in relatives. The variability and rareness of the disease hampers clinical diagnostics. Massive parallel sequencing is a most reliable diagnostic method.

Advances in the treatment of human T-cell lymphotropic virus type-I associated myelopathy.

INTRODUCTION: Nearly 2-3% of those 10 to 20 million individuals infected with the Human T-cell lymphotropic virus type-1 (HTLV-1); are predisposed to developing HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). It is a neuro-inflammatory disease; differentiated from multiple sclerosis based on the presence of typical neurologic symptoms, confirmation of HTLV-1 infection, and other molecular biomarkers. AREAS COVERED: A brief review of the epidemiology, host immune responses, and molecular pathogenesis of HAM/TSP is followed by detailed discussions about the host-related risk factors for developing HAM/TSP and success/failure stories of the attempted management strategies. EXPERT OPINION: Currently, there is no effective treatment for HAM/TSP. Anti-retroviral therapy, peculiar cytokines (IFN-α), some anti-oxidants, and allograft bone marrow transplantation have been used for treating these patients with limited success. Under current conditions, asymptomatic carriers should be examined periodically by a neurologist for early signs of spinal cord injury. Then it is crucial to determine the progress rate to adapt the best management plan for each patient. Corticosteroid therapy is most beneficial in those with acute myelitis. However, slow-progressing patients are best managed using a combination of symptomatic and physical therapy. Additionally, preventive measures should be taken to decrease further spread of HTLV-1 infection.

Severe mononeuritis multiplex as a rare case of neuro leptospirosis: A case report.

Leptospirosis is a zoonotic bacterial disease caused by infection of spirochetes of the genus Leptospira. While typically self-limiting and non-fatal, severe manifestations can arise, including various neurological complications that are often overlooked. This case study presents a 59-year-old man with serologically positive Leptospirosis, who subsequently developed asymmetrical progressive leg weakness, severe back pain, and overflow incontinence suggestive of mononeuritis multiplex. Doxycycline treatment was started and intended to last for seven days. The patient had ongoing paraparesis, but all other problems were disappeared. The present case emphasizes the significance of identifying and treating neurological problems brought on by leptospirosis. To improve suitable treatment plans and patient outcomes, more research on these problems is necessary.

Remarkable improvement of neurological deficits after surgery in patients with Idiopathic spinal cord herniations. The impact of peroperative neuromonitoring. Case reports.

Introduction: Chronic Idiopathic Spinal Cord Herniation (ISCH) is a very rare spinal cord deformation occurring predominantly in thoracic levels. ISCH lead to progressive myelopathy, spastic paraparesis and Brown Séquard syndrome. Research question: We want to hypothesize that a) the herniated segment can regain its function after untethering despite long-term and complete neurologic dysfunction. b) Intraoperative Electrophysiologic Monitoring (IOEPM) may identify intraoperative changes by monitoring specific neural pathways confirming the efficacy of the intervention in the forthcoming cases. Material & method: It is a retrospective review of data of two cases prospectively collected showing improvement of neurological deficit in cases of ISCH in thoracic levels. We describe two patients with progressive neurological deficits due to ISCH who underwent surgery using electrophysiologic monitoring and have been followed to reach remarkable clinical improvement. Results: The spastic paraparesis of the first case improved remarkably after surgery. Complete foot drop of the other case, persistent for 7 months before intervention, improved after the release of the herniated segment of the cord. Peroperative electrophysiological monitoring did not show changes during surgery. Conclusion: We want to hypothesize that the herniated segment can regain its function after untethering despite long-term and complete neurologic dysfunction. Intraoperative Electrophysiologic Monitoring (IOEPM) may confirm the efficacy of the intervention in the forthcoming cases.

Pneumorrhachis Secondary to Klebsiella pneumoniae Gas-Forming Paraspinal Abscess: A Case report and Review of Literature.

Pneumorrhachis is a rare entity, where air pockets are found in the spinal canal and the etiology can be categorized into traumatic and non-traumatic, the latter further categorized into spontaneous, iatrogenic, and associated with infections. Infective causes are often associated with gas-forming organisms and are associated with significant morbidity and mortality. Often the diagnosis is not suspected until imaging is done. We report the case of a 57-year-old man who presented with fever, backache, lower leg weakness, and dysuria. A computed tomography scan for evaluation of intra-abdominal sepsis incidentally showed pneumorrhachis affecting the thoracic and lumbar levels, gas-forming paraspinal abscess, prostate abscess, liver cirrhosis, and sigmoid colon carcinoma. Blood culture isolated Klebsiella pneumoniae. The patient recovered after six weeks of intravenous antibiotics followed later by sigmoid colectomy and chemotherapy. A literature review identified 63 cases of pneumorrhachis associated with infections and can be categorized into infections with spontaneous pneumorrhachis (predominantly respiratory tract infections), infections with pneumorrhachis (predominantly with emphysematous infections), and iatrogenic with infections and pneumorrhachis (predominantly postspinal interventions). Infections with pneumorrhachis occurred in older age groups and were associated with higher mortality compared to the other two categories.