ABSTRACT
Primary angiitis of the central nervous system (PACNS) refers to a rare form of vasculitis of unknown cause, with a challenging diagnostic work-up. We report the case of a 57-year-old patient who presented with transient episodes of headache and global aphasia. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis with moderate elevated protein and normal glucose. CSF and serum tests for infections and autoimmune/paraneoplastic antibodies were negative, except CSF polymerase chain reaction testing that detected Epstein-Barr virus (EBV). Magnetic resonance imaging of the brain with intravenous gadolinium showed meningeal enhancement and pachymeningitis. Due to continuous relapsing episodes of aphasia, a leptomeningeal and brain tissue biopsy was performed and revealed lesions of granulomatous necrotising vasculitis of medium-sized leptomeningeal and intracranial vessels, as well as negative in situ hybridism for EBV. A diagnosis of primary granulomatous necrotising angiitis of the central nervous system was made, and the patient was treated with intravenous methylprednisolone and oral cyclophosphamide, showing excellent response to treatment. Diversity in clinical and laboratory features makes it difficult for PACNS to be distinguished by other systemic vasculitides. Laboratory tests and neuroimaging can provide guidance in evaluation of the patients and exclude other possible causes, but tissue biopsy remains the gold standard for a definite diagnosis.
Subject(s)
Aphasia , Epstein-Barr Virus Infections , Vasculitis, Central Nervous System , Humans , Middle Aged , Epstein-Barr Virus Infections/complications , Herpesvirus 4, Human , Vasculitis, Central Nervous System/complications , Vasculitis, Central Nervous System/diagnosis , Vasculitis, Central Nervous System/drug therapy , Headache/etiology , Aphasia/complicationsABSTRACT
Vertebrobasilar dolichoectasia is a clinical entity associated rarely with obstructive hydrocephalus. We present a 48-year old male with a profound dilatation of the ventricular system due to a dolichoectatic basilar artery, as appeared in imaging studies. The patient suffered from longstanding hydrocephalus and presenile dementia. The underlying mechanism for obstructive hydrocephalus due to vertebrobasilar dolichoectasia is considered to be both a water-hammer effect and a direct compression of adjacent structures. We suggest prompt surgical intervention upon diagnosis as a first choice treatment in order to avoid further complications.
