Baris Topcular

The Effects of Cognitive Rehabilitation on Relapsing Remitting Multiple Sclerosis Patients

INTRODUCTION The aims of this study were to determine the severity of cognitive impairment with Brief Repeatable Battery Neuropsychology (BRB-N) and to show the benefits of cognitive rehabilitation programs to develop coping strategies for the retardation of cognitive losses in relapsing remitting multiple sclerosis (RRMS) patients. METHODS The cognitive screening with BRB-N was performed in RRMS patients who had applied to the MS outpatient clinic of Bakırköy Psychiatric and Neurological Diseases Hospital, had an Expanded Disability Status Scale (EDSS) score between 1.0 and 5.5, and had no other cognitive disease or used drugs that effected the cognitive status. Thirty two patients with cognitive impairment underwent consecutive computer-assisted cognitive rehabilitation program once a week for 8 weeks conducted by psychologists. The effects of the program were evaluated with the tests repeated every 4 weeks. RESULTS The mean age of the patients was 36.09±7.19 years. Their initial and control test scores were found to be paced auditory serial addition test (PASAT) 29.21±17.97; 42.43±15.43, Selective Reminding Test-Total Learning (SRT-TL) 8.03±2.36; 10.09±1.77, Selective Reminding Test-Long-term Memory (SRT-DR) 6.72±2.74; 9±2.29, Spatial Recall Test-Total Learning (SPART-TL) 4.72±2.02; 4.22±1.74, Spatial Recall Test-Long-term Memory (SPART-DR) 5.94±2.54; 5.16±2.23, Symbol Digit Modalities Test (SDMT) 40.44±17.04; 46.47±17.94, Word Fluency Test (WLG) 32.88±9.87; 40.44±9.95 respectively. There was no significant statistical difference between Multiple Sclerosis Neuropsychological Screening Questionnaire (MSNQ) and SPART values of preface, 1st control and 2nd control (p>0.05). There was significant statistical difference between PASAT, WLG, SDMT, SRT-TL, SRT-DR values of Preface, 1st control, and 2nd control (p=0.0001). CONCLUSION It was determined that on distinctive improvement on sustaining attention, working memory and information processing speed, and verbal fluency and verbal learning with cognitive rehabilitation, no changes were observed on visuospatial learning. This approach is hopeful and further comprehensive studies are needed.

Holmes’ Tremor with Shoulder Pain Treated by Deep Brain Stimulation of Unilateral Ventral Intermediate Thalamic Nucleus and Globus Pallidus Internus

A 21-year-old male was admitted with severe right arm and hand tremors after a thalamic hemorrhage caused by a traffic accident. He was also suffering from agonizing pain in his right shoulder that manifested after the tremor. Neurologic examination revealed a disabling, severe, and irregular kinetic and postural tremor in the right arm during target-directed movements. There was also an irregular ipsilateral rest tremor and dystonic movements in the distal part of the right arm. The amplitude was moderate at rest and extremely high during kinetic and intentional movements. The patient underwent left globus pallidum internus and ventral intermediate thalamic nucleus deep brain stimulation. The patient improved by more than 80% as rated by the Fahn-Tolosa-Marin Tremor Rating Scale and Visual Analog Scale six months after surgery.

Chronic inflammatory demyelinating polyradiculoneuropathy associated intracranial hypertension

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated demyelinating neuropathy. In this report, we detail the course of a 58-year-old male patient who had headache and double vision followed by progressive paresthesia and difficulty in walking. The patient had bilateral papilledema and mild leg weakness, absent ankle jerks and loss of sensation in distal parts of his lower and upper extremities. His electromyography (EMG) was concordant with CIDP and lumbar puncture revealed high opening pressure. The polyradiculoneuropathy as well as the papilledema and elevated cerebrospinal fluid (CSF) pressure improved under steroids. The improvement in intracranial hypertension (IHT) and papilledema under steroid treatment suggests that the IHT in this patient might be associated with CIDP.

IMPULSIVITY IN ALZHEIMER’S DISEASE AS DETECTED BY D-CPT

Background: Genetic studies identified an association between Alzheimer’s disease (AD) and common polymorphisms in the MS4A and TREM loci, each containing cluster of homologous genes. Methods:We searched for rare coding variants in 15 genes mapped to these loci by next generation sequencing of a North American dataset (210 cases and 233 controls). Results:Analysis of the MS4A gene-cluster revealed loss-of-function variants in 6 controls and 3 cases. Investigation of the TREM gene-cluster detected known AD associated TREM2 substitutions (p.R47H, p.D87N and p.H157Y) affecting both TREM2 isoforms (NM_018965 and NM_001271821). We also identified two cases with novel TREM2 variants (p.L205P and p.G219C), which mapped only to the isoform NM_001271821. A p.S248R substitution in the homologous TREML2 gene was detected in 5 controls and 1 case suggesting a protective effect (pooled p-value 1⁄4 0.033). Conclusions:Our study advocates for the importance of mutation analysis of controls, particularly for GWAS loci containing SNPs with a minor allele frequency higher in controls versus cases (e.g. MS4A locus), to search for functional variants with a protective effect.

Neuromyelitis optica and neuromyelitis optica spectrum disorders: the evaluation of 86 patients followed by Istanbul Bilim University, Department of Neurology

Background and objectives: Neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) are relatively rare disorders when compared to multiple sclerosis (MS). We aimed to evaluate clinical characteristics and disease course of the NMOSD patients followed at our department. Patients and methods: All the patients with the diagnosis of NMO/NMOSD followed since the establishment of our MS clinic in April 2011, were evaluated. Results: There were 70 patients (54 female, 16 male) with NMO/NMOSD followed at our MS unit; 22 had NMO, 32 had recurrent optic neuritis (RON); and 16 had longitudinally extensive transverse myelitis (LETM). The mean age of the patients was 41±14 (12-77) years. The disease duration was 4.5±4.7 years. The disease course was relapsing in 54 patients (77%). The first attack was bilateral ON (BON) and TM in 3 patients, ON and TM in 1 patient, ON in 44 patients (bilateral in 4) and TM in 22 patients. The mean of the duration between the first two relapses was 13.4±18.4 months. The mean EDSS score was 2.7±1.7 at the last visit. NMO IgG was positive in 12 patients with NMO (55%), 4 patients with LETM (25%), and 7 patients with RON (22%). Oligoclonal band was positive in 13 out of 36 patients (4 each with NMO and ON, two with ON and TM and three with LETM; 34%). In NMO/NMOSD patients, cranial magnetic resonance imaging (MRI) showed no abnormality in 51; nonspecific lesions in 28; and 1 patient had hypothalamic lesion. In spinal MRIs, 35 patients had LETM; four had suspected hyperintense T2 lesion in C5. Conclusion: This is one of the largest single center series collected over 2 years. NMO/NMOSD seems to be over-represented in our center since it is one of the few where NMO IgG testing is available. In NMO/NMOSD, early diagnosis and treatment, as well as differentiation from MS, is important to prevent the patient from the permanent disability. Disclosure: Dr. Altunrende has nothing to disclose. Dr. Altinkaya has nothing to disclose. Dr. Topcular has nothing to disclose. Dr. Meryem has nothing to disclose. Dr. Server has nothing to disclose. Dr. Firtina has nothing to disclose. Dr. Yenice has nothing to disclose. Dr. Akman-Demir has nothing to disclose.

Cognitive impairment in neurosyphilis: Alzheimer's disease-like pattern?

assessed, performance of both constructive and gestural praxias was impaired. Conclusions: The PCA and within it, the Biparietal syndrome, occurs with failures in the visuospatial abilities and apraxia and has been linked to AD in up to 100% in some reports confirmed by pathology. A great degree of heterogeneity in clinical presentations of AD exists and memory can be spared even into advanced stages of the disease. Probably because of these reports clinical criteria for AD have been modified and therefore, considering the possible emergence of disease-modifying drugs, awareness of this kind of atypical presentations is important, in order to make an early diagnosis and an adequate treatment.