Introduction: Progressive Multifocal Leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system caused by reactivation of the JC virus. It usually occurs in immunocompromised patients. The majority of PML cases present with supratentorial involvement. In contrast, infratentorial involvement is exceedingly rare in PML. The aim to report an atypical presentation of PML in a patient with ataxic syndrome and retrovirus with infra-tentorial involvement. This is a case report study. The information of this work was obtained through review of the medical record. Case report: A 38-year-old, female, HIV-infected since 2015 and started antiretroviral therapy only in 2018. In June 2019, she developed paresthesia in lower limbs, imbalance and difficulty walking, requiring support. Two months later she evolved with dysphagia, dysarthria, and limitation in activities of daily living. In September 2019, she presented for evaluation in a wheelchair. On neurological examination, she had bi-directional horizontal nystagmus, cervical dystonic tremor, global ataxia, altered deep sensation and spastic gait. Her metabolic profile was normal. Brain magnetic resonance imaging (MRI) showed atrophy of the cerebellar leaflets and vermis associated with extensive T2/FLAIR inversion recovery hyperintensities involving the cerebellum and bilateral middle cerebellar peduncle. Cerebrospinal fluid analysis revealed hyperproteinorraquia and posterior examination revealed positive JC virus DNA. Conclusion: In the diagnosis approach of acquired ataxias, it is essential to establish a propaedeutic directed to the patient’s clinical context. Investigating opportunistic infectious diseases is essential in care and treatment especially for immunosuppressed patients.
Introduction: Tumor necrosis factor (TNF) antagonists have emerged as one of the most effective treatment options for patients with autoimmune diseases, including psoriatic arthritis. However, these agents carry a risk of severe adverse effects, including demyelinating disorders. The aim to report a case of multiple sclerosis associated with the use of adalimumab. This is a case report study. The information of this work was obtained through review of the medical record. Case report: A 40-year-old female with psoriatic arthritis on adalimumab (ADM) treatment. She developed paresthesia in her lower limbs, which was initially attributed to anxiety. Six months later, she presented with decreased visual acuity in her left eye, which was diagnosed as optic neuritis. She received pulse therapy with methylprednisolone and underwent investigation for multiple sclerosis (MS). Magnetic resonance imaging (MRI) of the brain revealed multiple lesions with high signal intensity in T2/FLAIR on periventricular regions bilaterally. Furthermore, MRI of the thoracic spine showed high signal intensity on posterolateral cords at levels T5 to T9. These findings fulfilled the revised McDonald Criteria for MS and suggested a link to ADM treatment. On neurological examination, mild left lower limb paresis, bilateral plantar-cutaneous reflex, hyperreflexia in the lower limbs, and mild hypopalesthesia in her right leg. ADM treatment was discontinued, and although she reported a slight worsening of her skin condition, there were no further signs of psoriatic arthritis. Conclusion: Neurological adverse events associated with TNF antagonists are rare but must be considered. If neurological disorders develop during therapy, prompt discontinuation of the drug is recommended.
Introduction: Neurosarcoidosis (NS) is a rare and often underdiagnosed manifestation of sarcoidosis, which is an inflammatory disorder that can affect multiple organs, including the nervous system. The main clinical feature is cranial neuropathy, especially involvement of the optic nerve and facial nerve. These alterations make a differential diagnosis with Tolosa-Hunt syndrome. The aim is to report a case of neurosarcoidosis. This is a case report study. The information of this work was obtained through review of the medical record. Case report: Female, 39-year-old. In 2005, she started pain and paralysis of the lateral movement of the left eye, which was resolved with mucocele removal surgery. Presented three similar cases in 2010, 2015, and 2020, resolved with the use of anti-inflammatory drugs. In 2021, she presented left facial paralysis, left eyelid ptosis, visual blurring and headache. During hospitalization, the possibility of Tolosa-Hunt syndrome was investigated. Imaging exams and laboratory tests were performed without significant alterations. In 2022, she presented a similar clinical manifestation. Magnetic resonance imaging with angiography were performed, which showed thickening and enhancement of the pachymeninges in the occipital regions and cerebellar hemispheres, suggesting the possibility of meningeal granulomatous involvement. A biopsy with histopathological and immunohistochemical analysis was performed, confirming the diagnosis of neurosarcoidosis. Conclusion: This case report highlights the importance of considering neurosarcoidosis as a differential diagnosis in cases of multiple cranial neuropathy, such as in Tolosa-Hunt syndrome. Early recognition and diagnosis can lead to timely treatment and improved outcomes for patients.
Introduction: Cryptococcoma is a rare CNS infection caused by Cryptococcus neoformans, with a granulomatous lesion. It’s even rarer in immunocompetent patients. Diagnosis requires histopathological analysis and treatment with amphotericin B, often with surgical resection. The aim is to report four cases of cerebral cryptococcoma In Immunocompetent Patients. This is a case report study. The information of this work was obtained through review of the medical record. Case report: We report on four cases of cerebral cryptococcoma in immunocompetent patients. All cases presented with images of expansive lesions and compatible histopathological analysis. The first case was a 62-year-old man with dysphasia and decreased strength on the right side, and underwent total surgical resection, followed by 23 days of amphotericin B treatment. The second case was a 62-year-old woman who presented with orofacial dyskinesia and decreased strength on the right side, and underwent stereotactic surgery, followed by antifungal treatment. After 10 days of hospitalization, the patient developed respiratory insufficiency, progressed to cardiorespiratory arrest and brain death. The third case was a 54-year-old woman who presented with a skin infection caused by Cryptococcus. During treatment with fluconazole, the patient experienced altered sensory perception and left hemiparesis. Surgical resection of the lesion was performed, followed by 30 days of antifungal treatment. The fourth case was a 54-year-old man who presented with pulsatile headache and right hemiparesthesia, and underwent stereotactic neurosurgery, followed by 12 days of amphotericin B treatment. Conclusion: Cryptococcus neoformans infection in the CNS, manifested in the form of cryptococcoma, although rare, should be considered as a differential diagnosis for brain expansive lesions in immunocompetent individuals.
Introduction: Multiple sclerosis (MS) is an inflammatory, immune-mediated disease of the central nervous system, characterized by multifocal areas of demyelination. There is a knowledge gap in understanding the disease, particularly the progressive forms in different regions of the country, such as the Northeast. Objectives: To report the profile of progressive MS at a reference center in Ceará. Methods: This observational and cross-sectional study was conducted through a review of medical records and interviews at a Reference Center for Demyelinating Diseases in the city of Fortaleza, Ceará. Results: From 283 patients with MS diagnosis according to the 2017 McDonald criteria, 36 (12.7%) had progressive phenotype, 20 (56%) secondary progressive and 16 (44%) primary progressive. From those 36 patients currently followed up, 25 (69.4%) were women, 19 (52.8%) were caucasian and 18 (50%) were high educated. The median follow-up time was five years and the peak prevalence was between the 4th and 5th decades. Regarding disability, 27.8% already used support in the first assessment (EDSS 6.0), and in the last assessment, considering the last EDSS recorded, the percentage of patients in need of support almost doubled: 52.8%. Regarding the first disease-modifying drugs (DMD), there was a prevalence of interferons, considering that it was the most used class in the 1990s. Considering the current DMD, there is a prevalence of monoclonal antibodies (47%), with Natalizumab (25%) and Ocrelizumab (22%). In a comparative analysis, there is a drop in the use of interferons (8%). Conclusion: Understanding the clinical-epidemiological profile of MS patients with a progressive phenotype is essential to guide the propaedeutic reasoning, guise the conduct and restructure the dynamics of health services.
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