Thalassemia is a group of autosomal recessive hemoglobinopathies where exramedullary hemopoisis is frequently observed in the liver, lymph nodes, spleen and also around spinal cord which may cause spinal cord compression producing different clinical manifestations including para paresis or paraplegia. A case report of thoracic spinal cord compression in a 20-year-old male with Hb D-Hb E heterozygous thalassemia is reported from medicine department of Bangabandhu Sheikh Mujib Medical College, Faridpur. Bangladesh. This patient presented with progressive weakness of both lower limbs and difficulties in walking for 2 months. He also had history of fluctuating jaundice since childhood. His hemoglobin electrophoresis was suggestive of Hb D-Hb E heterozygous and MRI of dorsal spine with screening whole spine reveals extramedullary hemopoiesis at D2 to D12 causing spinal cord compression and low signal intensity of the bone marrow of the vertebrae. After blood transfusion he underwent referred to higher specialized center (National Institute of Neurosciences and Hospital, Dhaka) for further better management. Bangabandhu Sheikh Mujib Med. Coll. J. 2024;3(2):110-112

Retrospective longitudinal cohort study of veterans with SCI. Spinal cord injury (SCI) is associated with an increased risk of developing diabetes mellitus (DM), likely due to body composition alterations and autonomic nervous system dysfunction. These factors are more pronounced in persons with tetraplegia (TP) versus paraplegia (PP). However, the effect of level of injury (LOI) on DM incidence is largely unknown. Therefore, the objective is to examine the effect of LOI on DM incidence in persons with SCI. South Texas Veterans Health Care System. We obtained electronic record data on age, sex, race/ethnicity, LOI and HbA1c concentration from January 1st 2001 through December 31st 2021. Cox proportional hazard regression analyses were used to assess the association between LOI, DM and all-cause mortality. Among 728 non-diabetic veterans with SCI (350 TP/ 378 PP, 52 ± 15 years, 690 male/38 female) 243 developed DM, of which 116 with TP and 127 with PP. Despite chronological variations between TP and PP, DM risk over the entire follow-up did not differ between the groups (hazard ratio (HR): 1.06, 95% CI: 0.82-1.38). Mortality was higher in TP versus PP (HR: 1.40, 95% CI: 1.09-1.78). However, developing DM did not increase the risk of death, regardless of LOI (HR: 1.07, 95% CI: 0.83-1.37). Despite chronological variations between both groups, the level of injury had minimal effect on long-term DM development in this cohort of veterans with SCI. Sponsorship NIH (DK105379; MS), RR&D SPiRE (I21RX003724-01A1; MT and SH).

In able-bodied individuals, an increase in body mass index (BMI) enhances the risk of glucose tolerance deterioration and diabetes mellitus (DM). This relationship can be altered after spinal cord injury (SCI) due to muscle atrophy and the potential effects of autonomic dysfunction on glucose metabolism, which are more exaggerated in those with tetraplegia (TP) compared to paraplegia (PP). Here, we investigated the relationship between longitudinal changes in BMI and glycated haemoglobin (HbA1c), as well as the effect of level of injury (LOI) on this relationship, among nondiabetic patients with SCI. In a retrospective cohort of veterans with SCI, electronic data of BMI, LOI, glucose concentration and HbA1c were obtained from Jan 2001 to Dec 2021. A total of 728 patients without DM at baseline were identified (LOI: 350 TP/378 PP, age: 52±15 years, sex: 690 male/38 female), and followed up for a mean of 94 months (TP) and 114 months (PP). At baseline, using the SCI-specific BMI classification (normal weight BMI<22 kg/m2, obesity BMI> 25 kg/m2), 48% of TP and 65% of PP were obese at baseline (BMI TP: 25.6±5.9 kg/m2; PP: 28.0±6.6 kg/m2,; p<0.001). HbA1c at baseline was similar between TP and PP (5.21±0.59% vs 5.27±0.70%, p = 0.175). At the end of follow-up, BMI was increased to a larger extent in PP compared with TP (-0.02±4.11 kg/m2 in TP, +0.55±4.31 kg/m2 in PP; p = 0.05). However, HbA1c changes did not differ between TP versus PP (p = 0.323). The final BMI was associated with baseline BMI (p<0.001) and LOI (p = 0.007), but the last HbA1c value was only associated with baseline HbA1c (p<0.001) and not LOI (p = 0.821) or baseline BMI (p = 0.605). Together, these findings indicate that neither LOI nor BMI appear useful predictors of DM development in persons with SCI. Disclosure S. Hoekstra: None. M.J. Mader: None. M. Trbovich: None. M. Salehi: None.

The compression and decompression junction are a potential site for spinal cord distortion. Cord compression could result from the changed cerebrospinal fluid flow dynamics. Para paresis will occur due to direct trauma sustained during surgery or postoperative hematomas and there is 16% of secondary disease, because of non-traumatic injuries and 84% of spinal cord injury are due to trauma and 2 to 5 times the individuals will die prematurely due to spinal cord injury. Case report of a patient, having D8-L1 wedge compression fracture undergone surgical laminectomy, for which specific exercise protocol designed to improve muscle strength, activities of daily living and reduce clonus. post-operatively patient complained of weakness in bilateral lower limb, and he was dependent on others for his daily life activities, so our aim was to make the patient independent and he should be able to transfer from bed to wheelchair and reduce clonus. Tailored protocol was designed for patient’s functional independence by bed mobility, transfer skills, muscle strengthening by using different scales, and he was trained five sessions in a week for three months. After implementing the exercise program like strengthening exercises for bilateral upper limbs, bed mobility training, stretching of bilateral lower limb muscles for clonus and transfer skills using tailored protocol for twelve weeks there was improvement in all parameters. Keywords: Paraplegia, Functional independence, Tailored exercise

The effectiveness of evaporative cooling vest with ventilation fans on the thermal state of persons with paraplegia during exercise

Study designCross-sectional cohort study.ObjectivesTo compare ratings of perceived exertion (RPE) on Borg’s 6–20 RPE scale and Category Ratio 10 (CR10) in able-bodied (AB) participants during upper and lower body exercise, and recreationally active participants with paraplegia (PARA) and athletes with tetraplegia (TETRA) during upper body exercise only.SettingUniversity and rehabilitation centre-based laboratories in UK and Netherlands.MethodsTwenty-four participants were equally split between AB, PARA, and TETRA. AB performed maximal tests using cycle (AB-CYC) and handcycle (AB-HC) ergometry. PARA and TETRA performed maximal handcycle and wheelchair propulsion tests, respectively. Oxygen uptake (V̇O2) and blood lactate concentration were monitored throughout. RPE was rated each stage on Borg’s RPE scale and CR10. Thresholds were identified according to log-V̇O2 plotted against log-blood lactate (LT1), and 1.5 mmol L−1 greater than LT1 (LT2).ResultsRPE from both scales were best fit against each other using a quadratic model, with high goodness of fit between scales that was independent of exercise mode and participant group (range R2: 0.965–0.970, P < 0.005). Though percentage peak V̇O2 was significantly greater in TETRA (P < 0.005), there was no difference in RPE at LT1 or LT2 between groups on Borg’s RPE scale or CR10.ConclusionStrong association between Borg’s RPE scale and CR10 suggests they can be used interchangeably. RPE at lactate thresholds were independent of mode of exercise and level of spinal cord injury. However, inter-individual variation precludes from making firm recommendations about using RPE for prescribing homogenous exercise intensity.

Hydatid cyst disease is caused by the parasite Echinococcus granulosus. Hydatidosis affecting the spine constitutes only 1% of cases. In 90% of the spinal cases, the disease is confined to the bone and the epidural space. Here we report a 25-year-old female patient, followed since the age of 3 for hepatic, renal and peritoneal hydatidosis, undergoing medical treatment (Albendazole). Operated 15 years ago (dorsal laminectomy) for spinal cord compression in consequence of the extension of the disease to the spinal cord at D12-L1 level. The course was marked by the progressive worsening of gait disorders with spinal deformity. The current examination shows a steppage gait and para paresis, without sensitive disorders. The osteotendinous reflexes are abolished in the 2 lower limbs. We also noted the presence of a significant thoracolumbar kyphoscoliosis. A spinal MRI demonstrated a recurrence of hydatidosis centered on the vertebral body of D11 which is practically destroyed with right and left para-vertebral extensions at D10, D11, D12 and epiduritis in D11 (A, B, C, D). Spinal CT confirms the almost complete lysis of the D11 vertebral body (Image E). Albendazole (400 mg p.o.) is administered twice daily. A Spondylectomy D11, curettage of the lesion and aspiration of necrotic tissue with spinal stabilization were proposed. Her situation remained unchanged after 4 months of follow-up. Although the hydatid cyst is characterized as benign pathology, in consequence of its local growing pattern, it can be classified in the malign group because of its high potential for dissemination, which can result in high incidence of recurrences.

Clutched elastic actuator to drive a lower limb exoskeleton hip joint for paraplegic patients

Background: Hereditary Spastic Paraplegia (HSP) is a neurological disorder associated with causing progressive spasticity in the lower limb of humans. In this study, the computational analysis was limb of humans. In this study, the computational analysis was performed to screen out the pathogenic missense and splicing variants of Spartin. The mutations in this mitochondrial protein can subsequently lead to HSP. Method: To discover novel mutations of Spartin protein, the missense and variants were obtained from gnomAD (Genome Aggregation Database), and further subjected to CADD (Combined Annotation Dependent Depletion) analysis. To validate the results, it was compared with various in silico mutation analysis tools. To accomplish novelty in the recent work, the mutations were analyzed and compared with the web- based tool ClinVar for finding novel mutations. Results: After stringent analysis, 3 missense mutations and 4 spicing mutations were obtained which have not been previously reported in any kind of databases or scientific work, thus can be considered as novel.

Acute Flaccid Paralysis (AFP) is a rare serious neurological condition characterized by sudden weakness or paralysis of one or more extremities, the respiratory or bulbar muscles and reduced muscle tone without other obvious cause. Death occurs in about 7.5% of AFP affected patients worldwide. AFP is the most common sign of acute polio. Therefore, AFP studies are important for surveillance during polio outbreaks to differentiate polio cases from AFP cases. There is a lack of information about the clinical features & causative factors of AFP among children in Gadarif, Sudan. The identification of AFP cases and its causative factors are important in the management & prevention of the disease. This study assessed the etiology and the clinical features of AFP among children in Gadarif, Sudan aiming at effective management & prevention of the disease. It was a prospective cross sectional study conducted at Gadarif Pediatrics Teaching Hospital during the period of January 2017-December 2019. It comprised 73 children with confirmed AFP, ages 6 months to < 15 years old. Our study revealed that Poliomyelitis was not the cause of AFP. AFP was significantly affected children with youngest ages 0-5 years old and from the rural areas. Guillain-Barre syndrome (GBS) was the leading cause of AFP followed by meningitis, traumatic neuritis and hypokalemia and we believed that infections were the main triggers of GBS. Gender did not affect the prevalence of the AFP. Fever and paraplegia were the most prevalent clinical signs at onset of the weakness. Over 50% of the AFP victims showed symmetric paralysis. It is obviously that AFP-based awareness, provision of high-quality health services and fighting of illiteracy and poverty in the rural areas of Sudan are urgently needed for effective management of AFP.

Ewing sarcoma is typically seen in children involving long bones. Although well described, its presentation in extraskeletal tissues is relatively rare and is classified as an Ewing sarcoma family of tumors. They are mostly curable when they occur in children. An extraskeletal Ewing sarcoma in adults is uncommon, limiting the experience in adult oncologists. The biopsy is essential for definitive diagnosis, which shows small round blue cells that must be differentiated from lymphoma, embryonal rhabdomyosarcoma, and small cell carcinoma. Management is multimodal, involving surgery, radiation for local treatment of primary tumor, and systemic chemotherapy. A multidisciplinary approach, coupled with risk-adapted intensive neoadjuvant and adjuvant multi-agent chemotherapies and other modalities such as radiation and surgery for control of the primary site and metastatic disease, is needed. The primary multidrug chemotherapy regimen consists of alternating cycles of vincristine/doxorubicin/cyclophosphamide (VDC) and ifosfamide/etoposide (IE) given every 2 weeks with growth factor support. Prognosis and the 5-year survival rate are better for localized than the metastatic disease, and in metastatic disease, it is better for patients with lung metastasis than other metastatic disease sites. We describe a rare extraskeletal tumor arising from a lung that tested positive for Ewing sarcoma, also known as Askin’s tumor in a young adult. In our case, the tumor rapidly metastasized locally to involve the thoracic spine causing paraparesis. Timely diagnosis and early management are essential to improve outcomes. We also present how treatment can be delayed due to sepsis and emphasize the careful multispecialty approach’s importance. J Med Cases. 2020;11(12):388-393 doi: https://doi.org/10.14740/jmc3582

Introduction: HTLV-1 associated myelopathy/ tropical spastic paraparesis (HAM/TSP) is a chronic neuro-inflammatory disease related to human T lymphotropic virus type 1 (HTLV-1) infection. Interferon type III (IFN-λ), which includes IL28, IL29, and IL28R, and affects the outcome of viral infections, might be complicated in the progression of HAM/TSP.

Experimental study on the effectiveness of the PCM cooling vest in persons with paraplegia of varying levels.

Objective: Cardiovascular disease (CVD) is a leading cause of mortality in persons with SCI. While macrovascular remodeling and function after SCI is well documented, changes in the microvascular structure and function are comparably understudied, but importantly predict CVD risk. Specifically, the integrity of venoarteriolar (VAR), myogenic (MYO) and maximal vasodilation responses are largely unknown after SCI, especially in persons with tetraplegia (TP) at highest risk of CVD. This is the first to examine the differences in VAR (cuff inflation), MYO (limb dependency) and maximal vasodilation responses of the microvasculature between able bodied (AB) versus those with TP and paraplegia (PP). Design: Observational. Setting: Laboratory. Participants: Eight AB, 6 TP, and 8 PP persons. Interventions: One forearm and calf were treated topically with lidocaine 2.5%/prilocaine 2.5% while contralateral limb served as a control. Laser doppler flowmeters were applied over treated and control sites during limb dependency, cuff inflation and local skin heating (Tloc) up to 42°C. Outcome measures: Skin vascular resistance (SkVR) change with cuff inflation and limb dependency and maximal cutaneous vascular conductance (CVC) during local heating. Results: Change in SkVR was not significantly different between groups or extremity (upper vs. lower) during cuff inflation or limb dependency. However, CVC at Tloc 42°C was significantly different in the lower extremity (LE) of TP and PP (P = 0.007, 0.35) compared to AB. Conclusion: Increases in SkVR during cuff inflation (VAR) and limb dependency (VAR and MYO) are unaltered after SCI, however maximal vasodilation in the LE post-SCI is higher than AB persons.

Background: Conversion disorder, also called functional neurological symptom disorder in the Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5), is an illness of symptoms or deficits that affect voluntary motor or sensory functions, which suggest another medical condition, but that is judged to be caused by psychological factors because the illness is preceded by conflicts or other stressors.any case of FND is challenging and hard to manage due to overlapping of symptoms with other organic illnesses and also hard to be treated due to multidisciplinary approach needed. Case Report: A 34 year old patient known to have schizophrenia with previous admissions to psychiatric hospital suddenly complaining of paraplegia with no sensory deficit . these symptoms persist for 6 months. Conclusion: Long lasting symptoms is something unusually seen in conversion disorder cases where the course of illness stay for a couple of days.

Objective: Thermoregulatory dysfunction after spinal cord injury (SCI) impairs quality of life and predisposes persons to life-threatening sequela of heat-related illness (HRI) in conditions of high ambient temperature. SCI clinicians currently have no objective way to predict which persons are at greatest risk of HRI. Evaporative cooling via sweating is the body’s most efficient mechanism of heat dissipation. The relationship between the neurological level of injury (NLOI) and the degree of sudomotor dysfunction is not well defined. This study examines the relationship between the NLOI and sweating level of injury (SwLOI). This information can assist SCI clinicians in identifying individuals with SCI who have most impaired sudomotor function and thus highest risk of HRI. Design: Observational. Setting: Human physiology laboratory. Participants: 10 persons with tetraplegia (TP), 14 with paraplegia (PP) and 10 able-bodied (AB). Intervention: Passive heat stress (1°C rise in core temperature) with sweat responses (SR) quantified with the starch iodine test. Outcome measures: The most caudal dermatomal level in which sweating was visualized was recorded as the SwLOI, which was compared to the NLOI. Minimum, maximum and median differences between NLOI and SwLOI were calculated. Results: Persons with tetraplegia demonstrated no SR. Persons with paraplegia demonstrated SR at a median of 1 level below NLOI. Able-bodied controls demonstrated sweating on all skin surface areas. Conclusions: Persons with motor complete tetraplegia lack evaporative cooling capacity through SR during passive heat stress predisposing them to HRI. Meanwhile, persons with paraplegia sweat on average 1 dermatomal level below their NLOI.

Objective To summarize the surgical treatment and it’s results of patients with acute type A aortic dissection with cerebral malperfusion. Methods We collected and analyzed the clinical date of 16 patients, who were hospitalized and underwent operation Between January 2010 to June 2019, presented cerebral malperfusion due to acute type A aortic dissection. All of them showed preoperative newly developed neurologic deficits, left hemiplegia in 8 cases, left paraparesis in 5 cases and right paraparesis in 3 cases. Results Aortic valve was involved in 5 patients, 2 of whom underwent AVR surgery, 2 cases of CABG and 2 cases of artificial graft bypass were performed at the same time, 1 case underwent left leg amputation postoperatively. Seven patients died after surgery. Conclusion The results of surgical management of acute type A aortic dissection with cerebral malperfusion demonstrated high hospital mortality, which needs us to restore the cerebral perfusion as soon as possible. The specific method should be decided according to the patients' condition. Key words: Acute type A aortic dissection; Cerebral malperfusion

Degenerative spondylotic myelopathy is the most common cause of spinal dysfunction, as well as nontraumatic spastic paraparesis and quadriparesis. Although conventional MRI is the gold standard for radiographic evaluation of the spinal cord, it has limited application for determining prognosis and recovery. In the last decade, diffusion tensor imaging (DTI), which is based on the property of preferential diffusion of water molecules, has gained popularity in evaluating patients with cervical spondylotic myelopathy (CSM). The use of DTI allows for evaluation of microstructural changes in the spinal cord not otherwise detected on routine conventional MRI. In this review, the authors describe the application of DTI in CSM evaluation and its role as an imaging biomarker to predict disease severity and prognosis.

Thoracic and abdominal aortic aneurysm repair has always been a challenging procedure in cardiovascular surgery due to its high mortality and complication rate. At present, there are traditional open surgery, hybrid surgery and intracavitary repair for thoracic and abdominal aortic aneurysms. The main postoperative complication of spinal cord ischemia injury is paraplegia, which seriously affects the long-term survival rate and quality of life of patients. This paper summarized and considered the pathogenesis, risk factors and prevention measures of spinal cord ischemia injury after thoracic and abdominal aortic aneurysm repair.

The aim of this study is to relate the condition of paraplegia due to schistosomal myeloradiculopathy with an initial diagnosis of polyradiculoneuropathy in a patient from an endemic area This disease is the most severe and disabling ectopic form of Schistosoma mansoni infection being underreported of unknown prevalence and underestimated morbidity Its recognition and the early institution of treatment play a fundamental role in the prevention of severe and irreversible injuries as well as in the recovery of affected people usually young people in full production phase The diagnosis of schistosomal myeloradiculopathy is based on epidemiology clinical findings regarding neurological conditions and evidence of Schistosome mansoni in laboratory tests such as serological techniques or stool parasitological examination and other causes for the condition should be excluded In the case described the patient reported swimming in a lagoon in Sergipe an endemic zone which makes the epidemiology speak strongly in favor of the diagnostic hypothesis The clinical presentation presented by the patient is very similar to that of this myeloradiculopathy and that Schistosoma eggs were found in the feces and the serology and cerebrospinal fluid analysis CSF serology were positive for this parasite It is noteworthy that magnetic resonance imaging is a very sensitive propaedeutic method for the evaluation of schistosomal myeloradiculopathy although the diagnosed alterations are not specific for this disease The changes observed on magnetic resonance imaging MRI regress in parallel with the clinical improvement of the patient after treatment with schistosomicides and corticosteroids as can be observed in this case Finally the treatment was done with praziquantel ivermectin and corticosteroid therapy in association with motor physiotherapy The patient presented clinical improvement but maintained mild motor deficit