Neuro-Behçet's Disease Onset in the Context of Tuberculous Meningoencephalitis: A Case Report.

Behçet's disease (BD) is a systemic vasculitis that frequently presents with a relapsing-remitting pattern. CNS involvement (Neuro-Behçet) is rare, affecting approximately 10% of patients. Its etiological mechanisms are not yet fully understood. The most commonly accepted hypothesis is that of a systemic inflammatory reaction triggered by an infectious agent or by an autoantigen, such as heat shock protein, in genetically predisposed individuals. Mycobacterium tuberculosis is known to be closely interconnected with BD, both affecting cell-mediated immunity to a certain extent and probably sharing a common genetic background. We present the case of a 34-year-old Caucasian woman who had been diagnosed with tuberculous meningitis 15 months prior, with significant neurological deficits and lesional burden on MRI with repeated relapses whenever treatment withdrawal was attempted. These relapses were initially considered as reactivation of tuberculous meningoencephalitis, and symptoms improved after a combination of antituberculous treatment and corticosteroid therapy. After the second relapse, the diagnosis was reconsidered, as new information emerged about oral and genital aphthous lesions, making us suspect a BD diagnosis. HLA B51 testing was positive, antituberculous treatment was stopped, and the patient was started on high doses of oral Cortisone and Azathioprine. Consequently, the evolution was favorable, with no further relapses and slow improvements in neurological deficits. To our knowledge, this is the first report of Neuro-Behçet's disease onset precipitated by tuberculous meningitis. We include a review of the available literature on this subject. Our case reinforces the fact that Mycobacterium tuberculosis infection can precipitate BD in genetically predisposed patients, and we recommend HLA B51 screening in patients with prolonged or relapsing meningoencephalitis, even if an infectious agent is apparently involved.

Thunderclap headache revealing dural tears with symptomatic intracranial hypotension: Report of two cases.

Cerebrospinal fluid (CSF) leakage is considered the cause of spontaneous intracranial hypotension (SIH), an important etiology for new daily persistent headaches and a potentially life-threatening condition. Minor traumatic events rarely lead to CSF leakage, contrasting with iatrogenic interventions such as a lumbar puncture or spinal surgery, which are commonly complicated by dural tears. Most meningeal lesions are found in the cervicothoracic region, followed by the thoracic region, and rarely in the lumbar region, and extremely rarely in the sacral region. We describe two patients admitted to our hospital for severe headaches aggravated in the orthostatic position, with a recent history of minor trauma and sustained physical effort, respectively. In the first case, a bone fragment pierced an incidental congenital meningocele creating a dural fistula. An extensive extradural CSF collection, spanning the cervicothoracic region (C4-T10), was described in the second case. In both patients, the clinical evolution was favorable under conservative treatment.

Chorea and Cognitive Impairment in JAK2V617F-Positive Myeloproliferative Disorders: A Case Report and Literature Review.

Chorea is a hyperkinetic movement disorder, accompanied by dystonia, myoclonus, tics, stereotypies, and tremors. It is characterized by excessive, purposeless movements that are distressing, irregularly timed, and randomly distributed. Chorea can be present in many diseases, such as hereditary, metabolic disturbance, drug-induced, and functional disorders, and, rarely, genetic, autoimmune, and infectious diseases. Primary myelofibrosis (PMF) is a myeloproliferative neoplasm that leads to ineffective clonal hematopoiesis, fibrous tissue deposits in the bone marrow, extramedullary hematopoiesis, and splenomegaly. In rare cases, following uncertain pathological mechanisms, it can present with chorea, particularly affecting the limbs, head, and orofaciolingual muscles. We present a case of a male patient with evolving PMF over several years who was admitted for progressive cognitive impairment and generalized involuntary movement disorder. We also present a review of all cases of myeloproliferative disorders presenting with chorea published in the last 40 years.

Double seropositive neuromyelitis optica associated with COVID-19: A case report.

Neuromyelitis optica spectrum disorders are characterized by severe demyelination and axonal damage with autoimmune mechanisms, predominantly targeting the optic nerves and the spinal cord. Patients often test positive for anti-AQP4 antibodies, while some have anti-MOG antibodies. Double seropositivity has been described, with a variable prevalence (0 to 26%) dependent on the testing method. The clinical significance of double seropositivity remains unclear. We present the case of a 65-year-old patient, admitted to our clinic with optical neuritis, followed up approximately 10 days later by cervical myelitis, who tested positive for both anti-AQP4 and anti-MOG antibodies. The clinical onset coincided with a mild form of SARS-CoV-2 infection. The neurological symptoms were initially relatively subdued, which delayed the diagnosis. The patient was not vaccinated against SARS-CoV-2. The clinical picture was compatible with an anti-AQP4 phenotype. The patient was started on corticosteroid therapy, under which the clinical response was good. Our case reinforces the idea that SARS-CoV-2 can precipitate autoimmune demyelinating diseases since SARS-CoV-2 infection has already been demonstrated as a risk factor for NMOSD relapses. To the best of our knowledge, this is the first reported case of double seropositive neuromyelitis optica associated with COVID-19. We expect that in the near future, as the true burden of COVID becomes clearer, we shall encounter other cases which can trace their apparent clinical onset to a SARS-CoV-2 infection. Careful attention should be paid to the apparent minor neurological symptoms of COVID-19.

Reassessing the Diagnostic Utility of the Split Hand Index in Amyotrophic Lateral Sclerosis Patients-The Divide by Zero Problem.

We set out to assess the diagnostic utility of the split hand index (SHI) for amyotrophic lateral sclerosis (ALS) and also to see if and how it can be applied to severely atrophied muscles, a frequent finding in this setting. We enrolled 38 patients from our clinic, 19 diagnosed with ALS and 19 controls, matched for age and sex. The SHI was calculated, on both sides, for all the patients. We calculated a SHI of 0 when the abductor pollicis brevis muscle (ABD) or first dorsal interosseous muscle (FDI) compound muscle action potentials (CMAPs) were unobtainable, and we allotted a value of 0.1 mV to abductor digiti minimi muscle (ADM) CMAP, for mathematical purposes, when the value would have been 0. The means differences were large between groups, with a significant variance heterogeneity. We performed a ROC analysis and obtained an accuracy of 0.83 for a SHI of 7.2, p-value < 0.0001. In conclusion, we reaffirm the utility of the SHI in the diagnosis of ALS, especially in limb onset cases, and we think that it can be safely extended to severely atrophied muscles with absent or very low CMAP values, without endangering the sensitivity or specificity.

Magnetic Resonance Imaging of Multiple Cerebral and Spinal Cavernous Malformations of a Patient with Dementia and Tetraparesis.

Cavernomas are rare cerebrovascular malformations that usually occur in sporadic forms with solitary lesions located most often in the hemispheric white matter, but also in the infratentorial or spinal region. Multiple lesions at different CNS levels are considered a hallmark for the familial form of the disease. The diagnostic modality of choice for cerebral cavernous malformations (CCMs) is magnetic resonance imaging (MRI). We present an intriguing case of a 65-year-old male admitted to our hospital with tetraparesis and cognitive impairment where highly sensitive MRI sequences identified many cerebral cavernous lesions at the supra-, infratentorial and cervical-thoracic spine levels, some of them with recent signs of bleeding in a patient with oral anticoagulant therapy due to atrial fibrillation. The mechanism of cognitive impairment in this patient is most probably the interruption of strategic white matter tracts, as it is known to happen in other subcortical vascular pathologies. MRI can be helpful not only in mapping the anatomical distribution of lesions, but also in weighing the risks and making decisions regarding whether or not to continue oral anticoagulant therapy.

Zoster Cranial Polyneuropathy in a COVID-19 Patient.

BACKGROUND Ramsay Hunt syndrome is a rare form of herpes zoster caused by the reactivation of the varicella-zoster virus in the geniculate ganglion. The main clinical manifestations are peripheral facial palsy, vesicular rash in the ear, and ipsilateral auricular pain, and sometimes vertigo. COVID-19 is a new multisystemic infectious disease that, in addition to common respiratory manifestations, it is known to affect the immune system, primarily depressing cellular immunity. CASE REPORT A 54-year-old woman was admitted to our hospital with an acute vestibular syndrome and diplopia. She had been diagnosed 3 years prior with interstitial lung disease for which she was taking methylprednisolone. At admission, she tested positive for SARS-CoV-2. In the following days, she developed a sixth nerve palsy on the left side and a right peripheral facial palsy on the right side, followed by a typical zoster rash on the ipsilateral ear. One month later, she developed acute severe hearing loss on the right side. There were no COVID-19 symptoms during her stay in our hospital. The MRI showed Gd enhancement of both facial nerves. Under antiviral and corticoid treatment, the evolution was favorable, with marked improvement at 6 months. CONCLUSIONS COVID-19 increases the risk for herpes zoster infection, probably through induced depression of the cellular immunity. Our case suggests Ramsay Hunt syndrome can be the presenting symptom and sometimes the only symptom of COVID-19. This also seems to be true for other cranial neuropathies, and we recommend testing these patients even if there are no other manifestations.

Aortic Dissection: An Easily Missed Diagnosis when Pain Doesn't Hold the Stage.

BACKGROUND Type A aortic dissection (AD) is a rare disease, with a high mortality rate. Its most common symptom is thoracic pain, which is nevertheless absent in about 6% of cases. Neurologic complications are extremely rare and include ischemic stroke and ischemic neuropathy (which are the most common as presenting symptoms), spinal cord ischemia, and hypoxic encephalopathy. These rare neurological presentations can often be missed at initial clinical examination. CASE REPORT We report 2 cases of patients presenting with seemingly mild neurological symptoms. However, diagnostic tests revealed acute type A AD, and further steps were taken. CONCLUSIONS Although it is a rare cause of transient stroke or peripheral nerve ischemia, AD should be quickly recognized as a potential cause of new-onset neurological manifestations.