Tuncay Gündüz

Cognitive Impairment in Neuro-Behcet's Disease and Multiple Sclerosis: A Comparative Study

Both multiple sclerosis (MS) and neuro-Behcets disease (NBD) can cause a cognitive dysfunction mainly involving the executive functions. We conducted this study to clarify the probable differential cognitive/behavioral profiles of MS and NBD. Twenty consecutive cases with parenchymal NBD (13 male, seven female), and 20 cases with MS (five male, 15 female) were evaluated. Both groups had a thorough neurological examination; an evaluation for Expanded Disability Status Scale (EDSS), Multiple Sclerosis Functional Composite (MSFC), and Becks Depression Scale; and a detailed neuropsychological evaluation masked to the diagnosis. Among the two groups, male/female ratio differed significantly while other demographic and clinical features were not different. In California Verbal Learning Test, both short- and long-term delayed recall and cued recognition were worse in neuro-Behcets cases. They had impaired semantic clustering and increased false positives. Stroop Test was also more impaired in neuro-Behcets cases. They needed significantly more trials to complete the first category of the Wisconsin Card Sorting Test and had a poorer total Frontal Behavioral Inventory Score. Our results suggest that neuro-Behcets patients have a more severe “frontal”-executive dysfunction than MS patients.

Characteristics of isolated spinal cord involvement in neurobrucellosis with no corresponding MRI activity: A case report and review of the literature.

Brucellosis is the most common zoonotic infection that affects camels, sheep, and goats as primary hosts and humans as secondary hosts [1,2]. Brucellosis is transmitted through the consumption of uncooked meat or unpasteurized dairy products or by direct contact with tissue or blood from infected animals, and is more common in countries with poor public health [3]. According to the World Health Organization, N500,000 new cases occur each year worldwide [2]. Herein, we present an adolescent boy with isolated spinal cord involvement due to brucellosis with normal imaging.We also determined the clinical characteristics of similar cases through review of reports in the literature.

Neurobrucellosis presenting with mania

Brucellosis is caused by Brucella species (B. melitensis, B. abortus, B. suis) which are intracellular gram-negative bacteria. It is mainly transmitted by the ingestion of infected meat, unpasteurized dairy products, and close contact with the secretions of infected animals. Neurobrucellosis (NB) occurs in 2–7 % of brucellosis patients [1]. NB can affect the entire nervous system, including the meninges, vascular tree, brain and spinal cord parenchyma, and cranial and peripheral nerves, and thus, mimicking many other neurologic diseases. Some patients with NB may also present with obscure neuropsychiatric symptoms, which cause diagnostic difficulties. These include cognitive dysfunction, depression, and psychosis. However, brucellosis presenting with mania has never been reported before. Herein, we present a previously healthy male patient who was misdiagnosed as having bipolar disorder because of personality change and symptoms of mania. A 64-year-old male patient has been investigated in a local hospital because of weight loss, logorrhea, insomnia, hypersexuality, and fatigue, 4 years before his admission to our hospital. After a final diagnosis of mania, the patient was put on lithium treatment at that time. This treatment was stopped 6-months after initiation because of intractable nausea and vomiting, which was thought to be related to lithium intoxication at that time. Six months thereafter, the patient had two generalized tonic–clonic seizures. Levetiracetam 1000 mg/day was started after an electroencephalograph (EEG) that revealed slowing and focal epileptiform abnormalities in the left hemisphere. After losing 40 kg of body weight, the patient was admitted to the Department of Internal Medicine at Istanbul University due to intractable nausea and weight loss without any psychiatric symptoms. With the exception of mild anemia (hemoglobin 12.2 mg/dL), the patient’s routine blood tests including blood chemistry and erythrocyte sedimentation rate were unremarkable. A whole-body PET CT, screening for malignancy, gastric endoscopy, and colonoscopy were within normal limits. Infectious diseases such as syphilis, hepatitis, John Cunningham virus (JCV), human immunodeficiency virus (HIV), and Brucella were also investigated during his hospital stay. No evidence of Brucella was found after a negative Rose Bengal plate test. The patient’s brain magnetic resonance imaging (MRI) showed bilateral frontoparietal hyperintense white matter lesions in T2/FLAIR-weighted images without any contrast enhancement (Fig. 1a). The patient’s cerebrospinal fluid analysis (CSF) revealed 35 lymphocytes/mm with a protein level of 197 mg/dL (normal 15–40 mg/dL), and a glucose level of 56 mg/dL (normal 40–70 mg/dL). CSF oligoclonal bands were positive, and CSF cultures were negative. Tests for central nervous system vasculitis and genetic diseases for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), adult-onset leukodystrophies, including adrenoleukodystrophy, and metachromatic leukodystrophy were also negative. Subsequently, the patient was transferred to the Department of Neurology, without a specific diagnosis after an indecisive investigation. On admission to our clinic, the patient’s neurological examination revealed dysarthria, positive Kernig and Brudzinski signs, hyperactive deep tendon reflexes, and & Murat Kurtuncu murat.kurtuncu@istanbul.edu.tr

Prognostic factors in anti-neuronal antibody positive patients

Introduction Anti-neuronal antibodies (ANA) are found in paraneoplastic neurological syndrome and autoimmune encephalitis patients. Our aim was to analyze prognostic factors related with ANA seropositivity. Methods Twenty-seven consecutive ANA seropositive patients were included in the study. ANA were detected by immunofluorescent staining, immunoblot and cell-based assay methods. All patients were followed with a standard treatment protocol. Clinical syndromes, tumor types, modified Rankin scores, cranial MRI and oligoclonal band (OCB) findings were recorded. Cases were divided into subgroups due to clinical-laboratory features and ANA types. Prevalence of good prognosis, response to treatment and survival were compared among these subgroups. Results Patients showed antibodies to N-methyl-D-aspartate receptor (NMDAR) (6 cases), Hu (6 cases), Ma2 (5 cases), glutamic acid decarboxylase (GAD) (3 cases), Yo (3 cases), amphiphysin (1 case), gamma-amino butyric acid B receptor (GABABR) (1 case), Ri (1 case) and Zic4 (1 case). Associated neurological syndromes were limbic encephalitis (8 cases), subacute cerebellar degeneration (7 cases), brainstem encephalitis (5 cases), subacute sensory neuronopathy (4 cases), stiff-person syndrome (2 cases) and opsoclonus-myoclonus (1 case). A tumor (ductal breast, small cell lung cancer) was detected in six cases at first admission. Six patients died in an average follow-up time of 1.0±1.5 years. Detection of antibodies to extracellular or synaptic target antigens, but not presence of tumor, cranial MRI lesions or OCB, was associated with good prognosis and response to treatment. Conclusion NMDAR, Hu and Ma2-antibodies were the most prevalent ANA in this first antibody screening study in a Turkish cohort. Antibody type was determined to be the foremost prognostic factor in ANA seropositive cases.

Paraplegia following lumbar puncture: a rare complication in spinal dural arteriovenous fistula

Spinal dural arteriovenous fistula (SDAVF) is the most common vascular disorder in the spinal cord with an annual incidence of 5–10 per 1,000,000 [1]. The patients with SDAVF usually present with progressive paraparesis accompanied by urinary and bowel disturbance that can be misdiagnosed with a broad spectrum of relatively more common spinal disorders including autoimmune or infectious myelopathies and spinal tumors. Since cerebrospinal fluid analysis is essential in the differential diagnosis, most patients with SDAVF undergo lumbar puncture. However, this procedure may change the pressure of the cerebrospinal fluid (CSF) abruptly that may lower the perfusion pressure of the spinal cord causing further ischemia. Herein, we report a patient with SDAVF who developed paraplegia a few hours after lumbar puncture.