Emel Koseoglu

A 12-month study of the efficacy of rivastigmine in patients with advanced moderate Alzheimer's disease

The efficacy of a centrally active cholinesterase inhibitor, rivastigmine tartrate (ENA 713), in patients with advanced moderate Alzheimer’s disease (AD) was evaluated in a 12-month placebo-controlled study. We aimed to investigate whether there was any evidence for the benefits of rivastigmine in patients with severe disease. These patients were compared with matched controls. In this study, 24 patients with advanced moderate AD received rivastigmine for 12 months. Another 20 patients received placebo. Mean daily doses of rivastigmine in the higher-dose group at 3, 6, 9, and 12 months were 6.1 ± 1.0, 8.3 ± 1.2, 8.9 ± 1.3, and 10.7 ± 1.6 mg/day, respectively. Cognitive abilities were assessed using the 11-item cognitive subscale of the Alzheimer Disease Assessment Scale (ADAS-cog). Forty-five percent of placebo-treated patients declined by at least 4 points on the ADAS-cog. Conversely, only 18.3% of patients treated with rivastigmine declined by 4 or more points. Functional disabilities, as assessed using the Disability Assessment for Dementia Scale, remained significantly superior in rivastigmine-treated patients compared with placebo-treated patients. Patients benefited from high-dose rivastigmine treatment on all outcome measures, including the Mini-Mental State Examination, Progressive Deterioration Scale, as well as the Global Deterioration Scale. Patients receiving rivastigmine for 12 months significantly improved compared with placebo-treated patients (p < 0.001). By 52 weeks, patients originally treated with 6–12 mg/day rivastigmine had a significantly better cognitive function than patients originally treated with placebo. Long-term rivastigmine treatment appeared to be well tolerated in patients with advanced moderate AD and significantly benefits the cognitive and functional symptoms of AD.

Audio-vestibular disturbances in Behcet's patients : report of 62 cases

UNLABELLED This study was carried out to determine the characteristics and incidence of hearing loss and vestibular disturbance in Behcets syndrome with a large number of patients. Sixty-two patients with Behcets syndrome were included in this study, 34 men and 28 women whose mean age was 33.7 (15-60). Sixty-two healthy normal control subjects (38 male and 24 female) were included. Patient and control groups were questioned about any history of audio-vestibular disturbance and underwent physical and ENT examination and the following audiologic tests: pure tone audiometric test (0.25, 0.5, 1, 2, 4, and 6 kHz), tympanogram, speech discrimination, short increment sensitivity index, tone-decay test, auditory brainstem response. Vestibular system was evaluated by videonistagmogram and caloric test. Cranial and brainstem magnetic resonance imagine (MRI) of patients who have vestibular disturbances were practiced to examine the central nervous system. Both the patient and the control groups were tested with the HLA-B51 antigen. Pure tone audiogram showed sensory-neural hearing loss (25 dB hearing level in at least two frequencies) in 20 of the 62 (32%) Behcets patients while the control group were normal. There was a hearing loss involving high frequencies in the audiograms of Behcets patients with hearing disturbances. The recruitment investigation tests and auditory brain stem response confirmed cochlear involvement in all 20 patients. Caloric stimulation tests revealed a normal vestibular function in all patient and control group. In electronystagmography, 21 (34%) patients had hypometric or hypermetric saccades and smooth pursuit tests showing that 4 (6%) patients had pathological changes while the control group was normal. HLA-B51 antigen was found positive in 15 of 20 Behcets patient with hearing loss. CONCLUSION (1) The hearing and vestibular disturbances in Behcets syndrome is more prevalent than previously recognized; (2) Hearing loss in high frequencies in Behcets patients is an indicator of cochlear involvement in this disease; (3) There is a higher prevalence of central vestibular syndrome in Behcets patients than it was thought before; (4) HLA-B51 antigen may be able to be a prognostic factor for sensorineural hearing loss in Behcets patients.

Is Toxoplasma gondii a causal agent in migraine

Background:Many different tissues may be parasitized by Toxoplasma gondii, particularly, lung, heart, lymphoid organs, and the central nervous tissues. Tissue cysts of this parasite in the brain may spontaneously rupture, releasing parasites that cause antibody titers to rise. In immunocompetent subjects with acquired toxoplasmosis, the most frequent symptoms were lymphadenopathy and headache. In the neurogenic inflammation theory of the pathogenesis of migraine, the cause of initial release of ions and inflammatory agents has not been established. In this study, we aimed to investigate if T. gondii infection is a possible cause of neurogenic inflammation of migraines. Methods:The anti–T. gondii antibody status of 104 patients with migraine were studied and compared with those of control groups, 50 healthy subjects and 50 subjects with headache due to rhinosinusitis, by using a micro–enzyme-linked immunosorbent assay technique. Results:Forty-six (44.2%) patients with migraine, 13 (26.0%) healthy control subjects, and 12 (24%) control subjects with rhinosinusitis were positive for anti–T. gondii IgG antibody. The rate of positivity in the migraine patient group was statistically different from those of the control groups (P < 0.05). Conclusions:The results show the presence of chronic Toxoplasma infection in patients with migraine. Toxoplasma infection may contribute to neurogenic inflammation as the pathogenesis of migraine, as many studies in the literature have reported that Toxoplasma infection causes biochemical and immunologic changes.

SUNCT syndrome associated with compression of trigeminal nerve

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) represents a brief headache syndrome first described in 1989. Its name summarizes the clinical features: short-lasting, unilateral, neuralgiform headache attacks, with conjunctival injection, tearing and rhinorrhoea (1). Although usually occurring without demonstrable structural abnormalities, a small number of potentially symptomatic cases of SUNCT syndrome have been reported with pathologies of the cerebellopontine angle (2–4), brain stem (5–7) and cavernous sinus (4, 8). Additionally, the relationship between SUNCT syndrome and trigeminal neuralgia is an interesting clinical question and an important physiological issue in the literature (9). We describe a symptomatic case of SUNCT syndrome with compression of the trigeminal nerve by the anterior inferior cerebellar artery in the prepontine cistern. To our knowledge, this has not been previously described.

Factors that affect interictal cardiovascular autonomic dysfunction in temporal lobe epilepsy: Role of hippocampal sclerosis

The aim of this study was to evaluate possible factors affecting interictal cardiovascular autonomic function in temporal lobe epilepsy with complex partial seizures, paying special attention to hippocampal sclerosis. The study was carried out with 88 patients with epilepsy (22 with left hippocampal sclerosis, 22 with right hippocampal sclerosis, and 44 without hippocampal sclerosis) and 44 healthy subjects. All subjects underwent three tests of cardiac autonomic function: heart rate variation during resting activity, heart rate variation in response to deep breathing and blood pressure response to rising quickly from the supine position. Hippocampal sclerosis and disease duration were found to have significantly important effects on parasympathetic autonomic function, whereas seizure control and type of antiepileptic drug had significant effects on sympathetic autonomic function. This study shows that in addition to factors related to the chronic nature of epilepsy and antiepileptic drug use, hippocampal sclerosis may cause autonomic dysfunction during the interictal period in persons with temporal lobe epilepsy.

Trace metal concentrations in hair and nails from Alzheimer's disease patients: Relations with clinical severity.

BACKGROUND AND OBJECTIVES Metals, especially transition metals, seem to be important in the pathogenesis of Alzheimer disease. This study aims to determine the relationship of trace metal elements to the pathogenesis and/or course of Alzheimer Disease in terms of clinical severity. METHODS The hair and nail trace metal levels of 62 Alzheimer Disease patients at different clinical stages (21 mild, 20 moderate, 21 severe) and 60 healthy control subjects were measured by using inductively coupled plasma-mass spectrometry. The statistical comparisons were performed with regards to the study groups, clinical stages, disease duration and age. RESULTS The patient and control groups were significantly different from each other in regards to Mn, Fe, Cu, Cd, Hg (p<0.001), Zn (p<0.01) in nail concentrations and, Na, Al, Pb, Co (p<0.001), Fe, Mn (p=0.001), Hg, Cu, Cd, K in hair concentrations (p<0.01). No difference was detected in the levels of Mg and Ca. Nail Na level showed differences among different clinical stages of the disease (p<0.01). In comparing the mild degree Alzheimer patients to the control group; significant differences were detected in nail Mn, Fe, Cu, Co (p<0.001), Hg, Zn (p<0.01) and, hair Pb, Al (p<0.001), Na, K levels (p<0.01). CONCLUSIONS Our results have shown that transition and posttransition metals are especially important metals for the disease process. The relation of nail Na level with clinical stages of AD is an interesting new finding, making someone to think that alkali metals may be important in the progression of the disease.

Transverse myelitis and acute motor sensory axonal neuropathy due to Legionella pneumophila: A case report

Guillain–Barré syndrome is a rapidly progressive symmetrical muscle weakness associated with acute inflammatory disease. Transverse myelitis (TM) is the inflammation of the spinal cord characterized by rapidly evolving muscle weakness in the lower extremities, defects in sensory level and sphincter dysfunction. Guillain–Barré syndrome, and TM association occurs very rarely in childhood. A 7‐year‐old girl presented with complaints of neck pain, spout‐style vomiting, cough, shortness of breath, and acute paraparesis with sensory and sphincter disturbance. The patient was intubated because of increased respiratory distress. A positive direct fluorescein antigen test in bronchoalveolar lavage confirmed Legionella pneumophila infection. Imaging and neurophysiologic studies were diagnostic for TM with acute motor and sensory axonal neuropathy. She was treated with a combination of high‐dose methylprednisolone and intravenous immunoglobulins, and we observed incomplete recovery. The presented case is the first child with concomitant TM and acute motor and sensory axonal neuropathy related to L. pneumophila infection.

A database for screening and registering late onset Pompe disease in Turkey

The aim of this study was to search for the frequency of late onset Pompe disease (LOPD) among patients who had a myopathy with unknown diagnosis registered in the pre-diagnostic part of a novel registry for LOPD within a collaborative study of neurologists working throughout Turkey. Included in the study were 350 patients older than 18 years who have a myopathic syndrome without a proven diagnosis by serum creatine kinase (CK) levels, electrodiagnostic studies, and/or muscle pathology, and/or genetic tests for myopathies other than LOPD. Acid alpha glucosidase (GAA) in dried blood spot was measured in each patient at two different university laboratories. LOPD was confirmed by mutation analysis in patients with decreased GAA levels from either both or one of the laboratories. Pre-diagnostic data, recorded by 45 investigators from 32 centers on 350 patients revealed low GAA levels in a total of 21 patients; from both laboratories in 6 and from either one of the laboratories in 15. Among them, genetic testing proved LOPD in 3 of 6 patients and 1 of 15 patients with decreased GAA levels from both or one of the laboratories respectively. Registry was transferred to Turkish Neurological Association after completion of the study for possible future use and development. Our collaborative study enabled collection of a considerable amount of data on the registry in a short time. GAA levels by dried blood spot even from two different laboratories in the same patient may not prove LOPD. LOPD seemed to be rarer in Turkey than in Europe.

Nasu Hakola Disease: A Rare Cause of Dementia and Cystic Bone Lesions, Report of a New Turkish Family

The differential diagnosis of young-onset progressive dementia is an issue that requires effort. Recording the family history and careful clinical evaluation are useful tools in the diagnosis. In case of genetic bases, definitive diagnosis requires molecular analysis. We report consanguineous two patients presenting with young-onset progressive dementia characterized by behavioral changes and with bone cysts. Concomitant bone pathology and inheritance pattern directed us to investigate TREM2 gene, for differential diagnosis, which resulted with the identification of a causative mutation that confirmed the diagnosis of Nasu Hakola disease. The mutation (c.113A>G) is the same for a Turkish family with Nasu Hakola disease reported before. But the presence of bone cysts and absence of epilepsy in our patients are the different findings. Molecular analysis should be considered in patients with young age onset behavioral and cognitive deficits, with white matter lesions in brain magnetic resonance imaging, if especially associated with cystic bone lesions.