scholarly journals Primary Angiitis of the Central Nervous System: A Report of Three Cases from a Single Colombian Center

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Nicolás Coronel-Restrepo ◽  
Fabio Bonilla-Abadía ◽  
Omar A. Cortes ◽  
Jorge H. Izquierdo ◽  
Alberto M. Shinchi ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Brain Biopsy ◽  
System A ◽  
Primary Angiitis ◽  
The Central Nervous System ◽  
Systemic Manifestations ◽  
Low Incidence

The primary angiitis of the central nervous system (PACNS) is an entity with a very low incidence and prevalence. It is not clear why the inflammatory process of this entity is limited to the cerebral vasculature without systemic manifestations. Its clinical manifestations are very heterogeneous and make clinical diagnosis difficult. In most cases, a brain biopsy is required. Only the clinical suspicion and the ability to recognize the possible clinical and imagenological patterns of presentation make an accurate diagnosis possible. The vast majority of the treatment recommendations are given by series of case reports. The following paper described the clinical, imagenological, and histopathological characteristics of three Colombian patients with PACNS. The strategic therapeutic used in shown.

scholarly journals Neurobrucellosis mimicking primary vasculitis of the central nervous system: we should perform a metagenomic analysis of the cerebrospinal fluid prior to brain biopsy

2021 ◽  
Author(s):  
Marina Barrionuevo Mathias ◽  
Fernando Gatti ◽  
Gustavo Bruniera ◽  
Vitor Paes ◽  
Gisele Sampaio Silva ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Psychiatric Symptoms ◽  
Brain Mri ◽  
Genetic Material ◽  
Clinical Manifestations ◽  
Brain Biopsy ◽  
Metagenomic Analysis ◽  
Normal Brain ◽  
The Central Nervous System

Context Primary angiitis of the central nervous system (PACNS) is characterized by the inflammation of small and medium CNS arteries; the clinical manifestations include headache, cognitive impairment and focal neurological deficits. The gold standard test for diagnosis is brain biopsy. Neurobrucellosis is an infection associated with cattle farming, which leads to neurological and psychiatric symptoms. We report a case of neurobrucellosis mimicking PACNS. Case report Male, 32 years old, with fever, headache, dizziness and cognitive impairments for 30 days. History of stroke 2 years before, with mild sequelae right hemiparesis; investigation showed suspected intracranial dissection. On physical examination, he had apathy, preserved strength, reduced reflexes with plantar flexor responses. General laboratory tests, autoantibodies and serology were normal. Brain MRI showed deep left nucleocapsular gliosis and cerebral angiography revealed stenosis of the ICA and MCA. CSF showed 42 cells/ mm³, glucose 46 mg/dL, protein 82 mg/dL. Blood PCR was negative for Brucella. Immunophenotyping of the CSF and PET-CT excluded neoplasia. Brain biopsy was inconclusive for vasculitis. Metagenomic analysis of the CSF detected 78% of Brucella genetic material. Serum agglutination test was 1:40 for brucella. Conclusions PACNS is diagnosed by exclusion. The patient filled criteria for possible PACNS, image compatible with vascular stenosis, but inconclusive brain biopsy. Brucellosis is an endemic disease in underdeveloped countries that can present as CNS vasculitis. Metagenomic analysis allows the detection of different pathogens using a single method. The case illustrates the use of metagenomics in rare diseases characterized by vasculitis, with change in clinical outcomes and conduct.

Primary Angiitis of the Central Nervous System: A Misinterpreted Clinical Onset of CNS Vasculitis

2004 ◽  
Vol 53 (1) ◽  
pp. 40-42 ◽  
Author(s):  
Antonia Ceccarelli ◽  
Roberto De Blasi ◽  
Isabella Pavone ◽  
Paolo Lamberti ◽  
Aristide Carella ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cns Vasculitis ◽  
Clinical Onset ◽  
System A ◽  
Primary Angiitis ◽  
The Central Nervous System

scholarly journals Creutzfeldt–Jakob Disease with a Five-Year Clinical Course, Multicentric Cerebellar Prion Plaques and Prior History of Biopsy-Proven Primary Angiitis of the Central Nervous System: A Case for Iatrogenic Exposure?

Viruses ◽  
2020 ◽  
Vol 12 (12) ◽  
pp. 1411
Author(s):  
Kristina Jeon ◽  
Jeffrey T. Joseph ◽  
Gerard H. Jansen ◽  
Anne Peterson ◽  
J. David Knox ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Parietal Lobe ◽  
Clinical Course ◽  
Prior History ◽  
System A ◽  
Primary Angiitis ◽  
History Of ◽  
Jakob Disease ◽  
The Central Nervous System

Creutzfeldt–Jakob disease (CJD) is a rapidly progressive neurodegenerative disease that can arise spontaneously, genetically, or be acquired through iatrogenic exposure. Most patients die within a year of symptom onset. It is rare, affecting 1–2 per million per year, and the majority of cases are sporadic. Primary angiitis of the central nervous system (PACNS) is also rare, affecting 2.4 per million per year. We present a case of an unusually long clinical course of CJD, almost five years, which began with symptoms of apraxia. The patient had biopsy-proven PACNS 16 years prior to clinical presentation, and the site of biopsy was the left parietal lobe. Autopsy revealed multicentric prion plaques in the cerebellum, in the setting of normal genetic testing. The presence of plaques in the cerebellum, and prior neurosurgery, raises the possibility of iatrogenic exposure. We present the details of this case, including pathology from the original biopsy and final autopsy, as well as a review of relevant cases in the literature.

IVIG in childhood primary angiitis of the central nervous system: A case report

2020 ◽  
Vol 42 (9) ◽  
pp. 675-679
Author(s):  
Hiroya Nishida ◽  
Satoko Kumada ◽  
Takashi Komori ◽  
Keisuke Takai ◽  
Harushi Mori ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
System A ◽  
Primary Angiitis ◽  
The Central Nervous System

scholarly journals Original article Primary angiitis of the central nervous system: a study of histopathological patterns and review of the literature

2014 ◽  
Vol 2 ◽  
pp. 187-196 ◽  
Author(s):  
Vaishali Suri ◽  
Aanchal Kakkar ◽  
Mehar C. Sharma ◽  
Madakasira V. Padma ◽  
Ajay Garg ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Review Of The Literature ◽  
System A ◽  
Primary Angiitis ◽  
The Central Nervous System

scholarly journals Ataxia as the main manifestation of tumor-like primary angiitis of the central nervous system: a case report and literature review

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Yu-Jing Huang ◽  
Li Zhang ◽  
Ying Mao ◽  
Guang-Xian Nan
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Glucocorticoid Therapy ◽  
Cerebellar Hemisphere ◽  
Case Presentation ◽  
System A ◽  
Intravenous Drip ◽  
Primary Angiitis ◽  
The Central Nervous System ◽  
The Right

Abstract Background Primary angiitis of the central nervous system (PACNS) is a rare disease, and tumor-like primary angiitis of the central nervous system is even rarer. Histopathology is the gold standard for tumor-mimicking PACNS. However, pathological diagnosis is relatively limited due to fewer biopsy opportunities. Case presentation A 68-year-old male presented with ataxia, and was diagnosed with tumor-like primary angiitis of the central nervous system. The patient underwent Intravenous drip glucocorticoid therapy (10 mg of dexamethasone, daily). After 10 days, the symptoms of the patient were completely relieved. Radiology revealed that the low density lesion in the right cerebellar hemisphere obviously narrowed. Cyclophosphamide therapy was not initiated. Conclusion It is crucial for clinicians to be aware of changes in radiology that indicate PACNS, since the diagnosis of tumor-like PACNS remains quite challenging. Glucocorticoid therapy is an effective therapy in this condition, and the prognosis can be favorable.

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