Acute radiculomyelitis after antitetanus vaccination

Active or passive immunisation with vaccines or sera can cause lesions of immunomediated pathogenesis involving both the central (CNS) and the peripheral nervous system (PNS). Although very rare, the neurological complications described during antitetanus vaccinations almost exclusively affect the PNS, those affecting the CNS being even more rare. The authors report a case of transverse myelitis with a radicular component, which arose acutely following the administration of tetanus toxoid and had a partially favourable course.

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Sommario L'immunizzazione attiva o passiva con vaccini o sieri può provocare lesioni sia a carico del sistema nervoso centrale (N.S.C.) che periferico (S.N.P.), con patogenesi immunomediata. Anche se molto rare, le complicanze neurologiche descritte in corso di vaccinazione antitetanica si riferiscono quasi esclusivamente al S.N.P. Ancor più rare risultano quelle a carico del S.N.C. Gli Autori riportano il caso di una mielite trasversa con componente radicolare, insorta acutamente dopo la somministrazione di anatossina tetanica, a decorso parzialmente favorevole.

     

Neurosarcoidosis: A review of the rarer manifestations

Neurosarcoidosis characteristically presents with the onset of cranial nerve palsies or endocrine and electrolyte disturbances in a patient with known systemic sarcoid. However, the disease may occasionally manifest itself in patients with no evidence of somatic involvement and with a variety of neurological signs and symptoms. We present two cases that demonstrate several of the rare manifestations of the illness and review the literature on the diagnosis and treatment.     

A case of Sjögren’s syndrome with acute transverse myelitis and polyneuropathy in a patient free of sicca symptoms

Acute transverse myelitis (ATM) is an inflammatory process involving a restricted area of the spinal cord. The usually dramatic presentation with rapidly progressive symptoms involving motor, sensory, and autonomic functions makes ATM a medical emergency. ATM has been cited as a rare and unusual complication of Sjögrens syndrome, but early diagnosis and aggressive treatment might improve the prognosis. I report a case of Sjögrens syndrome with ATM and polyneuropathy in a patient free of sicca symptoms and having skin lesions and synovitis as the initial manifestations.     

Atopic myelitis: a clinical, biological, radiological and histopathological diagnosis

We describe a young patient with an unusual intramedullary lesion filled with eosinophils. The 21-year-old man developed chronic myelitis without optic neuritis or signs of systemic or infectious disease. A spinal biopsy was conducted because of the progressive extension and pseudo-tumoural aspect of the lesion. Histopathological analysis of the biopsy specimen revealed a severe inflammatory process with macrophages and numerous eosinophils. The eosinophil count in the blood and cerebrospinal fluid (CSF) were normal. Clinical, laboratory and radiological data did not correspond with the usual causes of eosinophilic myelitis. Abnormal mite antigen-specific IgE levels and features similar to Japanese cases of atopic myelitis suggested an allergic origin. Despite normal total IgE levels, this case may be the second case of atopic myelitis reported in a Caucasian patient. Striking differences with the first reported case are the absence of a typical history of atopy and normal total IgE levels. This case highlights that atopic myelitis should be considered in myelopathy occurring in Caucasian patients even in the absence of hyperIgEaemia.     

MRI characteristics of MOG-Ab associated disease in adults: An update

Our knowledge of the radiological spectrum of myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) is growing rapidly. An update on the radiological features of the disease, and its evolution is thus necessary. Magnetic resonance imaging (MRI) has an increasingly important role in the differential diagnosis of MOGAD particularly from aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD), and multiple sclerosis (MS). Differentiating these conditions is of prime importance because the management is different between the three inflammatory diseases, and thus could prevent further attack-related disability. Therefore, identifying the MRI features suggestive of MOGAD has diagnostic and prognostic implications. We herein review optic nerve, spinal cord and the brain MRI findings from MOGAD adult patients, and compare them to AQP4-NMOSD and MS.     

Diagnostic value of bright spotty lesions on MRI after a first episode of acute myelopathy

Background and purposeTo determine the diagnostic value of bright spotty lesions (BSLs) for aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (NMOSD^AQP4+), the predictive value of axial-BSLs for AQP4-IgG seropositivity, and the radio-clinical differences in NMOSD^AQP4+ patients with and without axial-BSLs.Materials and methodsRetrospective study that included patients aged ≥ 16 years, with a first acute spinal cord syndrome between 2005 and 2018 and abnormal spinal cord MRI with axial and sagittal T2 sequences. Patients with MRI findings consistent with compressive myelopathy were excluded. All spinal cord MRI were retrospectively evaluated for the presence of BSLs by 2 radiologists blinded to the diagnosis of acute myelopathy.ResultsA total of 82 patients were included; 15 aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder patients (NMOSD^AQP4+), and 67 other patients, considered as the other causes of myelopathy (OM) group. The specificity of axial-BSLs for NMOSD^AQP4+ patients was 94.0% (95% CI [85.6 to 97.7]). The sensitivity was 40.0% (95% CI [19.8 to 64.3]). In the multivariable analysis, the only MRI characteristic associated with AQP4-IgG positivity was the presence of axial-BSLs (OR: 9.2, 95% CI [1.2 to 72.9]; P = 0.022). In NMOSD^AQP4+ patients, the median of cord expansion ratio was higher with axial-BSL (1.2, IQR [1.1–1.3]) than without axial-BSL (1.1, IQR [1.0–1.2]; P = 0.046).ConclusionAfter a first acute spinal cord syndrome, the presence of axial-BSLs on spinal cord MRI seems very specific for NMOSD^AQP4+ and seems to be a predictor radiological marker of AQP4-IgG positivity.     

Central nervous system and muscle involvement in dengue patients: A study from a tertiary care center

BackgroundNeurological involvement in dengue virus (DENV) infection is being increasingly reported. There is paucity of studies evaluating the relative frequency of central nervous system (CNS) and muscle involvement in dengue.ObjectivesTo evaluate the frequency and prognosis of neurological and muscle involvement in dengue, and correlate these with dengue subtypes.Study designConsecutive dengue patients were included, and their clinical features, laboratory investigations and cerebrospinal fluid (CSF) findings were recorded. Cranial MRI was done in unconscious patients and electromyography and nerve conduction study in patients with flaccid weakness. Patients were categorized into encephalopathy, encephalitis, immune mediated and dengue associated muscle dysfunction (DAMD). Outcome at 1 month and its predictors were evaluated.Results116 patients aged 5–70 years were included; 82 had dengue fever (DF), 18 had dengue hemorrhagic fever (DHF), and 16 had dengue shock syndrome (DSS). Neurological manifestations were present in 92 (79%); encephalopathy in 17 (15%), encephalitis in 22 (19%), transverse myelitis in 1 (1%) and DAMD in 52 (45%) patients. Central nervous system (CNS) involvement was commoner in DHF/DSS compared to DF (44% vs 26%). 10 patients with CNS involvement died versus 1 with DAMD. The patients in the CNS group had more frequent hypotension, renal dysfunction and respiratory failure compared to the DAMD group, and had worse outcome. DENV2 and DENV3 were the commonest serotypes, but serotypes did not differ between CNS and DAMD groups.ConclusionsDAMD is commoner than CNS involvement in dengue. CNS involvement however, is associated with more serious illness and predicts poorer outcome.     

Acute ascending necrotizing myelitis in Okinawa caused by herpes simplex virus type 2

Summary A case of rapidly progressing ascending myelitis was necropsied. Necrosis was present throughout the whole length of the spinal cord and involved both the grey and white matter randomly. The perivascular lymphocytic infiltration in the spinal cord in the present case was more pronounced than that in the previously reported two cases of necrotizing myelopathy associated with malignancy. Using immunoperoxidase staining the presence of herpes simplex virus type 2 (HSV 2) antigen was demonstrated. Electron microscopic examinations revealed large numbers of HSV particles in the spinal cord. HSV 2 may be a common aetiological agent of necrotizing myelopathy and myelitis in Okinawa, an HSV 2 endemic area. In the present case, the necrosis was mainly found in the spinal cord but was also observed, to a very limited extent, in the brain.