Cerebral amyloid angiopathy with related inflammation masquerading as crescendo transient ischaemic attacks

Cerebral amyloid angiopathy with related inflammation (CAA-RI) is an uncommon inflammatory subtype of CAA, with a variety of presentations that can mimic other focal and diffuse neurological disorders. We present a 63-year-old man with recurrent stereotyped focal neurological symptoms, who was initially diagnosed as capsular warning syndrome and treated with antithrombotic therapy. Atypical imaging led to further investigation including a cerebral biopsy, which confirmed CAA-RI; he improved clinically and radiologically with immunosuppression. This case highlights how CAA-RI is often under-recognised and that patients risk receiving inappropriate anticoagulation and delay in starting immunosuppression.

Snorting the Brain Away: Cerebral Damage as an Extension of Cocaine-Induced Midline Destructive Lesions

Cocaine consumption is associated with a variety of clinical manifestations. Though cocaine intranasal inhalation always determines nasal mucosal damages, extensive septum perforations, and midline destructions—known as cocaine-induced midline destructive lesions (CIMDL)—affect only a limited fraction of patients. CIMDL is viewed as a cocaine-associated autoimmune phenomenon in which the presence of atypical anti-neutrophil cytoplasmic antibody (ANCA) promotes and/or defines the disease phenotype. A 51-year-old man presented with an intracranial tumor-like lesion by its space-occupying effect. CT also revealed the destruction of the nasal septum and skull base. A diagnosis of CIMDL was made in light of the patient’s history as well as findings of the physical and endoscopic examinations, imaging studies, and laboratory testing. There was no evidence of other pathologies. Histopathological results from cerebral biopsy led us to consider the intracranial pathology as an extension of the CIMDL. CIMDL is the result of a necrotizing inflammatory tissue response triggered by cocaine abuse in a subset of predisposed patients. The reported case is the first CIMDL consistent with brain extension mimicking a tumor-like lesion. While the presence of atypical ANCA seems to promote and/or define the disease phenotype, the specific role of these and other circulating autoantibodies needs further investigation.

MULTIPLE POSTERIOR FOSA ASPERGILOMAS IN A PATIENT WITH CHRONIC GRANULOMATOSIS

The invasive infection with Aspergillus fumigatus usually occurs in immunocompromised patients. CNS involvement is extremely rare in children. Usually, the diagnosis is hard, often made after the death of the patient. We describe the first report of a pediatric case of multiple CNS aspergillomas confirmed by a biopsy. We present the case of 3 years old boy who was hospitalized for evolving intracranial hypertension with a cerebellar syndrome. A brain CT showed a left cerebellar mass and early tonsillar commitment and a supra-tentorial lesion in the right occipital lobe. Therefore, a metastatic tumor was suspected and an emergency surgical intervention with cerebral biopsy and ventriculocisternostomy was performed. The cerebral biopsy revealed an Aspergillus fumigatus granuloma and further investigations showed that the patient has chronic granulomatosis due to a homozygous mutation of CYBA gene encoding p22phox. After a favorable evolution in reanimation, he continued the treatment with Voriconazole. At last follow-up, the neurological examination finds no sign of motor focus, no cerebellar syndrome or nystagmus. Walking is difficult with the enlargement of the support base. CNS invasive infection with Aspergillus fumigatus in a child is extremely rare and the presentation might mimic that of a tumor especially with a location of aspergilloma as in our case both supratentorially and infratentorially.

Diagnostic dilemmas of Rasmussen’s encephalitis in adults

Introduction. Rasmussen?s encephalitis (RE) represents a rare, progressive, and inflammatory disease of the brain. Its detection in adults is a great challenge in clinical medicine. The aim of this paper is to highlight the diagnostic dilemma of RE in adults. Case outline. A 46-year-old woman was hospitalized due to persistent intense diffuse headaches, followed by nausea and the urge for vomiting that made her wake up during the night. On several occasions, she had transitory speech and memory disorders, and right hand numbness. Magnetic resonance (MR) imaging findings were as follows: occipitoparietal left in the deep white matter, as well as subcortical T2/flair white matter hyperintensities, T1-hypointense change involving the corpus callosum. MR spectroscopy showed an increased level of choline/creatinine (Cr) (2.12), a reduction of N-acetylaspartate/ Cr (1.27), an increased level of myo-inositol/Cr (1.20), and the presence of lactate. The patient refused lumbar puncture. Due to the described changes close to the speech center, cerebral biopsy was not taken. Even after five years, MR and spectroscopic findings are unchanged, while the clinical condition remains stable and unchanged. Conclusion. This case highlights the diagnostic dilemmas that arise in adult-onset RE and suggests that this diagnosis should be considered in patients of any age with the appropriate clinical picture.

Diagnostic Performance of Galactomannan Antigen Testing in Cerebrospinal Fluid

Testing cerebrospinal fluid (CSF) for the presence of galactomannan (GM) antigen may help in diagnosing cerebral aspergillosis (CA). However, the use of the CSF GM test as a diagnostic test has been little studied. We evaluated its diagnostic performance by comparing the CSF GM optical density indexes (ODI) at different cutoffs in patients with probable and proven CA to those in patients without CA. Patients from 2 tertiary referral hospitals with suspected CA between 2004 and 2014 and in whom CSF GM ODI had been determined were selected. European Organization for Research and Treatment of Cancer/Invasive Infectious Diseases Study Mycoses Group (EORTC/MSG) definitions of invasive aspergillosis and CA were used, but with the exclusion of the test to be validated (i.e., the CSF GM test) as a microbiological EORTC/MSG criterion. The study population consisted of 44 patients (4 with proven CA, 13 with probable CA, and 27 with no CA). Of the 17 patients with CA, 15 had a CSF GM ODI of ≥2.0. Of 27 patients without CA, 26 had a CSF GM ODI of <0.5 and 1 had a CSF GM ODI of 8.2. When a GM CSF ODI cutoff of 1.0 was used, the sensitivity, specificity, and positive and negative predictive values were 88.2%, 96.3%, 93.8%, and 92.9%, respectively. The same results were found when a CSF GM ODI cutoff of 0.5 or 2.0 was used. Testing GM in CSF has a high diagnostic performance for diagnosing CA and may be useful to diagnose or virtually rule out the infection without the need for a cerebral biopsy.

Unexpected Diagnosis of Cerebral Toxoplasmosis by 16S and D2 Large-Subunit Ribosomal DNA PCR and Sequencing

The protozoan parasiteToxoplasma gondiicauses severe opportunistic infections. Here, we report an unexpected diagnosis of cerebral toxoplasmosis.T. gondiiwas diagnosed by 16S and D2 large-subunit (LSU) ribosomal DNA (rDNA) sequencing of a cerebral biopsy specimen and confirmed byT. gondii-specific PCR and immunohistochemistry. The patient was later diagnosed with HIV/AIDS.