Prevalence of Multiple Sclerosis in the Middle Black Sea Region of Turkey and Demographic Characteristics of Patients.

INTRODUCTION: Multiple sclerosis (MS), a demyelinating disease of the central nervous system, is increasing in prevalence worldwide. Other than small-scale studies, there is a scarcity of data on the prevalence of MS in Turkey. The present study aimed to elucidate the prevalence of MS in the Middle Black Sea Region and the demographic characteristics of patients. METHODS: Patients living in the Middle Black Sea Region and who were diagnosed with MS were included. The study was designed based hospital of Turkey. RESULTS: A total of 1,787 patients were interviewed, and a diagnosis of MS was confirmed in 1,584. The prevalence of MS was found to be 43.2/100,000 in the Middle Black Sea Region; this was calculated based on the total population and number of patients in the provinces and districts in this region. The mean age at the disease onset was 29.39±7.6 years. Among the patients, 1,299 (81.9%) had relapsing-remitting MS, 74 had secondary progressive MS, 77 had relapsing-progressive MS, 47 had primary progressive MS, and 87 had clinically isolated syndrome. CONCLUSION: The Middle Black Sea Region was found to have a high risk for the prevalence of MS. This study is the most comprehensive epidemiologic study having the largest geographical distribution on the prevalence of MS in Turkey.

The relationship between familial Mediterranean fever gene (MEFV) mutations and clinical and radiologic parameters in multiple sclerosis patients.

OBJECTIVE: Central nervous system (CNS) involvement in patients with familial Mediterranean fever (FMF) is considerably rare. Patients with FMF may exhibit clinical and radiologic symptoms similar to multiple sclerosis (MS). However, the impact of the Familial Mediterranean Fever Gene (MEFV) mutations on the clinical course of MS is not fully understood as yet. METHODS: In our study, we investigated the presence of probable MEFV mutations in patients diagnosed with definite MS and the association of these mutations with the clinical course, radiologic characteristics and disability status of the individuals. A total of 105 patients diagnosed with definite MS according to the McDonald criteria and a control group of 112 non-symptomatic individuals were included in the study. RESULTS: Thirty-seven patients (35.2%) had MEFV gene mutations; three were compound heterozygotes (M694V/E148Q; M694V/V726A; P369S/E148Q) and one was homozygous for P369S. No statistically significant differences were found among patients with MS and healthy individuals with respect to existing mutations. In addition, we did not observe a statistically significant relationship between MEFV mutations and the gender of the patients, oligoclonal band (OCB) positivity, Expanded Disability Status Scale (EDSS), disease onset age, clinical presentation, affected neurologic systems, existence of spinal lesions, response to immunomodulatory treatment, time to reach EDSS scores of 3 and 6, the number of attacks and the average number of lesions on a brain MRI. CONCLUSION: Our results indicate that MEFV gene mutations do not affect the neurologic prognosis in patients with MS. However, additional research studies involving more patients with MS and clinical forms are warranted to confirm our results.

Ultrasonographic findings in hereditary neuropathy with liability to pressure palsies.

OBJECTIVES: The aims of this study were to evaluate the sonographic findings of patients with hereditary neuropathy with liability to pressure palsies (HNPP) and to examine the correlation between sonographic and electrophysiological findings. METHODS: Nine patients whose electrophysiological findings indicated HNPP and whose diagnosis was confirmed by genetic analysis were enrolled in the study. The median, ulnar, peroneal, and tibial nerves were evaluated by ultrasonography. RESULTS: We ultrasonographically evaluated 18 median, ulnar, peroneal, and tibial nerves. Nerve enlargement was identified in the median, ulnar, and peroneal nerves at the typical sites of compression. None of the patients had nerve enlargement at a site of noncompression. None of the tibial nerves had increased cross-sectional area (CSA) values. There were no significant differences in median, ulnar, and peroneal nerve distal motor latencies (DMLs) between the patients with an increased CSA and those with a normal CSA. In most cases, there was no correlation between electrophysiological abnormalities and clinical or sonographic findings. DISCUSSION: Although multiple nerve enlargements at typical entrapment sites on sonographic evaluation can suggest HNPP, ultrasonography cannot be used as a diagnostic tool for HNPP. Ultrasonography may contribute to the differential diagnosis of HNPP and other demyelinating polyneuropathies or compression neuropathies; however, further studies are required.

Validation of self report version of the Leeds Assessment of Neuropathic Symptoms and Signs score for identification of neuropathic pain in patients from northern Turkey.

BACKGROUND: Diagnosis and treatment of neuropathic pain is an important clinical problem. OBJECTIVES: A self report version of the Leeds Assessment of Neuropathic Symptoms and Signs (S-LANSS) score provides identification of neuropathic pain without the help and need of a clinician. We targeted validation of the S-LANSS score in the northern Turkish population in this study. MATERIAL AND METHODS: For the linguistic validation of S-LANSS, translation and back-translation method was used to adapt S-LANSS into Turkish and a cognitive-debriefing test was performed. A total of 148 patients were enrolled in the present study. S-LANSS, The Leeds Assessment of Neuropathic Symptoms and Signs (LANSS), Visual Annalogue Score (VAS) and Neuropathic Pain Questionnaire (NPQ) were performed twice for every patient. The patients were examined and diagnosed as having nociceptive or neuropathic pain by neurologists, who were blind for S-LANSS, LANSS and NPQ scores of the patients. RESULTS: Results of the McNemar test indicated that S-LANSS scores were reliable when the first and the second scores were compared. The sensitivity and specificity of the scale were found to be 98% and 97% respectively. CONCLUSIONS: We believe that using S-LANSS scores for the diagnosis of neuropathic pain may help our colleagues as a tool for a quicker differential diagnosis of pain in daily practice.

Clinical REM sleep behavior disorder and motor subtypes in Parkinson's disease: a questionnaire-based study.

OBJECTIVES: Studies documenting the association between rapid eye movement sleep behavior disorder (RBD) and motor subtypes in Parkinson's disease (PD) are rare. Our hypothesis is that RBD may be more severe in non-tremor dominant (NTD) patients with RBD than those tremor dominant (TD) with RBD. In this study, we investigated the association between motor subtypes and clinical RBD in PD. PATIENTS AND METHODS: We evaluated 104 consecutive patients older than 18 years presenting with PD to the Neurology Clinic of the University Hospital for one year in this study. The clinical diagnosis of RBD was based on the minimal diagnostic criteria of International Classification of Sleep Disorders, revised. The Stavanger Sleepiness Questionnaire was used to rate the severity of clinical RBD. The patients were divided into two subgroups as TD and NTD. The patient and control groups were compared with each other for severity and frequency of clinical RBD, and the Unified Parkinson's Disease Rating Scale (UPDRS) and Hoehn-Yahr stage scores. The correlation between severity of clinical RBD and clinical severity of PD was analyzed in the patient groups. RESULTS: Of the patients, 45.2% (n=47) had the NTD subtype of PD and 54.8% (n=57) had the TD subtype of PD. There was no significant difference among the groups in terms of frequency and severity of clinical RBD. For the NTD patients, there was a weak positive correlation between severity of clinical RBD and clinical severity of PD. However, there was no correlation in the TD subgroup. CONCLUSION: In our study, frequency of clinical RBD was unrelated to motor subtypes of PD. However, in the present study, we found a weak correlation between clinical severity (UPDRS and the Hoehn-Yahr) of PD and severity of clinical RBD in the NTD subtype but not in the TD subtype.

Phenobarbital-induced pellagra resulted in death.

Pellagra is caused by deficiency of niacin or its precursor tryptophan. While cutaneous lesions are the most prominent feature of the disease, gastrointestinal, neurological and psychiatric signs and symptoms are the other characteristics of the disease. In this case report, we present a 29-year-old female patient with discoloration of hands and feet diagnosed with pellagra.

Does probe's eye subthalamic nucleus length on T2W MRI correspond with microelectrode recording in patients with deep brain stimulation for advanced Parkinson's disease?

AIM: Subthalamic nucleus (STN) deep brain stimulation (DBS) has become a well-accepted treatment for patients with advanced Parkinson's disease (PD). During surgical planning for DBS, the length of the STN is taken into account and verified during microelectrode recording (MER) intraoperatively. Here, we addressed the question to which extent the length of the STN measured with the T2 weighted MRI in the probe's eye view corresponded with the intraoperatively determined length of the STN with MER. MATERIAL AND METHODS: We included 10 consecutive Parkinson's disease patients who underwent STN DBS surgery. The length of the STN in the probe's eye view mode was calculated along the trajectory of the central MER electrode crossing the STN. RESULTS: Our analysis showed no statistical difference between the length of the STN measured with the T2 weighted probe's eye view mode and the MER (right STN length 5.8 ± 0.9 mm MRI vs. 6.3 ± 0.5 mm MER, p > 0.05; left STN length 5.6 ± 0.4 mm MRI vs 5.8 ± 1 mm MER, p > 0.05). CONCLUSION: This means that the entry and the exit of the STN can be adequately estimated using the probe's eye view preoperatively.

Syncope associated with subthalamic nucleus deep brain stimulation in a patient with Parkinson's disease.

In advanced Parkinson's disease (PD), deep brain stimulation (DBS) may be an alternative option for the treatment of motor symptoms. Side effects associated with subthalamic nucleus (STN) DBS in patients with PD are emerging as the most frequent sensory and motor symptoms. DBS-related syncope is reported as extremely rare. We wanted to discuss the mechanisms of syncope associated with STN DBS in a patient with Parkinson's disease. Case report. Sixty-three-year-old female patient is followed up with diagnosis of idiopathic Parkinson's disease for 6 years in our clinic. The patient has undergone STN DBS due to painful dystonia and drug resistant tremor. During the operation, when the left STN was stimulated at 5 milliampere (mAmp), the patient developed presyncopal symptoms. However, when the stimulation was stopped symptoms improved. During the early period after the operation, when the right STN was stimulated at 1.3 millivolts (mV), she developed the pre-yncopal symptoms and then syncope. Our case shows that STN DBS may lead to directly autonomic symptoms resulting in syncope during stimulation-on (stim-on).

Is symptomatic atherosclerotic cerebrovascular disease a risk factor for normal-tension glaucoma?

OBJECTIVE: To compare the incidence of glaucomatous optic disk appearance between patients with symptomatic atherosclerotic stroke and healthy individuals with normal intraocular pressures (IOP). SUBJECTS AND METHODS: 46 patients with ischemic stroke with evident lacunar infarction or large vessel atherosclerosis, and 93 age- and sex-matched healthy individuals, all with normal IOP, were included. Patients and controls were examined for the presence of high cup-to-disk ratios (> 0.5). RESULTS: Seven patients (15.22%) in the ischemic cerebrovascular disease (CVD) group and 3 controls (3.23%) had glaucomatous optic disk appearance. All subjects with glaucomatous optic disk appearance in the control group and 3 patients in the study group had visual field defects in concordance with normal-tension glaucoma (NTG). The incidence of glaucomatous optic disk appearance was significantly higher in the group with symptomatic atherosclerotic CVD. CONCLUSION: Atherosclerotic CVD is a risk factor for having glaucomatous optic disk appearance. Symptomatic atherosclerosis involving the brain vasculature may also affect the eye and lead to NTG. Patients with ischemic strokes due to large artery atherosclerosis or small artery occlusion must be examined and followed for NTG.

Visual evoked potentials in guillain-barré syndrome.

BACKGROUND AND PURPOSE: Guillain-Barré syndrome (GBS) is an acute demyelinating polyneuropathy with various clinical features. Optic neuritis occurs in rare cases. In this study we determined the incidence and patterns of visual evoked potential (VEP) abnormality in GBS in association with ophthalmologic findings. METHODS: Thirty-two patients with a diagnosis of GBS were included in the study. The correlation between pathologic VEPs and categories of neurologic deficit and electrophysiological findings were examined statistically. RESULTS: The patients ranged in age from 19 to 77 years. Five cases (16%) had abnormal VEPs. All five of these patients exhibited increased P100 latency differences between the two eyes. Other abnormalities were prolonged p100 latency, increased interocular amplitude difference, and distorted p100 configuration. Pathologic signs on ophthalmologic examination were observed in 80% of patients with abnormal VEPs. VEP abnormality was never present in pure axonal forms. There was no significant correlation between pathologic VEP and cerebrospinal fluid protein level or categories of neurologic deficits. CONCLUSIONS: Involvement of the optic pathways is not a frequent finding in GBS. When present it is always asymmetric and generally accompanied with pathologic findings on ophthalmologic examination. VEPs may be abnormal in different clinical variants of GBS, and especially in demyelinating forms.

Examination of the relationship between average plaque volume and clinical and demographic characteristics in multiple sclerosis patients using a stereological method.

AIM: The objectives of this study were to investigate the lesion load on brain magnetic resonance images and the volume of different cerebral anatomical structures, in patients with a diagnosis of definite multiple sclerosis, using a stereological method. METHODS: Fifty patients diagnosed with multiple sclerosis were included in the study. The volume fractions of the hemispheres, lateral ventricles, cerebellum, brain stem, and plaque volumes within the total brain volume were estimated. RESULTS: The mean plaque volume was 0.83 ± 0.14 cm(3) in the cerebellum and 1.14 ± 0.2 cm(3) in the brain stem. There was a significant relationship between left hemisphere volume to whole hemisphere ratio and ventricular volume (p = .034, r = 0.1) in patients with cerebellar lesions compared with patients without cerebellar lesions. Similarly, there was a significant correlation between left hemisphere volume ratio and ventricular volume ratio in all patients (r = -0.286, p = .044). CONCLUSION: These results show that there is significantly greater atrophy in the left hemisphere. In addition, the presence of cerebellar lesions can trigger or accelerate the development of left hemisphere atrophy. Thus, this finding may be an indicator of poor prognosis.

Does long term use of piracetam improve speech disturbances due to ischemic cerebrovascular diseases?

Aphasia causes significant disability and handicap among stroke survivors. Language therapy is recommended for aphasic patients, but not always available. Piracetam, an old drug with novel properties, has been shown to have mild beneficial effects on post-stroke aphasia. In the current study, we investigated the effects of 6 months treatment with piracetam on aphasia following stroke. Thirty patients with first-ever ischemic strokes and related aphasia were enrolled in the study. The scores for the National Institutes of Health Stroke Scale (NIHSS), Barthel Index (BI), modified Rankin Scale (mRS), and Gülhane Aphasia Test were recorded. The patients were scheduled randomly to receive either 4.8 g piracetam daily or placebo treatment for 6 months. At the end of 24 weeks, clinical assessments and aphasia tests were repeated. The level of improvement in the clinical parameters and aphasia scores was compared between the two groups. All patients had large lesions and severe aphasia. No significant difference was observed between the piracetam and placebo groups regarding the improvements in the NIHSS, BI and mRS scores at the end of the treatment. The improvements observed in spontaneous speech, reading fluency, auditory comprehension, reading comprehension, repetition, and naming were not significantly different in the piracetam and placebo groups, the difference reached significance only for auditory comprehension in favor of piracetam at the end of the treatment. Piracetam is well-tolerated in patients with post-stroke aphasia. Piracetam taken orally in a daily dose of 4.8 g for 6 months has no clear beneficial effect on post-stroke language disorders.

Pure sensory stroke due to bilateral basal ganglion hemorrhage: a case report.

Bilateral simultaneous hypertensive intracerebral hemorrhages are extremely rare. The predisposing factors and pathophysiological mechanisms leading to the development of this picture are not well known. Possible mechanisms of simultaneous multiple hemorrhages include concomitant primary hemorrhages in two or more regions, or development of a second hemorrhage in another region shortly after the primary hemorrhage. The etiology of the cases presenting with bilateral simultaneous basal ganglion hemorrhage include migraine, lightning stroke, hyperglycemic hyperosmolar coma, hypertension and diabetic ketoacidosis coma. Bilateral simultaneous hemorrhage has a poor prognosis. The case of bilateral simultaneous intracerebral hemorrhage presented here had a good clinical course similar to a pure sensorial stroke.

Changes in bone mineral density and bone metabolism markers in premenopausal women with multiple sclerosis and the relationship to clinical variables.

Bone mineral density (BMD) is affected in young adults with multiple sclerosis (MS), which leads to disabling disease. We aimed to show changes that were independent of immobilization by measuring BMD and laboratory markers of bone metabolism in mobile MS patients. We compared a total of 52 premenopausal female patients with relapsing-remitting multiple sclerosis (RRMS) to 41 women of similar age who had no risk factors for osteoporosis. The lumbar and femur BMD were measured using the dual energy X-ray absorptiometry (DXA) method. The urine concentration of serum 25-hydroxycholecalciferol (25-OH vit D(3)), and pyridinoline and deoxypyridinoline were also measured. The concentration of serum osteocalcin was measured to determine the speed of bone metabolism. The mean age of patients (+/- standard deviation [SD]) was 36.1+/-7.4. The average Expanded Disability Status Scale (EDSS) score was 2.2+/-1.8. The concentration of 25-OH vit D(3) and osteocalcin was lower, whereas the concentration of parathyroid hormone (PTH), alkaline phosphatase (ALP), pyridinoline and deoxypyridinoline was higher in the patient group. In the patient group, lumbar 2-4 BMD, T score and Z score and femur neck and trochantor BMD, T score and Z score were significantly lower than in the control group. There was a significant negative relationship between: the disease period and L 2-4 BMD, T score and Z scores; and the femoral neck BMD, T score and Z scores. There was a significant relationship between the total Functional Independence Measure score and the femoral neck, femoral trochanter BMD, T score, and Z score. There was a significant negative relationship between the average EDSS, L 2-4 and all the DXA measurements obtained from the femur. There was a significant relationship between the 25-OH vit D(3) concentration and L 2-4 T score and Z score from the DXA measurements obtained from the femur. There were no significant relationships between osteocalcin, pyridinoline, deoxypyridinoline levels and the BMD measurements. Therefore, the duration of the disease and decrease in functional capacity are the main factors that affect BMD in MS. Apart from the decrease in functional capacity, 25-OH vit D(3) deficiency and secondary PTH increase contribute to the BDM changes observed in MS.

Manifestation of Lambert-Eaton myasthenic syndrome during last trimester of pregnancy.

Lambert-Eaton myasthenic syndrome (LEMS) is a rare immune-mediated disorder of neuromuscular junctions. The knowledge of the effects of pregnancy on the course of patients with LEMS is limited. Here we describe a patient without a history of previous known illness who has complained of weakness during the last trimester of her pregnancy, delivered a healthy baby and was diagnosed with non-paraneoplastic LEMS during the postpartum period. With this case we wanted to emphasize the effects of pregnancy on the course of patients with LEMS.

Electrophysiological assessment of polyneuropathic involvement in rheumatoid arthritis: relationships among demographic, clinical and laboratory findings.

OBJECTIVES: The aims of this study were to electrophysiologically evaluate polyneuropathy in rheumatoid arthritis (RA) patients and to examine the relationships among polyneuropathy and demographic, clinical and laboratory findings. PATIENTS AND METHODS: Sixty consecutive patients (51 women and nine men) with a clinical diagnosis of RA were examined electrophysiologically for the evidence of polyneuropathy. Parameters including age, gender, subcutaneous nodules, erosions, joint deformities, laboratory parameters, duration of RA, as well as dose, duration and type of disease modifying anti-rheumatic drug (DMARD) and steroid usage were recorded. RA activity was assessed using a 28-joint disease activity score (DAS28). The functional status of patients was measured using the health assessment questionnaire (HAQ). The symptoms and signs of polyneuropathy were quantified using the neuropathy symptoms score (NSS) and the neuropathy disability score (NDS), respectively. RESULTS: Ten patients (17%, eight women and two men) had polyneuropathic involvement as defined by nerve conduction studies (NCS). Two patients had mild symmetric sensory neuropathy and eight patients had mild symmetric sensorimotor axonal polyneuropathy. There was no significant difference in age, gender, subcutaneous nodules, erosions, joint deformities, rheumatoid factor, as well as dose, duration and type of DMARD and steroid therapy administered. We found a significant relationship among polyneuropathy and duration of RA, DAS28, HAQ, as well as abnormal NSS and NDS values. The durations of RA and DAS28 were also associated with a four- and three-fold increase in the risk of polyneuropathy, respectively. CONCLUSION: Mild symmetric sensory or sensorimotor axonal polyneuropathies are common in RA patients and it is difficult to distinguish the symptoms of polyneuropathy from those of arthritis. An electrophysiological examination should be routinely carried out especially when patients have had a long disease duration and high scores for DAS28, HAQ, NSS and NDS.

Visual evoked potentials in differential diagnosis of multiple sclerosis and neurobehcet's disease.

Behcet's disease, a multisystemic vascular inflammatory disorder of unknown origin, is relatively rare and central nervous system involvement is seen in 5% of affected individuals. This form of the disease, called as neurobehcet's disease (NB), can be misdiagnosed as multiple sclerosis (MS), a demyelinating disorder of central nervous system, so their differential diagnosis is important. In this study, to identify the parameters of electrophysiological testing that might be useful in their differential diagnosis, we performed evoked potentials (EPs) and electroneuromyography (ENMG) on patients with MS and NB, and on normal volunteers. A total of 95 persons, 55 MS patients, 20 NB patients and 20 normal volunteers between ages 31 and 55, were studied electrophysiologically. Visual evoked potential (VEP), brainstem auditory evoked potential (BAEP), posterior tibial somatosensory evoked potential (SEP) and nerve conduction and needle electromyography studies were performed on all patients and volunteers. All parameters of EPs were compared among the groups. The results of the BAEP and SEP studies did not show statistically significant difference between NB and MS. However, the VEP study indicated that the amplitude values of cortical VEP potentials (P100) in the NB and MS groups were lower than those of the normal group (p < 0.01), and that the amplitudes in the NB group were lower than for the MS group (p < 0.05). Therefore, P100 amplitude measured from peak to peak seems to be more reliable and thus should be used in the differential diagnosis of MS and NB.

Hypothyroid myopathy with manifestations of Hoffman's syndrome and myasthenia gravis.

Although hypothyroid myopathy is seen frequently and the relationship with autoimmune hypothyroidism and myasthenia gravis is well known, specific forms of hypothyroid myopathy such as Hoffman's syndrome (HS) are rarely described. Here we describe a 40-year-old patient with Hashimoto thyroiditis showing symptoms and signs of two discrete forms of hypothyroid myopathy (HS and myasthenic syndrome) together. To our knowledge this is the first reported case with features of both of these syndromes. We discuss the diagnosis, speculate whether this patient may represent a unique form of hypothyroid myopathy, and report the 6-month follow-up of the patient both clinically and electrophysiologically.

Axillary neuropathy mimicking quadrilateral space syndrome and its follow up for one year.

Axillary neuropathy due to entrapment of the nerve in the quadrilateral space is seen rarely. Here, we describe a 24-year-old patient diagnosed with isolated axillary neuropathy that mimicked quadrilateral space syndrome. Quadrilateral or quadrangular space syndrome (QSS), first described by Cahill and Palmer in 1983, was defined as the entrapment of the distal branch of the axillary nerve and the posterior humeral circumflex artery (PHCA) in the quadrangular shaped anatomic space. We tracked the follow up of the patient for one year both clinically and electrophysiologically. Our aim in reporting this case is to stress the point that cases of such a nature usually represent situations of diagnostic and treatment challenges, where multidisciplinary approaches are required.