Postvaccination acute disseminated encephalomyelitis with <i>area postrema</i> syndrome and quasi benign paroxysmal positional vertigo: a case report

Area postrema syndrome (APS) develops in patients with lesions found in the floor of the fourth ventricle and manifests with nausea, intractable vomiting, and hiccup. APS is most commonly associated with neuromyelitis optica spectrum disorders although it may develop in some other conditions as well. We have presented a case study of APS with positional vertigo developed in a 41-year-old woman caused by acute disseminated encephalomyelitis after COVID-19 vaccination. Quasi benign paroxysmal positional vertigo acutely manifested with nausea, vomiting, and vertigo that dramatically worsened with head movement. Physical examination revealed patchy hypesthesia on the left side of the face and decreased convergence of the left eye. MRI scan showed a lesion adjacent to the floor of the fourth ventricle (area postrema). The manifestations totally regressed on glucocorticoids without any relapse during 1-year follow-up

Presyrinx in children with Chiari malformations

Our experience suggests that spinal cord inflammatory injury may trigger an otherwise silent structural abnormality, leading to the development of a presyrinx. This confirms the hypothesis that presyrinx derives from intraparenchymal accumulation of extracellular fluid

The Small Posterior Cranial Fossa Syndrome and Chiari Malformation Type 0

Patients showing typical Chiari malformation type 1 (CM1) signs and symptoms frequently undergo cranial and cervical MRI. In some patients, MRI documents &gt;5 mm of cerebellar tonsillar herniation (TH) and the diagnosis of CM1. Patients with 3&ndash;5 mm TH have &ldquo;borderline&rdquo; CM1. Patients with less than 3 mm of TH and an associated cervical syrinx are diagnosed with Chiari &ldquo;zero&rdquo; malformation (CM0). However, patients reporting CM1 symptoms are usually not diagnosed with CM if MRI shows less than 3&ndash;5 mm of TH and no syrinx. Recent MRI morphometric analysis of the posterior fossa and upper cervical spine detected anatomical abnormalities in and around the foramen magnum (FM) that explain these patients&rsquo; symptoms. The abnormalities include a reduced size of the posterior fossa, FM, and upper cervical spinal canal and extension of the cerebellar tonsils around the medulla rather than inferior to the foramen magnum, as in CM1. These morphometric findings lead some neurologists and neurosurgeons to diagnose CM0 in patients with typical CM1 signs and symptoms, with or without cervical syringes. This article reviews recent findings and controversies about CM0 diagnosis and updates current thinking about the clinical and radiological relationship between CM0, borderline CM1, and CM1

Pathogenetic role of myelitis for syringomyelia.

CSF-flow obstruction is regarded as a mandatory factor for the development of syringomyelia. However, there are conditions in which syringomyelia is not associated with evident persistent CSF-flow obstruction, as in the case of inflammatory spinal cord lesions. In these instances we hypothesize that the accumulation of vasogenic edema may play a role in the development of the syrinx. Recently proposed theories underline, even in the event of CSF-flow obstructions, a major role for the accumulation and final coalescence of interstitial spinal fluid, rather than CSF penetration through the spinal cord

Cerebellar Atrophy and Changes in Cytokines Associated with the CACNA1A R583Q Mutation in a Russian Familial Hemiplegic Migraine Type 1 Family

Background: Immune mechanisms recently emerged as important contributors to migraine pathology with cytokines affecting neuronal excitation. Therefore, elucidating the profile of cytokines activated in various forms of migraine, including those with a known genetic cause, can help in diagnostic and therapeutic approaches.Methods: Here we (i) performed exome sequencing to identify the causal gene mutation and (ii) measured, using Bio-Plex technology, 22 cytokines in serum of patients with familial migraine (two with hemiplegic migraine and two with migraine with aura) from a Russian family that ethnically belongs to the Tatar population. MRI scanning was used to assess cerebellar atrophy associated with migraine in mutation carriers.Results: Whole-exome sequencing revealed the R583Q missense mutation in the CACNA1A gene in the two patients with hemiplegic migraine and cerebellar ataxia with atrophy, confirming a FHM1 disorder. Two further patients did not have the mutation and suffered from migraine with aura. Elevated serum levels of pro-inflammatory and pro-nociceptive IL-6 and IL-18 were found in all four patients (compared to a reference panel), whereas pro-apoptotic SCGF-β and TRAIL were higher only in the patients with the FHM1 mutation. Also, cytokines CXCL1, HGF, LIF, and MIF were found particularly high in the two mutation carriers, suggesting a possible role of vascular impairment and neuroinflammation in disease pathogenesis. Notably, some “algesic” cytokines, such as β-NGF and TNFβ, remained unchanged or even were down-regulated.Conclusion: We present a detailed genetic, neurological, and biochemical characterization of a small Russian FHM1 family and revealed evidence for higher levels of specific cytokines in migraine patients that support migraine-associated neuroinflammation in the pathology of migraine