Subacute Combined Degeneration Research Articles

FT4-to-FT3 ratio is a novel prognostic marker in subacute combined spinal cord degeneration patients.

The FT4-to-FT3 ratio (FFR) variations in patients with subacute combined spinal cord degeneration (SCSD) as a potentially useful prognostic indicator are still unknown. This study aimed to investigate the changes of FFR as a potentially valuable prognostic predictor in patients with SCSD. This study included 144 consecutive SCSD patients who received standard diagnostic and therapeutic procedures between January 2015 and December 2021 and were admitted to the Department of Neurology at the First Affiliated Hospital of Bengbu Medical University. At the time of admission, we gathered data on all patients' demographics, daily routines, previous chronic conditions, medication histories, and other clinical details. For the purpose of measuring FFR, blood samples were specifically taken within 48 h of admission. The degree of neurological impairment of patients was assessed using the functional disability scale at the time of admission. At 6 months following discharge, the Modified Rankin Scale (mRS) was used to evaluate the clinical prognosis. To evaluate the relationship between the FFR and the risks of a poor outcome (mRS > 2), univariate and multivariate logistic regression analysis was utilized. The significance of the FT4/FT3 ratio in predicting the clinical outcomes in SCSD patients 6 months after discharge was assessed using the area under curve-receiver operating characteristic (AUC-ROC). About 90 patients (62.5%) of the 144 patients had poor outcomes, while 54 (37.5%) had favorable outcomes. Higher FFR at admission was independently linked to higher odds of a poor outcome, according to a logistic analysis. With an optimized cutoff value of >2.843, the FFR exhibited the maximum accuracy for predicting a poor outcome, according to the AUC‒ROC curve (AUC 0.731, P < 0.001; sensitivity, 77.8%; specificity, 83.3%). FFR was identified as an independent predictor of poor outcomes by multivariate logistic regression (OR, 2.244; 95% CI, 1.74-2.90; P < 0.001). We discovered that in patients who had a bad result 6 months after discharge, the FFR had dramatically increased at the time of admission, providing a unique prognostic marker in patients with SCSD.

New Methods of Treatment of Funicular Myelosis in Anemia with Vitamin V12 Deficiency Have Been Developed

Abstract: Funicular myelosis (Lichtheim syndrome) is a rare neurological disorder associated with vitamin B12 deficiency, often concurrent with pernicious anemia. This deficiency is typically due to malabsorption issues in the digestive tract, particularly prevalent among the elderly population. Vitamin B12 plays a crucial role in various metabolic processes, including DNA synthesis, hematopoiesis, and neurological function. Here, we present a case study of a 73-year-old patient with severe acquired B12 deficiency anemia, hemolytic jaundice, glossitis, and funicular myelosis. The patient exhibited pronounced motor and sensory deficits, which significantly improved following treatment with cyanocobalamin. We highlight the clinical features, diagnostic considerations, and therapeutic outcomes of this case, emphasizing the importance of early recognition and intervention in B12 deficiency-related neurological disorders.

The other side of funny gas (nitrous oxide – N2O), a case report of subacute combined degeneration of cord

The other side of funny gas (nitrous oxide – N2O), a case report of subacute combined degeneration of cord

Poūmės kombinuotos nugaros smegenų degeneracijos, sukeltos diazoto monoksido („linksminančių dujų”) vartojimo, klinikinio atvejo aprašymas

Although nitrous oxide (N2O) has long been used as an anesthetic, it’s use as a recreational drug has recently increased. As N2O abuse is associated with a number of adverse health effects, it has become a significant public health issue. Chronic abuse can lead to a number of neurological complications, most commonly related to functional vitamin B12 deficiency. One of the most encountered forms of N2O neurotoxicity is a subacute combined degeneration, which can cause permanent neurological damage. The medical community should therefore be aware of the increasing scale of recreational N2O exposure and the possible complications of this drug abuse. Early recognition and treatment of N2O toxicity are crucial. We present the case of an 18-year-old male who was diagnosed with subacute combined spinal cord degeneration related to N2O abuse.

Subacute combined degeneration of the spinal cord of toxic etiology (clinical case)

For more than two hundred years after its discovery, nitric oxide (I) has been widely used in medicine as an anesthetic, in the car industry as an engine performance enhancer, in the food industry as a preservative, and, unfortunately, among young people as a mild narcotic substance. Despite the widespread opinion that there is no harm from the use of “laughing gas” for recreational purposes, in the literature and in practice, there are a lot of cases when patients suffer from various complications, including neurological ones, caused precisely by the use of nitric oxide (I). One of the main mechanisms of the pathological effects of nitrous oxide on the nervous tissue is the inactivation of vitamin B12. This has been proven by studying the physicochemical properties of the gas, the effect on the organisms of laboratory animals, the change in the concentration of certain substances in the human body, and also by the use of cyanocobalamin for the treatment of these patients. This article describes a clinical case of the development of myelopolyneuropathy caused by the toxic effects of nitric oxide (I).

Nitrous oxide-induced subacute combined degeneration of the spinal cord

The differential diagnoses of a patient presenting with motor weakness is broad and requires a systematic approach at the Emergency Department when evaluating its underlying cause. While nitrous oxide neurotoxicity is uncommon, it is important to consider it as a possible differential in patients with preceding exposure, as treatment is readily available. We present a case report with magnetic resonance imaging images of subacute combined degeneration of the spinal cord due to nitrous oxide induced vitamin B12 deficiency.

Unhappy Balloon, Nitrous Oxide-induced Subacute Combined Degeneration of the Spinal Cord: A Review of Case Series

Background: The younger generation in Korea easily exposed to nitrous oxide (N&lt;sub&gt;2&lt;/sub&gt;O) under the name “Happy Balloon” may abuse it. N&lt;sub&gt;2&lt;/sub&gt;O can irreversibly oxidize vitamin B&lt;sub&gt;12&lt;/sub&gt; and cause abnormal hematopoiesis or nervous system toxicity such as subacute combined degeneration (SCD). The objective of this study was to assist in early diagnosis of N&lt;sub&gt;2&lt;/sub&gt;O-induced SCD of spinal cord by characterizing its clinical manifestations.Methods: Four patients with myelopathy after abusing N&lt;sub&gt;2&lt;/sub&gt;O were enrolled. To characterize N&lt;sub&gt;2&lt;/sub&gt;O-induced myelopathy, previously reported cases of N&lt;sub&gt;2&lt;/sub&gt;O-induced SCD were searched through PubMed and KoreaMed. Collected cases and our four patients were analyzed.Results: A total of 30 patients with N&lt;sub&gt;2&lt;/sub&gt;O-induced myelopathy (26 males and 4 females with mean age of 24 years) were analyzed. These patients took a median dose of 650 canisters for a median duration of 3.5 months. All patients presented sensory disturbances, which involved the lower extremities more frequently (100%) than the upper extremities (63.3%). Gait ataxia (76.7%), weakness in the upper (23.3%) and lower (36.7%) extremities, bladder symptoms (26.7%), Rhomberg sign (43.3%), and Lhermitte’s phenomenon (10.0%) were observed. Serum vitamin B12 levels were decreased in many (63.3%) patients and homocysteine levels were elevated in all. Of 20 patients who underwent magnetic resonance imaging (MRI) of the spine, 19 had abnormal findings. Three patients presented with contrast enhancement in lesions.Conclusions: We strongly recommend that history of N&lt;sub&gt;2&lt;/sub&gt;O abuse should be asked for young patients with suspected myelopathy, especially those presenting with gait ataxia and sensory disturbances suggesting posterior column dysfunction and those presenting long-segment lesion involving the upper cervical cord on MRI.

Co-occurrence of subacute combined degeneration of the spinal cord and neuromyelitis optica spectrum disorder

Co-occurrence of subacute combined degeneration of the spinal cord and neuromyelitis optica spectrum disorder

Subacute combined degeneration: When neuroimaging complements the clinic—A case report

Introduction: Low serum B vitamin levels are potentially causally related to these prevalent mental symptoms. Because of this, cobalamin—vitamin B12—is crucial for producing myelin and neurotransmitters. Additionally, subacute combined degeneration (SCD) is one of the consequences of B12 deficiency. Symptomatology often is paraplegia, spasticity, and contractures which are typically progressive. In addition, as the disease advances, they experience a distal sensorimotor loss that ascends proximally and causes spastic quadriparesis and generalized hypoesthesia. Case Report: Male, 33 years old, with a history of a fall in December 2015, when pain and discomfort started in the left lower limb. A year ago, she evolved with paraparesis, loss of sphincter control, sensory changes in the extremities of the limbs and sensory level at T10, in addition to a report of visual disturbance in the left eye. At that time, the dosage of vitamin B12 was very low—50 pg/mL (reference above 300 pg/mL). On physical examination: extrapyramidal syndrome with hypoesthesia at the distal level. Bilateral Hoffmann’s sign, spastic paraparesis, hyperreflexia in the lower limbs, clonus in the feet, and loss of sphincter control. Sensitive level at T10. Magnetic resonance imaging (MRI) showed cervical dorsal lumbar spinal cord and cone with hypersignal focus on the lateral and posterior cords and skull hypersignal focus in the topography of the corticospinal tracts, suggestive of combined subacute degeneration. Conclusion: Vitamin B12 deficiency, in addition to other pathologies, is also found in SCD. When treated early, these alterations found in the imaging exam can be resolved without signs of atrophy. Thus, neuroimaging plays a relevant role in the diagnosis, complementing the clinic.

Progressive Lower Extremity Paralysis Caused by Intrathecal MTX-Induced Myelopathy Mimicking Guillain-Barre Syndrome: A Case Report.

Methotrexate (MTX) is commonly used in intrathecal chemotherapy for patients with acute lymphocytic leukemia (ALL) to prevent central nervous system (CNS) involvement. However, the use of MTX-based chemotherapy can lead to rare yet severe complications, such as MTX-induced myelopathy. Here, we report the case of MTX-induced myelopathy initially misdiagnosed as Guillain-Barre syndrome, leading to a delay in diagnosis and treatment. We present a case of a 39-year-old male with a history of B-cell acute lymphoblastic leukemia (B-ALL) who experienced bilateral foot paresthesia and progressive lower extremity weakness after intrathecal methotrexate (MTX) treatment. Initially, the patient was suspected as having Guillain-Barre syndrome (GBS) due to similar clinical features and nerve conduction studies. The patient received intravenous immunoglobulin (IVIG) treatment, but his condition worsened. T2-weighted images of the thoracic spinal cord revealed high signal intensity in both lateral and posterior columns, typically associated with subacute combined degeneration. However, elevated vitamin B12 levels ruled out SCD in this case. Based on the aforementioned findings, intrathecal methotrexate-induced myelopathy was diagnosed. This case highlights the diagnostic challenge posed by the similarity in clinical presentation between MTX-induced myelopathy and GBS. Differentiating between these conditions is critical for appropriate management. Prompt recognition and treatment with folate metabolism compounds may mitigate neurological sequelae.

Nitrous-oxide-induced polyneuropathy and subacute combined degeneration of the spine: clinical and diagnostic characteristics in 70 patients, with focus on electrodiagnostic studies.

Nitrous oxide (N2 O) induced neurological symptoms are increasingly encountered. Our aim is to provide clinical and diagnostic characteristics with a focus on electrodiagnostic studies. Patients with neurological sequelae due to N2 O presenting in our hospital between November 2018 and December 2021 reporting clinical and diagnostic data were retrospectively reviewed. Seventy patients (median 22 years) were included. Median N2 O usage was 4 kg/week during 12 months. Patients' history revealed a higher rate of sensory symptoms compared to motor (97% vs. 57%) and 77% walking difficulties. Clinical diagnosis was polyneuropathy (PNP) in 44%, subacute combined degeneration (SCD) of the spine in 19%, both in 37%. Median vitamin B12 level was low (159 pmol/L), normal in 16%. The median methylmalonic acid was increased (2.66 μmol/L). Electrodiagnostic abnormalities were observed in 91%, with 72% fulfilling axonal PNP criteria, 20% showing mild to intermediate slowing. One patient fulfilled demyelinating PNP criteria not related to N2 O abuse (Charcot-Marie-Tooth type 1a). More prominent motor nerve conduction abnormalities were found; lower limbs were more affected. In 64% with normal conduction, myography showed signs of axonal loss. Magnetic resonance imaging showed cervical myelopathy in 58% involving generally five to six segments. Nitrous oxide (N2 O) leads to neurological symptoms by causing PNP and/or SCD primarily involving the legs. Distinguishing PNP and SCD clinically was shown to be insufficient. Electrodiagnostic studies showed axonal PNP. Demyelinating PNP due to N2 O abuse was not present in our cohort. Therefore, further diagnostic work-up is warranted if demyelinating features are present.

Nitrous oxide induces subacute combined degeneration by affecting vitamin B12 metabolism

Nitrous oxide induces subacute combined degeneration by affecting vitamin B12 metabolism

Clinical profile of longitudinally extensive transverse myelitis: A study in a tertiary care hospital in Western Rajasthan

As there are wide differentials for longitudinally extensive transverse myelitis (LETM), its aetiology should be found to optimise therapy.Clinical profile of LETM.ambispective observational study.73 patients with acute to subacute myelitis involving 3 or more vertebral segments were properly investigated.Student t test and Mann-whitney test used, and comparison of data done using SPSS version 22.0The etiology found were in form of neuromyelitis optica spectrum disorder (NMO-SD) in 18(24.6%), para-infectious in 13(17.8%), idiopathic in 17(23.2%), infectious in 9(12.3%), and rest were acute disseminated encephalomyelitis (ADEM), subacute combined degeneration (SACD), infarct, radiation myelitis etc.It is important to find etiology of LETM to optimise the therapy as the in inflammatory myelitis other than NMOSD, the line of management differs. Relapsing nature of some myelitides is an important aspect in management

Early treatment of subacute combined degeneration of the spinal cord in pernicious anemia will improve the outcome

Vitamin B12 deficiency is probably more common than what is reported in the literature. B12 is an essential vitamin, and its deficiency can lead to neuropsychiatric manifestations such as peripheral neuropathy, myelopathy, cerebellar ataxia, dementia, delirium, even psychosis and mood disorders. We report a case of 45 year old polish male presented with symptoms and signs suggestive of SCD of the spinal cord, due to severe vitamin B12 deficiency as a result of pernicious anemia. Cervical spine MRI showed enhancement in the bilateral posterior columns of the cervical spinal cord with a classic inverted V appearance. Metabolic workup revealed severe vitamin B12 deficiency, complete blood analysis indicated megaloblastic anemia. Intramuscular injection of cobalamin was started on a timely manner with complete resolution of symptoms.

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Bilateral sixth nerve palsy with subdural hematoma: a unique presentation of B12 deficiency.

Vitamin B12 is inextricably associated with the development and maintenance of neuronal functions. It is classically associated with subacute combined degeneration and peripheral neuropathy; however, cranial neuropathy is uncommon. We observed the rarest neurological manifestation of B12 deficiency. A 12 months infant had history of lethargy, irritability, anorexia, paleness, vomiting, and neurodevelopmental delay for 2 months. He also developed inattention and altered sleep pattern. His mother noticed bilateral inward rotation of both eyes. On examination, the infant had bilateral lateral rectus palsy. The infant was found to have anemia (7.7g/dL) and severe B12 deficiency (74pg/mL). On MRI, there was cerebral atrophy, subdural hematoma (SDH) and wide cisternal spaces and sulci. On supplementation with cobalamin, he improved clinically though mild restriction of lateral gaze on the left side persists. Follow up MRI showed significant improvement in cerebral atrophy with resolution of SDH. To date, such clinical presentation of B12 deficiency has never been reported. The authors suggest B12 supplementation for at risk population esp at antenatal stage and lactating mothers in national programs. The treatment of this condition should be initiated early to prevent long term sequelae.

P83 Central neuraxial blockade in an obstetric patient with subacute combined degeneration of the spinal cord

P83 Central neuraxial blockade in an obstetric patient with subacute combined degeneration of the spinal cord

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.

A Comparison Between Somatosensory Evoked Potentials and Spine MRI in the Diagnosis of Non-compressive Myelopathy: Which Is More Accurate?

Non-compressive myelopathy is a neurological disorder due to pathological processes affecting the spinal cord in the absence of clinical and radiological evidence of spinal cord compression. Two commonly used diagnostic tools for non-compressive myelopathy are somatosensory evoked potentials (SSEPs) and magnetic resonance imaging (MRI). SSEPs are a neurophysiological tool used to assess the functional integrity of the spinal cord. MRI, on the other hand, is the mainstay imaging modality used for identifying compressive lesions and other structural abnormalities in the spinal cord.The aim of this study was to test the diagnostic accuracy of SSEPs versus spine MRI in the diagnosis and assessment of the severity of non-compressive myelopathy using the Modified Japanese Orthopaedic Association (mJOA) clinical severity score. Our study included 63 subjects. Whole spine MRI and SSEPs (median and tibial SSEP bilaterally) were done for all subjects; their results were compared according to their relation to the mJOAscore and classified into mild, moderate, and severe. The control group was examined to establish normative data for SSEPresults and compared with cases. Blood investigations such as complete blood count, thyroid function test, A1C, HIVtests, venereal disease research laboratory test, erythrocyte sedimentation rate, C-reactive protein, and antinuclear antibodytests were done. Blood tests for vitamin B12 levels were done for patients who were suspected of sub-acute combined degeneration of the spinal cord; cerebrospinal fluid (CSF) analysis was done for patients suspected of multiple sclerosis (MS), acute transverse myelitis (ATM), or other inflammatory/infectious diseases. CSF was analyzed for cell count, cytology, protein, and oligoclonal bands (if indicated). No mild categories were registered in this study; 30%of patients were moderateand 70%were severe. Causes for non-compressive myelopathy in this study werehereditary degenerative ataxias in 12 (38.71%), ATM in 8 (25.81%), and MS in 5 (16.13%); other causes included vitamin B12 deficiency in 2 (6.45%), ischemia in 2 (6.45%), and an unknown cause in 2 (6.45%). SSEPs showed abnormal results in all patients (31; 100%) whereas MRI showed abnormality in only seven patients (22.6%). SSEPsensitivity for detecting severe cases was around 63.6% while that for MRI was 27.3%. The study concluded that SSEPs were more reliable for the detection of non-compressive myelopathies rather than MRI and correlated better with clinical severity. Performing SSEPs is recommended for all patients with non-compressive myelopathy, especially those with negative imaging.

Nitrous Oxide-Induced Subacute Combined Degeneration in a 38-Year-Old Pregnant Female After Recreational Use

Nitrous oxide (N2O) misuse creates a diagnostic dilemma due to its clinical presentation, difficulty in identification, and toxicity related to its chronic abuse, with resultant morbidity and mortality. Chronic abuse can lead to myeloneuropathy and subacute combined degeneration in otherwise healthy individuals. Health professionals should be aware of the commercial availability and abuse of N2O by the public, and N2O toxicity should be included in the differential diagnosis in patients presenting with myelopathy of unknown etiology. A case report was conducted on a 38-year-old female at approximately 30 weeks of gestation who presented to the emergency department with worsening bilateral lower extremity numbness, tingling, and weakness. The patient admitted to nitrous oxide inhalation during the two months prior to admission. She reported using four cans of whippets per week (approximately 8 g of N2O per whippet) up to 50 cans per day (400 g N2O) prior to the onset of symptoms. An MRI of the cervical spine was performed, showing T2 hyperintensity from C2 to C6 involving dorsal columns indicative of subacute combined degeneration. The patient was treated with intravenous vitamin B12 due to the clinical and radiographic evidence of nitrous oxide-induced myelopathy. The pathophysiology of N2O toxicity involves the oxidation of the cobalt atom of cobalamin (vitamin B12) from its reduced active 1+ valent state to its oxidized inactive 3+ valent state. This oxidation inactivates the enzyme methionine synthetase. B12 is an essential cofactor for downstream DNA synthesis. Consequently, excess N2O creates functional B12 deficiency leading to irreversible nerve damage if left undiagnosed and untreated.