We describe a rare case of post-infective Acute Motor Axonal Neuropathy (AMAN) variant of Guillain-Barrè Syndrome (GBS) associated with myelitis and anti-GD1b positivity after SARS-CoV-2 infection. The patient referred to the hospital reporting a history of ten days lasting moderate fever, myalgia and anosmia, with the onset of progressive quadriparesis and ascending paraesthesias in the four limbs since five days from defervescence. A chest computed tomography demonstrated interstitial pneumonia with "ground glass opacities", suggesting Coronavirus disease (COVID-19). The patient exhibited three negative reverse-transcription polymerase chain reaction (RT-PCR) nasopharyngeal swabs, while SARS-CoV-2 IgG was found in plasma. The electrophysiological examination demonstrated an AMAN and the spinal cord Magnetic Resonance Imaging (MRI) showed a T2-weighted hyperintense lesion in the posterior part of the spinal cord at the C7-D1 levels. Furthermore, anti-GD1b IgM was detected. GBS and myelitis could exceptionally develop simultaneously. Our findings reasonably support a causality link between COVID-19 and the neurological symptoms, suggesting a post-infective autoimmune reaction.
We examined MRI of two patients with progressive multifocal leukoencephalopathy (PML), including diffusion-weighted imaging (DWI), with calculation of apparent diffusion coefficients (ADC). The pathology findings of one patient were compared with those of MRI. The lesions had different ADC and DWI appearances, depending on the stage of the disease. Newer lesions and the advancing edge of large lesions had normal-to-low ADC and gave high signal on DWI. Older lesions and the centre of large lesions had increased ADC and gave low signal. High signal on DWI and low ADC mark the regions of active infection and cell swelling, distinguishing them from areas of reparative gliosis.
Table 1 Patientcharacteristics Onset of neurological syndrome Neurological signs and symptoms CSF findings* Antiganglioside antibodies MRI results Treatment and outcomes 10 days after fever, cough, sore throat and anosmia Flaccid paraplegia; global areflexia; lower limb paresthesia (day 2). Muscle weakness rapidly evolved to a flaccid areflexic tetraparesis, facial diplegia and respiratory failure admitted to the ICU (day 3) Day 2: protein level, 198 mg/dL; white cell count, 18/mm 3 ; negative PCR assay for SARS-CoV-2 Positive †: Anti-GM1 (1:70) Anti-GD1a (1:72) Anti-GD1b (1:64) Not tested Received one cycle of IVIg; still partially respiratory supported. Significant clinical improvement: MRC superior limb 4/5 and 3/4 inferior limb (day 33) *On CSF analysis, all the patients had a normal glucose level and IgG index and a polyclonal pattern on electrophoresis. The normal range for the protein level is <55 mg/dL. †Antiganglioside antibody ELISA test (Bühlmann GanglioCombi) was used to test for antibodies to GM1, GQ1b, GD1a and GD1b. Normal value <50. CSF, cerebrospinal fluid; ICU, intensive care unit; IVIg, intravenous immunoglobulin; MRC, Medical Research Council. copyright.
We have studied three members of a family (mother and two siblings) where the mother and father were first cousins and who presented a history of progressive mental deterioration, hyperkinetic extrapyramidal disorders, and epileptic seizures. They underwent the following examinations: cupremia, cupruria, and level of ceruloplasmin, genetic analysis for SCA1, 2, 3, 6, dentato-rubric-pallido-luysian atrophy, and Huntington's disease, electromyography (EMG), electroencephalography (EEG), brain magnetic resonance imaging (MRI), and investigation of acanthocytes with scanning electron microscopy. Genetic analysis was negative in all patients and acanthocytes were positive. EMG showed an axonal neuropathy in one sibling, EEG showed epileptiform activity in the two siblings, and MRI showed cortical atrophy in all subjects. This family shows the great variability of neuroacanthocytosis and a dominant autosomal transmission, as described only once previously in the literature.
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