Severe but reversible neuropathy and encephalopathy due to vitamin E deficiency

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Clinical and functional change in multifocal motor neuropathy treated with IVIg

We determined the clinical progression, disability and outcome of 11 Multifocal Motor Neuropathy (MMN) patients from Malaysia. Mean patient age was 46.8 (SD 13.3), with mean disease duration of 108.0 months (SD 80.2). All reported unilateral limb weakness at onset. At diagnosis, after mean 49.9 months (SD 73.5) delay, 7 (63.6%) had more than 2 limbs involvement. Nine (90%) of 10 patients received induction IVIg dose of 2.0 gm/kg responded, demonstrated improvement in MRCSS of > 2 points or mRS score of > 1 point. We observed 38.5% drop in IVIg dose to mean 1.12 gm/kg/month after 12 months of treatment, and a further 34.8% drop upon 24th month treatment to mean dose of 0.73 gm/kg/month. This was in parallel with initial improvement in MRCSS and mRS, observed among 88.9% and 77.8% of the patients, and later further improvement (33.3%) or stabilization (66.7%) of mRS score toward 2nd year. During the same period, 50% of patients reported deterioration in ONLS, 33.3% in grip strength and 16.7% in MMN-RODS. Beyond 36th month, average annual IVIg dose increased at 0.12 gm/kg/year (SD 0.09) or 11.2%, up to the 84th months. Despite that, progressive deterioration was observed in term of number of limbs involvement, definite motor conduction blocks on electrophysiology study, and both clinical as well as functional scores. Although IVIg dose reduction for maintenance treatment in MMN is recommended, careful clinical assessment is required to prevent under-treatment. Use of reliable and responsive modern outcome measures is important to quantify clinically relevant change to guide therapy

Malignancy in Guillain-Barré syndrome:A twelve-year single-center study

The relationship between Guillain-Barré syndrome (GBS) and malignancy is uncertain. We retrospectively analyzed data of 118 consecutive patients admitted with GBS from Birmingham, U.K. (2001 − 2012). We calculated relative cancer risk using different definitions and determined characteristics of malignancy-associated GBS. Malignancy was globally commoner in our GBS cohort compared to the general population (odds ratio: 2.08; CI: 1.06–3.71; p = 0.036). However, this was unconfirmed if paraneoplastic criteria were applied. GBS patients with cancer were significantly more likely to be older (p = 0.043), hyponatremic (p = 0.037) and demonstrate more axonal loss (p < 0.05). Cerebrospinal fluid (CSF) protein levels were lower in the malignancy group (p = 0.002) and neurological improvement less likely (p = 0.023). In-patient mortality was significantly higher in patients with malignancy (p < 0.01). We conclude global cancer risk is higher in GBS than in the general population, although definition-dependent. Malignancy requires consideration in elderly, hyponatremic subjects with normal CSF protein, severe axonal loss, who fail to improve post-treatment

Practical needs and considerations for refugees and other forcibly displaced persons with neurological disorders: Recommendations using a modified Delphi approach

Background: There are \u3e70 million forcibly displaced people worldwide, including refugees, internally displaced persons, and asylum seekers. While the health needs of forcibly displaced people have been characterized in the literature, more still needs to be done globally to translate this knowledge into effective policies and actions, particularly in neurology. Methods: In 2020, a global network of published experts on neurological disease and refugees was convened. Nine physician experts from nine countries (2 low, 1 lower-middle income, 5 upper-middle, 1 high income) with experience treating displaced people originating from 18 countries participated in three survey and two discussion rounds in accordance with the Delphi method. Results: A consensus list of priority interventions for treating neurological conditions in displaced people was created, agnostic to cost considerations, with the ten highest ranking tests or treatments ranked as: computerized tomography scans, magnetic resonance imaging scans, levetiracetam, acetylsalicylic acid, carbamazepine, paracetamol, sodium valproate, basic blood tests, steroids and anti-tuberculous medication. The most important contextual considerations (100% consensus) were all economic and political, including the economic status of the displaced person\u27s country of origin, the host country, and the stage in the asylum seeking process. The annual cost to purchase the ten priority neurological interventions for the entire displaced population was estimated to be 220 million USD for medications and 4.2 billion USD for imaging and tests. Conclusions: A need for neuroimaging and anti-seizure medications for forcibly displaced people was emphasized. These recommendations could guide future research and investment in neurological care for forcibly displaced people

Clinical and Laboratory Features in Anti-NF155 Autoimmune Nodopathy

BACKGROUND AND OBJECTIVES: To study the clinical and laboratory features of antineurofascin-155 (NF155)-positive autoimmune nodopathy (AN). METHODS: Patients with anti-NF155 antibodies detected on routine immunologic testing were included. Clinical characteristics, treatment response, and functional scales (modified Rankin Scale [mRS] and Inflammatory Rasch-built Overall Disability Scale [I-RODS]) were retrospectively collected at baseline and at the follow-up. Autoantibody and neurofilament light (NfL) chain levels were analyzed at baseline and at the follow-up. RESULTS: Forty NF155+ patients with AN were included. Mean age at onset was 42.4 years. Patients presented with a progressive (75%), sensory motor (87.5%), and symmetric distal-predominant weakness in upper (97.2%) and lower extremities (94.5%), with tremor and ataxia (75%). Patients received a median of 3 (2-4) different treatments in 46 months of median follow-up. Response to IV immunoglobulin (86.8%) or steroids (72.2%) was poor in most patients, whereas 77.3% responded to rituximab. HLA-DRB1*15 was detected in 91.3% of patients. IgG4 anti-NF155 antibodies were predominant in all patients; anti-NF155 titers correlated with mRS within the same patient (r = 0.41, p = 0.004). Serum NfL (sNfL) levels were higher in anti-NF155+ AN than in healthy controls (36.47 vs 7.56 pg/mL, p < 0.001) and correlated with anti-NF155 titers (r = 0.43, p = 0.001), with I-RODS at baseline (r = -0.88, p < 0.001) and with maximum I-RODS achieved (r = -0.58, p = 0.01). Anti-NF155 titers and sNfL levels decreased in all rituximab-treated patients. DISCUSSION: Anti-NF155 AN presents a distinct clinical profile and good response to rituximab. Autoantibody titers and sNfL are useful to monitor disease status in these patients. The use of untagged-NF155 plasmids minimizes the detection of false anti-NF155+ cases. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that anti-NF155 antibodies associate with a specific phenotype and response to rituximab

Anti-NMDAR encephalitis in association with herpes simplex virus and viral and bacterial zoonoses

Multiple co-infections can predispose a patient to autoimmune encephalitis. Out of thirty cases of N-methyl-D-aspartate receptor (NMDAR) encephalitis seen at a single tertiary referral center, only two cases of co-infection with NMDAR encephalitis were identified. One of these cases was highly interesting due to the presence of more than one co-infections along with the presence of cortical dysfunction, seizures, and orofacial dyskinesias at the onset in a male in the absence of tumors, which was refractory to initial treatment

Clinical efficacy and safety of single cycle rituximab as induction therapy for aggressive neuromyelitis optica spectrum disorder in a resource limited center: a preliminary study

Aim: To analyse the efficacy of single dose rituximab (RTX) as induction therapy followed by conventional oral steroid-sparing agents (azathioprine, mycophenolate mofetil or methotrexate) in a cohort of patients with aggressive neuromyelitis optica spectrum disorder (NMOSD) without CD19, 20 and 27 biomarker testing.Methods: A retrospective analysis of clinical outcomes in eight patients with aggressive NMOSD treated with one course of RTX induction therapy in the Neurology Department at Kuala Lumpur Hospital from 2005 to 2018 was performed. The effectiveness of the treatment was determined by the number of relapses, expanded disability status scale, annualized relapsed rates, and modified Rankin Scale both before and after treatment. B cell enumeration testing was done instead of CD19, 20 and 27 biomarker testing.Results: There was a reduction in the mean annualized relapse rate from 4.7 to 0.5 attacks per year after treatment (P = 0.011). Mean expanded disability status scale and modified Rankin Scale values improved from 5.4 to 3.6 (P = 0.018) and 3.6 to 2.6 (P = 0.023), respectively. No patient developed any adverse effect.Conclusion: Single-course RTX induction therapy regime may be an alternative therapeutic option in resource limited hospitals to suppress NMOSD disease activity in the short term as pulse induction therapy whilst awaiting the effectiveness of conventional steroid-sparing agents. Further prospectively designed studies are required to prove efficacy

Optic Nerve Demyelination in IgG4 Anti-Neurofascin 155 Antibody-Positive Combined Central and Peripheral Demyelination Syndrome

Optic nerve demyelination is one of the clinical features of combined central and peripheral demyelination (CCPD), an entity with heterogenous immunopathogenesis and clinical characteristics, overlapping between multiple sclerosis (MS) and chronic inflammatory demyelinating polyneuropathy (CIDP). Of interest, earlier studies among patients with CIDP prior to discovery of antibodies against paranodal protein neurofascin 155 (anti-NF 155) also reported optic nerve dysfunction. We aimed to evaluate optic nerve demyelination among anti-NF 155 CIDP patients. We studied 2 patients with anti-NF 155 CIDP using visual-evoked potentials (VEP) and optical coherence tomography (OCT). Both patients had distal acquired demyelinating symmetric (DADS) subtype CIDP. Other common features were prominent sensory ataxia, hand tremors, significantly elevated cerebral spinal fluid protein, high titre anti-NF 155 antibodies and poor response to corticosteroid and intravenous immunoglobulin (IVIg). No central nervous system neuroradiological abnormality detected. Both had normal visual acuity and colour vision, but one had subclinical right relative afferent pupillary defect (RAPD). VEP of both showed bilateral prolonged P100 latencies. OCT for patient with RAPD demonstrated moderate to severe retinal nerve fibre layer (RNFL) thinning. Identification of optic nerve demyelination among subclinical CIDP with anti-NF 155 antibodies expanded the spectrum of demyelination within the subset of CCPD