Case Of Subacute Combined Degeneration Research Articles

Case Series of Nitrous Oxide Related Subacute Combined Degeneration from 2016 to 2022 (P9-4.002)

<h3>Objective:</h3> To report laboratory, radiologic, and clinical findings in a case series of patients hospitalized with subacute combined degeneration (SCD) caused by nitrous oxide (NO) abuse. <h3>Background:</h3> NO is legal and widely available as a food additive, making it an easily accessible recreational drug. NO inhibits methionine synthase and inactivates vitamin B12. Prolonged use can lead to B12 deficiency and SCD. <h3>Design/Methods:</h3> We reviewed hospital records from March 2016 until August 2022 and found three cases of SCD associated with NO abuse. <h3>Results:</h3> One 43-year-old male and two females, 27 and 44 years old, had SCD in the setting of NO abuse. All had numbness, weakness, and ataxia. Cognitive and behavioral changes with loss of vibratory sensation and proprioception were not universal. MRI of the cervical cord in all patients demonstrated T2 hyperintensity of the posterior columns in the classic “inverted V” shape consistent with SCD. B12 levels in two patients were 0.54 and 0.73X lower limit of normal (LLN), while in the third patient it was 2.3X the LLN. Methylmalonic acid (MMA) and homocysteine levels were elevated in all patients up to 34X the upper limit of normal. All patients were treated with high-dose B12 repletion, physical therapy, and counseled on nitrous oxide cessation. Two patients were discharged home, while one was discharged to a skilled facility for further rehabilitation. <h3>Conclusions:</h3> NO abuse can induce SCD. Patients may have a normal B12 level but still be functionally B12 deficient because NO inactivates the B12 molecule. Elevated MMA and homocysteine levels are consistent with B12 inactivation as well as deficiency, illustrating the utility of these additional diagnostic tests when B12 deficiency is suspected. Profound neurological deficits can persist at the time of hospital discharge in NO-related SCD. <b>Disclosure:</b> Dr. Warren has nothing to disclose. Dr. Taylor has nothing to disclose. Dr. Savage has nothing to disclose.

An interesting case of subacute combined degeneration

Vitamin B12 deficiency is a common cause of both hematological and neurological impairment, not necessarily together. The diagnosis maybe challenging sometimes. This case presents a classical magnetic ressonance imaging sign of severe subacute combined degeneration in spinal cord.&#x0D;

V for vitamin, inverted V for lack of it: A rare case of subacute combined degeneration of cord with bladder symptoms and associated multiple Tarlov cysts

Subacute degeneration of cord (SACD) is a rare presentation of a common disease and it should be kept in mind when the patient develops symptoms of peripheral neuropathy. This is a case of a 65-year-old male who presented with pins and needles sensation of bilateral hands and feet and with difficulty in voiding. Neurological examination showed large fiber neuropathy with an exaggerated knee jerk, absent ankle jerk, extensor plantar, and a positive Romberg’s sign. Routine laboratory investigations showed low hemoglobin and low vitamin B12 levels. Magnetic resonance imaging of the spinal cord showed a hyperintense lesion in the posterior aspect of the cervical cord with a classical “inverted V” sign on the axial section which is suggestive of SACD. Coincidentally, there were multiple Tarlov cysts present. Finally, the patient improved with vitamin B12 injections. This case is unique as an association of SACD with Tarlov cysts with a classical inverted V sign has not been reported according to the literature.

Prognostic indicators of subacute combined degeneration from B12 deficiency: A systematic review.

A systematic review was conducted on published data of subacute combined degeneration (SCD) from B12 deficiency to investigate potential prognostic indicators of final ambulatory function in affected patients. TYPE: Systematic review. Records published from 1999 through 2018 were searched on the electronic databases MEDLINE, PUBMED, and SCOPUS. The publication language was restricted to English and French. Records that described cases of SCD from B12 deficiency in patients ≥16 years of age at onset were included. From a final total of 66 cases of SCD identified, ambulation scores were assigned based on the clinical descriptions reported. Patient characteristics, clinical manifestations, and ambulatory function were reported descriptively. Subanalyses on potential prognostic indicators were performed. Greater ambulatory function at diagnosis was associated with recovery of normal ambulatory function at follow-up (P <.001). Nearly 90% of patients walking unsupported at diagnosis made a complete recovery regardless of etiology. For patients initially walking with support, 22% of cases from impaired B12 digestion/absorption made a complete recovery compared with ≥50% of cases from other etiologies. For patients initially requiring a wheelchair, 33% of cases from impaired digestion/absorption were able to walk with support compared with ≥50% of cases from other etiologies. The total B12 administered over the course of treatment was correlated with improved ambulation (P=.024) for all patients, with a higher B12 replacement regimen associated with better outcomes in patients who were unable to walk unsupported at diagnosis (number needed to treat=4). Initial ambulatory function may be a useful clinical marker of the severity of spinal cord dysfunction and final functional outcome. Etiological risk factors influenced both clinical manifestations and final walking ability in SCD, suggesting a distinct pathophysiological mechanism among the causes. In addition, a more intensive B12 replacement regimen may improve ultimate ambulatory function in advanced cases of SCD.

The Level of Serum Pepsinogen in Diagnosing and Evaluating the Severity of Subacute Combined Degeneration Due to Vitamin B12 Deficiency.

Subacute combined degeneration (SCD) is a neurological complication of cobalamin deficiency, which is usually caused by chronic autoimmune atrophic gastritis. Serum pepsinogen 1 and the ratio of pepsinogen 1/pepsinogen 2 (PG1/2) can reflect the severity of gastric atrophy.Objective: This work aims to investigate whether decreased serum PG1 and PG1/2 ratio are helpful in diagnosing SCD and reflecting the severity of SCD.Methods: We retrospectively analyzed the clinical and laboratory tests of 65 cases of SCD due to vitamin B12 deficiency and compared the laboratory parameters of SCD with 65 age- and sex-matched amyotrophic lateral sclerosis (ALS) patients.Results: PG1 and PG1/2 ratio were decreased in 80 and 52.3% of SCD patients, respectively. Compared to patients with PG1/2 ratio ≥3.0, patients with PG1/2 ratio <3.0 had more severe anemia, larger mean corpuscular volume (MCV), lower level of vitamin B12, higher folate and homocysteine (Hcy), more severe changes in somatosensory evoked potential (SEP), and higher rate of lesions in spinal MRI (P < 0.05). PG1 and PG1/2 ratio had inverse correlation with MCV and N20 latency in SEP examination (P < 0.05). PG1/2 ratio, RBC count, and Hcy were independent risk factors for SCD in logistic regression analyses. The ROC curve analysis revealed that the diagnostic accuracy of PG1 and PG1/2 ratio was 72.2 and 73.0%, respectively, while the cutoff values were 22.4 ng/ml and 2.43 for SCD, respectively.Conclusions: Decreased PG1 and PG1/2 ratio are helpful for the diagnosis and evaluation of the severity of SCD due to vitamin B12 deficiency.

Nitrous Oxide Abuse‐Induced Subacute Combined Degeneration: Classic Neuroimaging Findings on MRI

Subacute combined degeneration is uncommonly a result of nitrous oxide abuse and presents with high signal in the dorsal columns of the spinal cord on T2-weighted MR imaging. We present a case of subacute combined degeneration in a young patient who abused nitrous oxide, which is an uncommon cause and infrequently seen in this patient population. Symptoms are often reversible with treatment of vitamin B12, and radiologists should be aware of these findings to avoid delay in treatment.

Subacute combined degeneration of the spinal cord following nitrous oxide anesthesia: A systematic review of cases

ObjectiveVitamin B12 deficiency can lead to subacute combined degeneration (SCD). Nitrous oxide (N2O) is an anesthetic which oxidizes the cobalt ion of vitamin B12, interfering with its function as a coenzyme. In this study, we conduct a systematic review of reported cases of SCD following nitrous oxide anesthesia. Patients and MethodsA comprehensive search of multiple databases was conducted, and information about patient characteristics, symptomatology, clinical work-up, and treatment was extracted from eligible articles. Univariate analyses were performed to identify predictors of poor neurological recovery following SCD. Results32 studies, reporting 39 cases of nitrous oxide-induced SCD, were included through the screening process. These cases included 22 male patients and 17 female patients, with an average age of 51.3 years (SD 17.6). An etiology for subclinical B12 deficiency was determined in 31 reports; of these, 26 were due to vitamin malabsorption secondary to a gastrointestinal disorder. Duration of nitrous oxide exposure was described in 19 reports, and ranged from 30 min to 11 h. Univariate analysis failed to find an association between post-operative recovery and age (p = 0.41), sex (p = 0.48), positive MRI findings (p = 0.42), post-operative serum B12 (p = 0.96), post-operative hemoglobin (p = 0.17), type of surgery (p = 0.58), or post-operative high mean corpuscular volume (p = 0.14). ConclusionIn patients with postsurgical myelopathy, surgeons should evaluate B12 status and consider the possibility that nitrous oxide could cause a subclinical B12 deficiency to become overt, particularly in patients with malabsorptive GI comorbidities. Treatment with B12 in this population can result in significant improvement of neurological function.

A case of subacute combined degeneration in duced by nitrous oxide

A case of subacute combined degeneration in duced by nitrous oxide

Clinical and imaging characteristics of subacute combined degeneration complicated with white matter lesions in the brain: a report of five cases

Purpose: To report five cases of subacute combined degeneration (SCD) with brain involvement and explore its clinical and imaging characteristics.Methods: A retrospective study was performed on the clinical data and brain MRI of five patients with subacute combined degeneration with brain involvement (out of 107 cases with SCD in total). White matter lesions (WML) assessment was performed qualitatively using Fazekas scale score.Results: The main symptoms in four patients were weakness in both lower extremities and unstable walking (limb weakness in three patients, dizziness in three patients, and blurred vision in one patient). One patient had memory loss and cognitive dysfunction. The MMSE scale indicated mild dementia in one patient. On head MRI (Magnetic Resonance Imaging), multifocal and symmetrical high signals of T2WI and FLAIR were observed in the frontal lobe and periventricular white matter in four patients, while another patient showed preferential atrophy in frontal regions. Fazekas scale scores ranged from 1–6.Conclusion: Adult subacute combined degeneration seldom involves the brain. Multifocal and symmetrical high signal white matter lesions can be found on FLAIR and T2WI, as well as frontal atrophy on head MRI.

Subacute combined degeneration of the spinal cord in a Nigerian child: a need for a high index of suspicion

Background: Vitamin B12 deficiency has been reported to be associated with a spectrum of neurological disorders among which is subacute combined degeneration of the spinal cord. Method: We report a case of subacute combined degeneration of the spinal cord secondary to vitamin B12 deficiency and discussed the clinical presentation and management challenge. Result: The diagnosis was made through a high index of suspicion when the clinical presentation ranked highly with the mode of presentation of Vitamin B12 deficiency. Patient responded well to parentheral vitamin B12 preparation but subsequently defaulted from follow up for 8 years after which he represented with paraparesis and urinary incontinence. He was managed again with parentheral vitamin B12 preparations with good outcome and total resolution of symptoms at discharge. Conclusion: A high index of suspicion is needed in identifying vitamin B12 deficiency related paraparesis in paediatric practice while the need for indefinite treatment and follow up is hereby emphasized. Keywords: Vitamin B12 deficiency; myelinopathy; paraparesis; subacute combined degeneration

Correlation of Clinical and MRI Features of Neuropsychiatric Manifestations in Sub-acute Combined Degeneration of Spinal Cord: Neurological Syndrome Associated with Vitamin B12 Deficiency

Vitamin B12 or cynacobalamin is an important water soluble vitamin which plays a key role in erythropoiesis, proper nervous system functioning and for the metabolism of carbohydrate, fat and protein. Patients with cynacobalamin deficiency may present with haematological, gastro-intestinal, oral, dermatological, psychiatric and neurological disturbances. We present a case of sub-acute combined degeneration (SACD) of spinal cord in a fortynine year old female presenting with one month history of progressive symptoms of lower limb paraesthesia, sensory deficit, psychotic symptoms and postural instability. This case report is unusual as it elucidates the characteristic triad, i.e., haematological, psychiatric and neurological symptoms in sub-acute combined degeneration of spinal cord (SACD) associated with B12 deficiency, correlation of its clinical manifestations, electrophysiological signs, laboratory investigations ( especially biomarkers of B12 status) and spinal magnetic resonance (MR) imaging in establishing the diagnosis, treatment outcomes, and potential therapeutic relevance of vitamin B12 replacement therapy in symptoms remission.

Myelopathy mimicking subacute combined degeneration in a Down syndrome patient with methotrexate treatment for B lymphoblastic leukemia: Report of an autopsy case

We report clinicopathological features of a 23-year-old woman with Down syndrome (DS) presenting with subacute myelopathy treated with chemotherapy, including intravenous and intrathecal administration of methotrexate (MTX), and with allogenic bone-marrow transplantation for B lymphoblastic leukemia. Autopsy revealed severe demyelinating vacuolar myelopathy in the posterior and lateral columns of the spinal cord, associated with macrophage infiltration, marked axonal loss and some swollen axons. Pathological changes of posterior and lateral columns were observed from the medulla oblongata to lumbar cord. Proximal anterior and posterior roots were preserved. Cerebral white matter was relatively well preserved. There were no vascular lesions or meningeal dissemination of leukemia. Longitudinal extension of cord lesions was extensive, unlike typical cases of subacute combined degeneration (SACD), but distribution of lesions and histological findings were similar to that of SACD. DS patients show heightened sensitivity to MTX because of their genetic background. Risk factors for toxic myelopathy of DS are discussed, including delayed clearance of MTX despite normal renal function, alterations in MTX polyglutamation and enhanced folic acid depletion due to gene dosage effects of chromosome 21. Alteration of folate metabolism and/or vitamin B12 levels through intravenous or intrathecal administration of MTX might exist, although vitamin B12 and other essential nutrients were managed using intravenous hyperalimentation. To the best of our knowledge, this is the first report of an autopsy case that shows myelopathy mimicking SACD in a DS patient accompanied by B lymphoblastic leukemia. The case suggests a pathophysiological mechanism of MTX-related myelopathy in DS patients with B lymphoblastic leukemia mimicking SACD.

Spastic Quadreparesis - An Unusual Presentation of Sub-acute Combined Degenaration of Spinal Cord

We report a case of a 45-year-old gentleman presenting with numbness of both legs and hands. He has anemia, spastic quadriplegia with exageraterd tendon jerks with lose of vibration and position sense. Investigations revealed combined deficiency anemia with low serum vitamin B12. His MRI of cervicel spine was normal. Endoscopically he had atrophic gastritis. Ultimately he was dignosed as a case of sub-acute combined degeneration of spinal cord due to pernicious anemia. Patient was treated with injection vitamin B12 and well responded. On his followup, he had significant clinical improvement. This is a reversible condition if early diagnosis can be made and vitamin B12 therapy promptly given. Delayed treatment can result in irreversible neurological impairment.DOI: http://dx.doi.org/10.3329/jom.v14i1.14585 J MEDICINE 2013; 14 : 85-87

A Systematic Review on the Use of Intrathecal Rituximab in CNS Lymphoma

Abstract 4987 Introduction:Central nervous system (CNS) involvement by non-Hodgkin lymphoma (NHL) is a rare but serious complication with a high mortality rate and few effective therapeutic options. Rituximab is an anti-CD20 monoclonal antibody that is administered systemically in combination with chemotherapy, but can also be given intrathecally. When administered intrathecally (IT), rituximab achieves more predictable and higher cerebrospinal fluid (CSF) concentrations compared to its intravenous administration, however the efficacy and safety of IT rituximab are unknown (Rubenstein et al., J Clin Oncol 2007). We recently treated a 52 year old woman for a CNS relapse of diffuse large B-cell lymphoma (DLBCL) using a combination of IT rituximab, cytarabine, methotrexate and hydrocortisone, as well as craniospinal radiation. After initial improvement, she developed subacute combined degeneration (SCD) of the spinal cord. This prompted the present systematic review of the literature in order to explore the efficacy and safety of administering rituximab directly into the CSF for the treatment of CNS involvement by NHL. Methods:We identified all relevant citations in MEDLINE/EMBASE/CINAHL/BIOSIS Previews from their date of inception to June 20th, 2011. Prospective studies, case reports and case series reporting on intrathecal or intraventricular (via ommaya-reservoir) use of rituximab in relapsed primary (PCNSL) or secondary CNS-lymphoma were included. All articles citing the included articles, as well as the reference lists of included articles were reviewed to ensure capture of all relevant studies. In some cases, authors were contacted to gather additional information. Two authors independently evaluated study eligibility. Disagreements were resolved by consensus and agreement was assessed using the kappa statistic. All non-human studies were excluded. Due to the nature of the studies, a meta-analysis was not performed. Results:The search strategy identified 148 potential reports published between 2002–2011 of which 22 were included (3 prospective trials, 2 phase-I trials, 2 case-series and 15 case reports). The kappa value for inter-author agreement was 0.881. A total of 74 patients, aged 4–84 years old (Mean age 48), with PCNSL (28%) or secondary CNS-Lymphoma (ALL 14%, DLBCL 8%, Burkitt’s 4%, Mantle Cell Lymphoma 5%, Post-transplant lymphoproliferative disorder 4%, Unknown histology 37%), received 1 to 22 doses of 10–50mg of intrathecal (43%) or intraventricular (IVT) rituximab, either alone or in combination with other therapies. At a mean follow-up of 43 weeks (Range 2–178), 34% of patients were in complete remission and 28% showed a partial response. Patients having both leptomeningeal and parenchymal involvement of CNS lymphoma were the most likely to have progression of disease with IT/IVT rituximab (44%), as compared to those with isolated disease in the leptomeninges (29%) or parenchyma (33%). Fifty percent of patients with isolated disease in the leptomeningeal compartment showed complete remission with IVT/IT rituximab therapy. Fatigue, nausea/vomiting and paresthesias were the most commonly reported side effects. In one series, seven patients developed arachnoiditis. There was a single case of Hepatitis B virus reactivation in a patient with parenchymal PCNSL. In addition, our patient developed symptoms and findings consistent with SCD and in whom a relation between SCD and rituximab could not be ruled out. Conclusion:In case series and reports, IT rituximab appears to be a safe and modestly effective therapy when used in conjunction with other salvage therapies for the management of refractory CNS-lymphoma. The overall proportion of complications appears to be reasonably low. To our knowledge, our patient is the first case of SCD reported in a patient who received IT rituximab. Further studies with a particular emphasis on safety are needed before widespread use can be recommended. Disclosures:Off Label Use: Systematic literature review on the use of intrathecal rituximab for CNS lymphoma (off-label use). Lazo-Langner:Leo Pharma: Honoraria; Pfizer Inc.: Honoraria.

MRI findings in subacute combined degeneration of the spinal cord in a patient with restricted diet

Subacute combined degeneration (SCD) is a rare cause of demyelination of the dorsal and lateral columns of the spinal cord, and is a neurogenic complication due to vitamin B12 deficiency, that we observe with unusual frequency. Improvement in myelopathy with normalization of the abnormalities in the MRI may occur if the replacement of the vitamin B12 is started early in the course of the disease. A rare case of SCD of the spinal cord due to vitamin B12 deficiency with a history of diet restrictions is presented.

Hyperintense signal on spinal cord diffusion-weighted imaging in a patient with subacute combined degeneration

Symmetrical hyperintense signals in lateral columns and posterior columns in T2-weighted images is a characteristic finding in subacute combined degeneration (SCD) secondary to vitamin B12 deficiency. Manifestation of SCD on diffusion-weighted imaging (DWI) has not been well characterised till date. We reported a case of SCD with spinal cord hyperintense signals in DWI sequence. We suggest that spinal cord DWI may be a useful magnetic resonance sequence for the diagnosis of SCD.

Subacute Combined Cord Degeneration: A Rare Complication of Nitrous Oxide Misuse

We describe a severe case of subacute combined degeneration of the spinal cord precipitated by overuse of the analgesic Entonox (a 50:50 mixture of nitrous oxide and oxygen) in a 19-year-old female with previously undiagnosed vitamin B12 deficiency. MRI of the spine revealed symmetric abnormal signal in the dorsal and anterolateral columns of the cervical and thoracic cord, correlating with the patient's symptoms. Myeloneuropathy is caused by inactivation of vitamin B12 by nitrous oxide. The patient's condition slowly improved with cessation of nitrous oxide exposure and vitamin B12 replacement therapy.

Subacute Combined Degeneration Mimicking Traumatic Spinal Cord Injury

Subacute combined degeneration (SCD) of the spinal cord is the most common neurologic manifestation of vitamin B12 (cobalamin) deficiency and is usually secondary to autoimmune gastritis, but may also be seen in malnutrition syndromes such as chronic alcoholism, strict vegetarianism, gastrectomy, and also in nitrous oxide abuse. Although traumatic spinal cord injury is routinely encountered in the medical examiner's office, medical causes of spinal cord abnormalities such as SCD should be considered in the appropriate clinical setting. We report a case of alcohol-associated SCD mimicking traumatic spinal cord injury.

Subacute combined degeneration of the spinal cord caused by nitrous oxide anaesthesia

Vitamin B12 deficiency causes haematological, gastrointestinal and neurological diseases. Subacute combined degeneration (SCD) of the spinal cord is characterised by degeneration of the posterior and lateral columns. We report a case of SCD associated with nitrous oxide anaesthesia.

Contribution of spinal MRI for unsuspected cobalamin deficiency in isolated sub-acute combined degeneration

Sub-acute combined degeneration (SCD) is a rare cause of demyelination of the dorsal and lateral columns of the spinal cord and is a neurological complication due to vitamin B12 deficiency. Revealing forms of SCD without anemia are rare. We report a case of SCD of the spinal cord in a 33-year-old woman without anemia but with a 10-month history of paresthesis and urine imperiosity. Magnetic resonance imaging (MRI) of the spine showed intramedullary hyperintensity seen on T2-weighted images in the posterior column of the cervico-dorsal spinal cord, extending from C1 to D1. A diagnosis of SCD of the spinal cord was considered and confirmed by a low serum cobalamin. The patient was treated with vitamin B12 supplements and showed gradual improvement in her clinical symptoms.