Leber’s Hereditary Optic Neuropathy Plus Causing Recurrent Myelopathy due to an MT-DN1 Mutation at G3635A

A 51-year-old man with known Leber’s hereditary optic neuropathy (LHON) presented with worsening lower extremity weakness and numbness. Following an episode of myelopathy two years before, he had been ambulating with a walker but over two weeks became wheelchair bound. He also developed a sensory level below the T4 dermatome to light touch, pinprick, and vibration. MRI of his cervical and thoracic spine showed a nonenhancing T2 hyperintense lesion extending from C2 to T12. At his presentation two years earlier, he was found to have a longitudinally extensive myelopathy attributed to his LHON. Genetic testing revealed a 3635 guanine to adenine mutation. MRI at that presentation demonstrated a C1-T10 lesion involving the central and posterior cord but, unlike the new lesion, did not involve the ventral and lateral horns. Given the similarity to his prior presentation and a negative evaluation for alternative etiologies, he was thought to have recurrent myelopathy secondary to Leber’s Plus. To our knowledge, recurrent myelopathy due specifically to the G3635A mutation in Leber’s Plus has not been reported previously.

Four weeks of electrical stimulation improves glucose tolerance in sedentary overweight or obese Hispanic population

Introduction/Purpose: Most U.S. adults (54%) do not meet minimum exercise recommendations by American College of Sports Medicine (ACSM). Neuromuscular electrical stimulation (NMES) is a novel alternate strategy to induce muscle contraction. However, effectiveness of NMES to improve insulin sensitivity and energy expenditure is unclear. The purpose of this study was to investigate the effects of four weeks of NMES on glucose tolerance in a sedentary overweight or obese population. Methods: Participants (n=10; age: 36.8 ± 3.8 years; BMI=32 ± 1.3 kg/ m2) were randomized into either control or NMES group. All participants received bilateral quadriceps stimulation (12 sessions; 30 minutes/session; 3 times/week at 50 Hz and 300 µs pulse width) altering pulse amplitude to either provide low intensity sensory level (control; tingling sensation) or at high intensity neuromuscular level (NMES; maximum tolerable levels with visible muscle contraction). Glucose tolerance was assessed by three-hour oral glucose tolerance test (OGTT), substrate utilization was measured by indirect calorimetry and body composition via dual X-ray absorptiometry at baseline and after four weeks of NMES intervention. Results: Control and NMES groups had comparable fasting blood glucose, glucose tolerance, substrate utilization, and muscle mass at baseline. Four weeks of NMES resulted in a significant improvement in glucose tolerance measured by OGTT, whereas no change was observed in control group. There was no change in substrate utilization and in muscle mass in both control and NMES groups. Conclusion: NMES is a novel and effective strategy to improve glucose tolerance in an at-risk overweight or obese sedentary population.

Perioperative Outcomes in COVID-19 Obstetric Patients Undergoing Spinal Anesthesia for Cesarean Section: A Prospective Observational Study

Coronavirus disease 2019 (COVID-19) adds more challenges to the perioperative management of parturients. The aim of this study is to examine perioperative adverse events and hemodynamic stability among COVID-19 positive parturients undergoing spinal anesthesia. This prospective observational investigation was conducted at a tertiary teaching hospital in Jordan between January and June 2021, during which 31 COVID-19 positive parturients were identified. Each COVID-19 positive parturient was matched with a COVID-19 negative parturient who received anesthesia under similar operating conditions as a control group. Of the 31 COVID-19 patients, 22 (71%) were otherwise medically free, 8 (25.8%) were emergency cesarean sections. The sensory level of spinal block after 10 min was T8 (T6–T10) among COVID-19 positive group, compared to T4 (T4–T6) among control group (p = 0.001). There were no significant differences in heart rate, SBP, DBP, and MAP intraoperatively (p > 0.05). Twelve (36.4%) neonates born to COVID-19 positive patients were admitted to NICU, compared to four (11.8%) among control group (p = 0.018). There was no statistically significant difference in postoperative complications. In conclusion, spinal anesthesia is considered a safe anesthetic technique in COVID-19 parturients, and therefore it is the anesthetic method of choice for cesarean deliveries among COVID-19 patients.

Longitudinally Extensive Transverse Myelitis (LETM) in COVID-19 Infection, A Case-Report

IntroductionSince the COVID-19 pandemic, a growing number of central nervous system (CNS) complications in patients with COVID-19 have been reported. Isolated, longitudinally extensive transverse myelitis (LETM), is a unique presentation of CNS involvement. The limited reports, its diverse clinical manifestations and the possible long-term consequences make the reporting crucial to further our understanding of those syndromes occurring in COVID-19 positive patients.Case PresentationA 63-year old male consulted the emergency department after a sudden onset of gait ataxia, a one-week history of paresthesia progressing from the feet to the midsternal area and urinary. He tested positive on a SARS-CoV-2 RNA RT-PCR nasopharyngeal swab two days prior to the onset of his symptoms. Neurological examination showed a sensory level at T7 with symmetrically reduced fine touch, vibration, proprioception and furthermore an ataxic gait was observed. Cerebrospinal fluid on day one of admission showed pleocytosis, predominantly neutrophils, elevated protein count and normal glucose level and IgG. MRI of the spinal cord revealed a diffusely increased signal intensity involving the near-complete spinal cord, from the brainstem to level T12, fitting the diagnosis of LETM. ConclusionThe few cases of transverse myelitis in association with COVID-infection are believed to have an immune-mediated postinfectious mechanism. In this case however, parainfectious direct viral invasion of the spinal cord is far more likely because of a neutrophilic predominance in CSF and a short timespan between infection and symptoms. It could provide more clues that the SARS-CoV-2 is acutally capable of causing direct neurotoxic effects.

Optimum dose of spinal ropivacaine with or without single intravenous bolus of S-ketamine during elective cesarean delivery: a randomized, double-blind, sequential dose-finding study

Background Maternal hypotension after spinal anaesthesia occurs at a high rate during caesarean delivery and can lead to adverse maternal or foetal outcomes. The aim of this study was to determine the optimal dose of spinal ropivacaine for caesarean section with or without intravenous single bolus of S-ketamine and to observe the rates of hypotension associated with both methods. Methods Eighty women undergoing elective caesarean delivery were randomly allocated into either a ropivacaine only or ropivacaine with intravenous S-ketamine group. If the upper sensory level of the patient reached T6 and the visual analogue scale (VAS) scores remained below 3 points before delivery, the next patient had a 1/9th chance of receiving a lower dose or an 8/9th chance of receiving the same dose as the previous patient. If the patient had VAS scores of more than 2 points or needed an extra epidural rescue bolus before delivery, a higher dose was used for the next patient. The primary outcome was the successful use of spinal ropivacaine to maintain patient VAS score of < 3 points before delivery and the incidence of post-spinal hypotension in both groups. Secondary outcomes included the rates of hypotension-related symptoms and interventions, upper sensory level of anaesthesia, level of sedation, neonatal outcomes, Edinburgh Postnatal Depression Scale scores at admission and discharge, and post-operative analgesic effect. The 90% effective dose (ED90) and 95% confidence interval (95% CI) were estimated by isotonic regression. Results The estimated ED90 of ropivacaine was 11.8 mg (95% CI: 11.7–12.7) with and 14.7 mg (95% CI: 14.6–16.0) without intravenous S-ketamine, using biased coin up-down sequential dose-finding method. The rates of hypotension and associated symptoms were significantly lower in S-ketamine group than in the ropivacaine only group. Conclusions A spinal dose of ropivacaine 12 mg with a single intravenous 0.15 mg/kg bolus dose of S-ketamine may significantly reduce the risk of hypotension and induce sedation before delivery. This method may be used with appropriate caution for women undergoing elective caesarean delivery and at a high risk of hypotension or experiencing extreme nervousness. Trial registration http://www.chictr.org.cn (ChiCTR2000040375; 28/11/2020).

Long-term survival following resection of extracompartmental osteosarcoma of the spine – A case report and literature review

Background: Osteosarcoma, representing 3% of sarcomas, rarely involves the axial skeleton. The overall 5-year survival is just 18%. Here, we present a 15-year-old female with an extracompartmental osteosarcoma, who following radical spine surgery, chemotherapy, and intensive radiotherapy remained disease-free 15.5 years later. Case Description: A 15-year-old female presented with an acute right lower extremity monoparesis and T4 relative sensory level attributed to a T4 extracompartmental Osteosarcoma. Following circumferential spine surgery, chemotherapy, and radiotherapy, her tumor did not recur over the 15.5-year follow-up period. Conclusion: Osteosarcoma rarely presents focally in the spine. When it originates in the spine, there are typically few long-term survivors. Here, we report a 15-year-old female who presented with an acute monoparesis attributed to an extracompartmental T4 spinal osteosarcoma. Following circumferential tumor resection, adjuvant chemotherapy, and radiotherapy, the patient remains disease-free 15.5 years later.

NCMP-11. SPINAL CORD INFARCT AFTER CAR-T TREATMENT

Cortical and subcortical neurotoxicity from CAR-T therapy is a well described complication in literature, with over 40% of patients experiencing at least one neurologic side effect. However, spinal cord toxicity from CAR-T therapy is less well described. To our knowledge, this is the first reported case of a spinal cord infarct following CAR-T therapy. A 44 year old male with primary refractory DLBCL without CNS involvement, which was refractory to R-CHOP, R-ICE, and hyperCVAD part B underwent CD-19 CAR-T treatment. The day after infusion he developed grade 1 cytokine release syndrome (CRS) with fever and up trending inflammatory markers. Infectious work up was negative and he was treated with tocilizumab and dexamethasone. His fever resolved and markers down trended. On day 5 post CAR-T, he became encephalopathic, developed severe back pain, and was unable to move his bilateral lower extremities. He was treated with 2nd and 3rd doses of tocilizumab, dexamethasone and was started on anakinra. Patient’s mental status cleared by day 7 and he was found to have a dermatome sensory level at T10 with flaccid bilateral lower extremity paralysis. MRI Brain was unremarkable, but a spinal MRI showed longitudinally extensive cord edema and diffusion restriction at T10. Due to an initial question of transverse myelitis, he was treated with a 3-day course of IV methylprednisolone, with no improvement in symptoms. CSF studies were unable to be obtained due to his thrombocytopenia. Repeat MRI obtained 10 days after initial imaging showed resolution of cord edema, but continued areas of FLAIR hyperintensity at T10 through the conus. Despite aggressive rehabilitation services, four months later, patient remained paralyzed in his lower extremities with an indwelling foley catheter. He remains in a complete remission.

COMPARISONS OF EFFECTS OF DIFFERENT DOSES OF DEXMEDETOMIDINE AS ADDITIVE IN INTRATHECAL BLOCKADE WITH 0.5% HYPERBARIC BUPIVACAINE IN LOWER LIMB ORTHOPEDIC SURGERIES

INTRODUCTION Lower limb surgeries may be performed under local, regional (spinal or epidural) or general anaesthesia, but neuraxial blockade is the preferred mode of anaesthesia. Spinal block is still therst choice because of its rapid onset, superior blockade, low risk of infection as from catheter in situ, 1 less failure rates and cost-effectiveness. Intrathecal local anaesthesia alone is associated with relatively short duration of action and thus early analgesic intervention is needed in post-operative period. AIMS AND OBJECTIVES With use of dexmedetomidine in 3 different graded doses with hyperbaric bupivacaine intrathecally as regional anaesthesia for lower limb surgeries. MATERIALS AND METHODOLOGY This Prospective double blinded randomized controlled study. The study will be conducted in adult patients aged between 18-50 years undergoing lower limb surgeries under spinal anaesthesia in orthopedic OT, dept. of Anaethesiology, Medical college & hospital, Kolkata. Duration of the study One year (9 months for data collection & 3 months for data analysis, review& report writing). Total 63 patients in our study. RESULTS We found the mean of two segment regression time from highest sensory level in Group B was 130.56min; in group C was 171.34min; ingroup D was 217.85min So, block regression was signicantly slower with the addition of intrathecal dexmedetomidine (Group D) as compared to group C & B ( P < 0.0001) The mean regression time to S1 from highest sensory level for Group Bwas 289.43 min ( SD- 12.43) For Group C, the mean regression time to S1 from highest sensory level was 402.71 min ( SD- 28.60) For Group D, the mean regression time to S1 from highest sensory level was 584.43min ( SD- 38.92) Overall the mean regression time to S1 from highest sensory level was 425.52 min ( SD- 125.71) The sensory regression time to S1 from highest sensory level was signicantly higher with increasing dose of dexmedetomidine i. e. D>C>B . (P< 0.0001). CONCLUSION We recommend the use of 10mcg of intrathecal dexmedetomidine as an adjuvant to bupivacaine as it seems to be a good alternative to other additives for long duration surgical procedures due to its profound intrathecal anesthetic and analgesic properties. It provides good quality of intraoperative analgesia, thermodynamically stable conditions, minimal side effects, and excellent quality of postoperative analgesia.

Progressive Myelopathy After Neck Pain

A 36-year-old woman with a history of hypothyroidism, gout, fibromyalgia, depression, substance use disorder, and nephrolithiasis had development of neck pain. Three months later, she noted numbness in the left leg, which slowly worsened over the course of several months, spreading to involve the right leg and eventually forming a sensory level across the trunk at T8. At that time she also noted numbness in both hands. She had stiffness and weakness in both legs and had trouble emptying her bladder. Neurologic examination showed mild weakness restricted to the bilateral iliopsoas and hyperreflexia in the upper and lower extremities. Hoffmann and Babinski signs were positive bilaterally. There was moderate spasticity in both lower extremities and mild distal vibratory sensation loss, with a sensory level across the trunk at T8. Her gait examination indicated a spastic gait, and she had a mildly positive Romberg sign. On re-evaluation of her previous magnetic resonance image, a transverse band or pancakelike enhancement pattern was noted at the center of a moderate to severely stenotic region of the cervical spine sparing gray matter on axial sequences. The magnetic resonance imaging findings were highly suggestive of cervical spondylotic myelopathy. A neurosurgical referral was made, and the patient underwent anterior cervical discectomy with decompression and fusion from C4-C7. At her follow-up visit 4 months after surgery, the patient reported improvement in her strength and walking. Her neurologic examination showed normal lower extremity strength, resolution of spasticity, and negative Babinski sign bilaterally but persistent sensory deficits. Magnetic resonance imaging of the cervical spine at that time showed a decrease in the degree of T2 hyperintensity and enhancement, consistent with interval response to surgery. The presence of a progressive myelopathy over many months in this case patient argued against a diagnosis of transverse myelitis. Furthermore, the cerebrospinal fluid was noninflammatory, which also favored cervical spondylosis over idiopathic transverse myelitis. However, the gadolinium enhancement pattern was the key diagnostic feature that strongly suggested cervical spondylotic myelopathy as the diagnosis and ultimately led to neurosurgical referral for decompression.

Progressive Myelopathy After Spine Surgery

A 69-year-old man with a progressive myelopathy for 2 years was referred for evaluation of suspected transverse myelitis. His medical history included discectomies, a severe episode of herpes simplex virus type 1 meningoencephalitis, and development of insidiously progressive numbness and weakness of his hands. Cervical spine magnetic resonance imaging showed 2 small, dural-based, gadolinium-enhancing lesions. Biopsy of these lesions showed only normal neural tissue. Subsequently, the dura was stripped away surgically from the lower cervical region, in an effort to remove these lesions. During the next year, a sensory level developed at about the level of the nipples (T4), along with a squeezing sensation on his trunk below. Imbalance and bilateral lower extremity weakness and numbness then developed. Magnetic resonance imaging showed a longitudinally extensive cord signal abnormality. The cause of the patient’s initial subjective hand numbness and weakness was indeterminate. The onset of severely progressive symptoms after surgical removal of those lesions and the reported stripping of dura made it likely that the progressive cord edema was due to chronic adhesive arachnoiditis. His prior meningoencephalitis was a potential additional risk factor for arachnoiditis. Computed tomography myelography showed a markedly abnormal spinal canal with scalloping of the cord contour, with delayed flow of contrast above C6-C7, consistent with arachnoid adhesions causing obstruction of normal cerebrospinal fluid flow. The patient was diagnosed with chronic adhesive arachnoiditis. A C4-C7 laminectomy and surgical lysis of the cord meningeal adhesions was performed, with subsequent intensive neurorehabilitation. Follow-up spinal cord magnetic resonance imaging 6 months after surgery showed improvement of the T2-signal abnormality but persistent myelomalacia and spinal cord atrophy. Adhesive arachnoiditis is an uncommon cause of progressive myelopathy resulting from an insult to the arachnoid meningeal layer, followed by inflammation and fibrosis. This process renders the arachnoid abnormally thick and adherent to the pia and dura mater. Abnormal adhesion of nerve roots or spinal cord to the dura produces neurologic impairment. Typical symptoms include back pain, paresthesias, lower limb weakness, and sensory loss. It is diagnosed clinically with supportive magnetic resonance imaging and computed tomography myelography findings.