Fahr's disease with neuropsychiatric symptoms and intermittent course: a case report.

Fahr's disease is a rare neurodegenerative disorder with brain calcifications and neuropsychiatric symptoms. It can have variable phenotypic expression and intermittent symptomatology, making diagnosis challenging. In this report, we describe a young female patient presenting with symptoms of psychosis and confusion, which could be indicative of a delirium superimposed on the cerebral vulnerability associated with Fahr's disease. Notably, about two years prior, she experienced multiple episodes of tonic-clonic seizures that spontaneously resolved without pharmacological intervention. She had no previous psychiatric history. Following comprehensive investigations, other organic causes were ruled out, and Fahr's disease was diagnosed based on bilateral symmetrical brain calcifications seen on a head CT scan. Her treatment regimen encompassed antipsychotics and anticonvulsants. This case highlights the importance of considering Fahr's disease as a differential diagnosis in patients with new-onset neuropsychiatric symptoms. The case also explores the atypical early onset and intermittent nature of symptoms in the absence of a positive family history, highlighting the complexity of Fahr's disease. A multidisciplinary approach and regular follow-up are crucial for optimizing patient care and monitoring disease progression. Further research is needed to enhance our understanding of Fahr's disease and develop standardized treatment strategies for this rare condition.

Rituximab and risk of COVID-19 infection and its severity in patients with MS and NMOSD.

BACKGROUND: Choosing a safe disease modifying therapy during the COVID-19 pandemic is challenging. This case series study was conducted to determine the incidence rate and the course of Covid-19 infection in MS/NMOSD patients treated with Rituximab. METHODS: In this study, we designed a web-based questionnaire. Baseline information such as patient- reported walking disability, total number of Rituximab infusions received, delayed injections, occurrence of any relapse, and the use of corticosteroids during the pandemic were collected. Also, information regarding the Covid-19 pandemic such as adherence to self-isolation, any recent exposure to an infected individual and the presence of suggestive symptoms were collected. In case of positive test results, patients were grouped into 2 categories; mild to moderate and seriously ill and outcomes were evaluated as favorable (improved/ discharged) and unfavorable (expired). RESULTS: Two hundred fifty-eight patients with Multiple Sclerosis were enrolled in this study, 9 of the subjects (3.4%) were confirmed positive for Covid-19, five of which required hospitalizations (55.5%), two patients required ICU admission (22.2%) and 2 two patients died (22.2%). None of these patients ever mentioned using corticosteroids during the pandemic. In comparison to MS patients who were not receiving disease modifying therapy (DMT), our study indicated a higher incidence of Covid-19 infection, higher ratio of serious illness and a higher fatality ratio. CONCLUSIONS: Rituximab seems not to be safe enough during the pandemic.

Trans-lateral ventricular approach for surgical treatment of a high located basilar apex aneurysm: report of a rare presentation of the disease and surgical technique.

Surgical management of basilar apex aneurysms is considered among the most technically challenging procedures and high located basilar apex makes the procedure more challenging. Pterional trans-sylvian (with or without orbitozygomatic extension) and subtemporal are the most commonly used approaches, however, it is sometimes difficult or impossible to access a high-positioned aneurysm of basilar bifurcation without excessive retraction of brain and neurovascular structures. We are reporting a 65-year-old woman presented with chronic hydrocephalus secondary to a high-located basilar apex aneurysm surgically clipped through trans-frontal trans-ventricular trans-choroidal approach. We described anatomical details for selecting this approach and surgical steps during the procedure.

Wegener's Granulomatosis Presenting as Wallenberg Syndrome: A Case Report.

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, a vasculitis affecting small and medium sized vessels usually affects the upper and lower respiratory tract, the kidneys, and the eyes. Neurologic manifestation in central nervous system (CNS) is less frequent than the peripheral and usually is in form of stroke. Few cases of lateral medullary ischemic stroke (Wallenberg syndrome) due to GPA have been reported. A 41 year-old female, presented with acute vertigo, nausea/vomiting, hiccups, dysphagia. In physical examination she had a saddled nose, horner syndrome, soft palate paralysis, crossed hypoesthesia of face, and limbs and hemi-ataxia. Brain magnetic imaging revealed a left lateral medullary infarction and sinusitis confirmed by paranasal Sinus CT scans. Chest CT showed a cavitary mass. Laboratory findings were remarkable for anemia, elevated erythrocyte sedimentation rate, and C-reactive protein. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA)/anti-proteinase 3 was positive. Diagnosis of GPA was established and treatment was started. During 6-month follow-up improvement was satisfying and no relapses occurred. Medullary infarct is reported in few GPA patients, especially at presentation. Definite diagnosis is based on tissue biopsy. Although in context of extra CNS involvement and positive ANCA diagnosis can be made confidently. Treatment of choice in CNS involvement is not clear, corticosteroids and immunosuppressives seem effective. CNS involvement, especially stroke may present GPA or accompany extra CNS symptoms. Prompt diagnosis and treatment is essential.

Important Factors Influencing Severity of Common Variable Immunodeficiency.

BACKGROUND: Common variable immunodeficiency (CVID) is a primary immune deficiency with heterogeneous complications. The purpose of this study is to determine disease severity in a cohort of CVID patients based on the suggested scoring system and investigate predisposing factors which would be helpful to predict the severity of the disease. METHODS: The study population comprised 113 CVID patients (69 males and 44 females) who were visited at Children's Medical Center (Pediatrics Center of Excellence affiliated to Tehran University of Medical Sciences, Tehran, Iran) during the last 30 years (from 1984-2014). According to a suggested severity scoring system, patients were divided into two groups, A and B. The clinical severity of the disease in patients was assessed with severity scores including 15 unlucky complications of the disease such as numbers of past meningitis, encephalitis or pneumonias, development of bronchopulmonary pathologies, presence of lymphoproliferative disorders, autoimmunity or malignancy. RESULTS: The mean serum IgG level was significantly higher in group B (308.6±195.9) compared to group A (177.8 ± 151.9; P = 0.03). Patients in group B had a significantly higher percentage of CD8 (P = 0.003). However, they had lower percentage of CD4 lymphocytes (P = 0.08), switched memory B cells (CD27+IgM-IgD-) (P < 0.01) and regulatory T cells (P = 0.02) than group A. CONCLUSION: Using standard and universal scoring system and understanding of related factors can be applicable in clinical settings for prognosis assessment of CVID patients.