Treatment Options and Outcomes in Susac Syndrome: A Retrospective Study

Susac syndrome (SS) is a rare autoimmune vasculo-occlusive disorder characterized by the classic triad of branch retinal artery occlusion, encephalopathy, and hearing loss. Prompt recognition of this entity and initiation of immunosuppressive therapy is vital. We reviewed the treatments utilized in a cohort of patients at a single academic center who met the criteria for definite and probable SS

A Case of Tuberculosis-related Leukocytoclastic Vasculitis Presenting With Peripheral Neuropathy.

Tuberculous granulomatous vasculitis is commonly associated with meningitis and retinitis. We describe a 39-year-old male, with a history of pulmonary tuberculosis (TB) who presented with progressive weakness, pain, tingling and numbness in the bilateral lower extremities. Significant atrophy and weakness of the lower extremities were evident along with absent reflexes. Nerve conduction studies and electromyography showed severe axonal polyneuropathy and denervation on the lower extremities. Nerve biopsy demonstrated small vessel leukocytoclastic vasculitis without any granuloma formation. Muscle biopsy was consistent with denervation and atrophy with target fiber changes. Tuberculosis-related vasculitis causing peripheral neuropathy is extremely rare and our case is unique in manifesting this presentation

Executive Dysfunction and Behavioral Symptoms Are Associated with Deficits in Instrumental Activities of Daily Living in Frontotemporal Dementia.

BACKGROUND: Deficits in instrumental activities of daily living (ADLs) may be more prominent in behavioral variant frontotemporal dementia (bvFTD) than in nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) or semantic variant primary progressive aphasia (svPPA). It is uncertain whether frontotemporal dementia (FTD) subgroups exhibit different patterns and/or predictors of functional impairment. METHODS: We examined data from participants diagnosed with bvFTD (n = 607), svPPA (n = 132), and nfvPPA (n = 155) who were included in the National Alzheimer\u27s Coordinating Center (NACC) Uniform Data Set (UDS) and assessed with the Functional Activities Questionnaire (FAQ). Stepwise multiple linear regression analyses were performed to identify associations between FAQ scores and cognitive/behavioral deficits using the NACC UDS neuropsychological testing battery and the Neuropsychiatric Inventory Questionnaire. RESULTS: FAQ scores were higher in bvFTD than svPPA or nfvPPA. Functional deficits across FTD subtypes differed in severity, but not pattern, and were driven by executive dysfunction and behavioral symptoms. CONCLUSION: Executive dysfunction and behavioral symptoms underlie instrumental ADL deficits in FTD, which are most prominent in bvFTD

Late-Onset Encephalopathy Associated With SARS-CoV-2 Infection

Several typical and atypical neurological manifestations of viral pandemics have been reported. Neurological manifestations of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have recently been reported. In this case report, we present a patient with encephalopathy as a late neurologic manifestation of SARS-CoV-2 infection. The patient initially tested positive for the novel coronavirus after presenting with fever, cough, and altered mental status. The symptoms resolved within 5 - 7 days and the patient was discharged home. He subsequently developed worsening encephalopathy in the absence of respiratory symptoms, required hospitalization, and tested positive for SARS-CoV-2. Complete workup was unrevealing otherwise. We advise clinicians to be aware of late neurological manifestations of coronavirus disease 2019 (COVID-19) including encephalopathy

Late-Onset Encephalopathy Associated With SARS-CoV-2 Infection.

Several typical and atypical neurological manifestations of viral pandemics have been reported. Neurological manifestations of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have recently been reported. In this case report, we present a patient with encephalopathy as a late neurologic manifestation of SARS-CoV-2 infection. The patient initially tested positive for the novel coronavirus after presenting with fever, cough, and altered mental status. The symptoms resolved within 5 - 7 days and the patient was discharged home. He subsequently developed worsening encephalopathy in the absence of respiratory symptoms, required hospitalization, and tested positive for SARS-CoV-2. Complete workup was unrevealing otherwise. We advise clinicians to be aware of late neurological manifestations of coronavirus disease 2019 (COVID-19) including encephalopathy

The Progressive Acalculia Presentation of Parietal Variant Alzheimer\u27s Disease.

BACKGROUND: Many patients with early-onset Alzheimer\u27s disease (EOAD; age of onset \u3c65 \u3eyears) have non-amnestic presentations involving language (logopenic primary progressive aphasia, lvPPA), visuospatial abilities (posterior cortical atrophy, PCA), and even asymmetric symptoms consistent with corticobasal syndrome (CBS). An inferior parietal lobule variant of EOAD commonly presents with progressive difficulty with calculations. METHODS: We reviewed 276 EOAD patients for presentations with predominant acalculia. These patients were diagnosed with clinically probable Alzheimer\u27s disease (AD) verified by positron emission tomography (PET) or cerebrospinal fluid amyloid-β or tau biomarkers. RESULTS: We identified 18 (9M/9F) (6.5%) EOAD patients with progressive acalculia that did not meet most criteria for lvPPA, visual PCA, or CBS. Their ages of onset and presentation were 56.6 (5.0) and 59.4 (6.5), respectively. Their acalculia was consistent with a primary acalculia ( anarithmetia ) not explained by language or visuospatial impairments. Many also had anomia (14/18), ideomotor apraxia (13/18), and the complete Gerstmann\u27s syndrome (7/18). Visual analysis of their diverse magnetic resonance imaging disclosed biparietal atrophy, disproportionately worse on the left. CONCLUSIONS: Primary acalculia may be the most common manifestation of an inferior parietal presentation of EOAD affecting the left intraparietal sulcus. This parietal variant also commonly involves progressive anomia, ideomotor apraxia, and other elements of Gerstmann\u27s syndrome. The early recognition of patients with this variant, which is distinguishable from lvPPA, visual PCA, or CBS, would be facilitated by its recognition as a unique subtype of EOAD

Changes in functional magnetic resonance imaging with

BACKGROUND: The neural substrates of AIMS AND OBJECTIVE: Pilot data of a functional magnetic resonance imaging (fMRI) study is presented to observe and understand the selective activations of designated brain regions during meditation. MATERIAL AND METHODS: Four trained healthy Patanjali Yoga practitioners in their mid-60s participated in this prototype interventional study. A three-part 1-min block design alternating between meditation (test) and relaxation (control) phase with an imaginary visual fixation and auditory stimulation was used. RESULT AND OBSERVATION: The fMRI images revealed strong activation in the right prefrontal regions during the visual and auditory fixation meditation phases compared to no activations during the relaxation phase. A comparison between the visual and auditory fixations revealed shifts within the prefrontal and temporal regions. In addition, activation in occipital and temporal regions was observed during the meditation phase. Occipital lobe activation was more apparent during visual meditation phase. CONCLUSION: It is concluded that specific fMRI brain activations are observed during different forms o