An epidemiologic investigation of amyotrophic lateral sclerosis in Thrace, Turkey, 2006-2010.

OBJECTIVE: The aim of the study was to investigate the incidence and prevalence of amyotrophic lateral sclerosis (ALS) in Thrace, Turkey in a five-year time period (2006-2010). METHODS: Study population included residents of three provinces (Edirne, Tekirdag, Kirklareli) in the Thrace region. Cases were ascertained from all of the neurologic centers and hospitals of these provinces. Demographic and clinical information was collected for each patient. Newly diagnosed ALS patients who are fulfilling the El Escorial revised diagnostic criteria were enrolled into the study. RESULTS: We identified a total of 145 patients (93 males, 52 females). The mean age at diagnosis was 57.0 ± 13.6. According to El Escorial criteria, 60.0% of the cases were definite ALS, 24.8% were probable, and 15.2% were possible ALS. Thirty-two cases were bulbar (22.1%), 113 cases (77.9%) were spinal onset. Mean time delay from onset to diagnosis was 12.0 ± 11.2 months. Age-gender standardized incidence rates with reference to Turkey, USA 2008 census were 1.9 (95% confidence interval (CI), 1.8-2.1), 1.9 (95%CI, 1.8-2.2) for overall. There were 112 living ALS patients at the end of the study. Crude point prevalence was calculated as 7.3 per 100,000 population (95%CI, 5.9-8.7). CONCLUSIONS: This is the first study to provide fundamental data about demographic and clinical characteristics about ALS in Thrace region of Turkey. Incidence and prevalence of ALS in Thrace region of Turkey appear to be comparable with European countries.

Possible promoting effects of melatonin, leptin and alcar on regeneration of the sciatic nerve.

Peripheral nerve injury is a widespread and disabling condition that can impair the individual's daily life. Studies involving medications that may positively affect peripheral nerve regeneration are rare. The aim of this study was to investigate new treatments after peripheral nerve injury using various neuroprotectants, melatonin, alcar and leptin, in the regenerative process in an experimental rat model. Wistar albino rats were randomly divided into eight groups containing equal number of animals. Intraperitoneal injection of melatonin (50mg/kg, for 21days), leptin (1mg/kg, for 21days) and acetyl-l-carnitine (50mg/kg, for six weeks) was performed postoperatively. Histological and electromyographical assessments of the regenerated nerves were performed 12 weeks after surgery. Stereological analysis was performed to estimate myelinated and unmyelinated axon numbers, surface area, myelin thickness and the myelin thickness/axon diameter ratio for each group. The results showed that only alcar has a beneficial effect on the regeneration of unmyelinated axons. Neither melatonin and leptin nor alcar were observed to have any therapeutic effect on the regeneration of myelinated axons. Alcar therapy has a positive effect on the regeneration of unmyelinated fiber in the sciatic nerve. However, the same effect was not observed in myelinated nerve fibers after intraperitoneal application of melatonin and leptin.

The value of electroencephalography in differential diagnosis of altered mental status in emergency departments.

OBJECTIVE: To evaluate the value of electroencephalography in patients with altered mental status in emergency departments. METHODS: Demographical characteristics, types and aetiologies of seizures, and clinical outcomes of the patients were recorded. Patients were divided into 4 groups according to the complaints of admission: findings and symptoms of seizure; stroke and symptoms of stroke-related seizures; syncope; and metabolic abnormalities and other causes of altered mental status. The electroencephalography findings were classified into 3 groups: epileptiform discharges; paroxysmal electroencephalography abnormalities; and background slowing. Electroencephalography abnormalities in each subgroup were evaluated. SPSS 21 was used for statistical analysis. RESULTS: Of the total 190 patients in the study, 117 (61.6%) had pathological electroencephalography findings. The main reason for electroencephalography in the emergency department was the presence of seizure findings and symptoms in 98 (51.6%) patients. The ratio of electroencephalography abnormality was higher in patients who were admitted with complaints of metabolic abnormality-related consciousness disturbances (p < 0.001). A total of 124 (65.3%) patients had neuroimagings. Electroencephalography abnormalities were found to be significantly higher in patients with neuroimagings compared to those without neuroimagings (p < 0.003). CONCLUSION: Despite advanced neuroimaging techniques, electroencephalography is still an important tool in the differential diagnosis of altered mental status such as epileptic seizures, metabolic abnormalities, pseudo-seizures and syncope.

Cerebral fat embolism syndrome after long bone fracture due to gunshot injury.

Cerebral fat embolism syndrome is a lethal complication of long-bone fractures and clinically manifasted with respiratory distress, altered mental status, and petechial rash. We presented a 20-year-old male admitted with gun-shot wounds to his left leg. Twenty-four hours after the event, he had generalized tonic clonic seizures, decorticate posture and a Glascow Coma Scale of seven with localization of painful stimuli. Subsequent magnetic resonance imaging of the brain showed a star-field pattern defining multiple lesions of restricted diffusion. On a 4-week follow-up, he had returned to normal neurological function. Despite the severity of the neurological condition upon initial presentation, the case cerebral fat embolism illustrates that, cerebral dysfunction associated with cerebral fat embolism illustrates reversible.

Has admission blood pressure any prognostic value in patients with subarachnoid hemorrhage: an emergency department experience.

Hypertension is a well-known risk factor for the development and rupture of cerebral aneurysms. The authors conducted a study to investigate the prognostic value of admission blood pressure (BP) on prognosis in patients with subarachnoid hemorrhage (SAH). Two hundred patients with SAH were divided into two groups according to Hunt Hess score (good prognosis: 1 to 3, and poor prognosis: 4 and 5) and according to death in hospital (surveyed and died). The prognostic factors of SAH and BP changes according to Hunt Hess scores in the acute stages of the event were evaluated. Admission mean arterial BP values of the patients who died in hospital were significantly lower than in the patients who were surveyed (P=.026). The admission mean arterial BP values were found to be lower in the poor prognostic patients (Hunt Hess score of 4 and 5) (P<.001). Decreased admission BP values were found to be associated with poor prognosis and mortality.

Phrenic neuropathy in diabetic and prediabetic patients without neuromuscular complaint.

Neuropathy, one of the major reasons of morbidity in diabetes mellitus (DM), is associated with prediabetic conditions as well as DM. The present study aims to compare phrenic and peripheral nerves in prediabetic, diabetic patients and healthy controls. A total of 37 diabetic, 40 prediabetic patients and 18 healthy controls were enrolled in the study. All subjects underwent conventional sensory and motor nerve conduction studies. Bilateral phrenic and peripheric nerve conduction studies were performed. In both right and left phrenic nerves, the amplitudes were lower in prediabetic and diabetic patients than control subjects, respectively (p: 0.005 and p: 0.001). Both of the phrenic nerve conductions were altered similarly. The results of our study demonstrate that phrenic nerves are affected like peripheric nerves in prediabetic and diabetic patients. We suggest reminding phrenic neuropathy in newly onset respiratory failure in diabetic and prediabetic patients.

Entrapment neuropathies in chronic stroke patients.

Stroke is the third most common cause of mortality and is one of the most common causes of morbidity in the world. Entrapment neuropathies may cause morbidity after stroke. In this study, we aimed to evaluate the development of entrapment neuropathies in severe stroke patients within the chronic stages of the event. Thirty-two patients with first ever ischemic or hemorrhagic stroke were included in the study. The nerve conduction studies were performed at least 6 months after the event. Ten age- and sex-matched healthy subjects were evaluated as control subjects. Twelve patients (37.5%) had median nerve neuropathy at the wrist, and 12 patients (37.5%) had ulnar nerve neuropathy at the elbow in the symptomatic extremities. Eight patients (25%) had median nerve neuropathy at the wrist, and 6 patients (18.7%) had ulnar nerve neuropathy at the elbow in the asymptomatic extremities. Our results confirm that in chronic stroke patients, the entrapment neuropathies may be an important cause for morbidity, and these entrapment neuropathies could be seen bilaterally but more prominent in the paretic sides.

The effect of admission blood pressure on the prognosis of patients with intracerebral hemorrhage that occurred during treatment with aspirin, warfarin, or no drugs.

BACKGROUND: Hypertension is the most important modifiable risk factor for intracerebral hemorrhage (ICH), but blood pressure (BP) management during the acute phase of ICH is still controversial. Approximately one-fourth of ICHs occur during treatment with warfarin or aspirin. AIM: This study was designed to determine the effect of admission BP on the early prognosis of ICH patients by dividing them into three groups (warfarin, aspirin, and no drugs). METHODS: Three hundred and sixty-nine patients with supratentorial ICH were divided into three groups according to medication. Each group was evaluated in terms of prognosis and the risk for mortality based on the modified Rankin Scale (mRS) score at discharge (good prognosis: mRS ≤ 3; poor prognosis: mRS > 3). The effect of admission BP on prognosis was evaluated for each group. RESULTS: The in-hospital mortality rate was 72% for ICH patients treated with warfarin, 41.6% for ICH patients treated with aspirin, and 35% for ICH patients treated with no drugs. Admission mean arterial blood pressure (MABP) values were higher in patients with poor prognosis compared with patients with good prognosis for the aspirin (P = .002) and no-drug (P = .001) groups, but not in the warfarin (P = .067) group. CONCLUSION: A high MABP at admission was found to be an independent predictor of poor prognosis for ICH patients treated with aspirin or with no drugs, but not for ICH patients treated with warfarin.

Does cervical radiculopathy have an effect on peripheral nerve conduction studies? An electrophysiological evaluation.

BACKGROUND: Peripheral nerve neuropathies are more common in patients with cervical radiculopathy (CR) and a proximal lesion along an axon might predispose that nerve to injury at distal sites. To evaluate this hypothesis, the frequency of median nerve neuropathy at the wrist and the frequency of ulnar nerve neuropathy at the elbow were investigated in 80 patients with one-sided CR. METHODS: The symptomatic and asymptomatic extremities were compared for the electrophysiological parameters of ulnar and median nerve conduction studies. The patients were divided into three diagnostic subgroups (C5/C6 radiculopathy, C7 radiculopathy and C8/Th1 radiculopathy), and ulnar nerve conduction studies were compared between symptomatic and asymptomatic extremities in each subgroup. RESULTS: Although, the median and ulnar nerves are mainly derived from C8/Th1 roots, we did not observe an increased frequency of ulnar nerve involvement at the elbow (3.7% in symptomatic, 2.5% in asymptomatic extremities) while increased median nerve involvement was present at the wrist (27.5% in symptomatic, 12.5% in asymptomatic extremities). CONCLUSION: The electrophysiological data and the frequency of ulnar and median nerve neuropathy did not support an etiological association between CR and distal neuropathy. SIGNIFICANCE: the association between CR and peripheral neuropathy is associational rather than causative.

Ischemic stroke in young adults: risk factors, subtypes, and prognosis.

BACKGROUND: ischemic strokes occurring in patients younger than 47 years is a relatively rare event and accounts for less than 5% of all ischemic strokes in western countries. OBJECTIVE: the etiologic spectrum in younger patients and older patients differs considerably. METHODS: in this hospital case series study, we enrolled 192 patients with ischemic stroke, aged 18 to 47 years, all of whom were submitted to a diagnostic protocol. The risk factors for stroke and the distribution of stroke subtype and prognosis were studied. Modified diagnostic criteria adopted from the Trial of ORG 10172 in Acute Stroke Treatment and the Baltimore-Washington Cooperative Young Stroke Study, were used for etiologic classification. RESULTS: hypertension was found to be the main risk factor (45%) followed by cigarette smoking (37%), hyperlipidemia (35.4%), diabetes mellitus (17%), and family history of stroke (18%). Hypertension, diabetes mellitus, hypercholesterolemia, and smoking were present either alone or in combination in the majority of our patients. Oral contraceptives were being taken by 25% of the women. The etiology of stroke was as follows: atherothrombosis 26.5%, cardioembolism 20%, nonatherosclerotic vasculopathies 13%, other determined causes 10%, lacunar stroke 6%, migraine 3.6%, and undetermined causes 21%. CONCLUSIONS: hypertension, diabetes mellitus, hypercholesterolemia, and smoking were the most common risk factors in our ischemic stroke patients between 18-47 years of age. Health care programs targeting the prevention and treatment of these factors will reduce the associated morbidity and mortality of stroke among this socioeconomically active age group.

Cost of acute ischemic and hemorrhagic stroke in Turkey.

OBJECTIVE: The aim of this study is to examine the direct medical costs and outcomes of patients with stroke. MATERIAL AND METHODS: The records of the patients admitted with ischemic and hemorrhagic stroke to the University of Trakya, School of Medicine, Department of Neurology were reviewed retrospectively in year 2007. Direct medical costs (total costs, radiological, laboratory, medicine, and other) were calculated, additionally cost per life saved and per life-year saved were calculated for stroke patients. RESULTS: The study group consisted of 328 patients (169 male/159 female) and mean age was 66.5 ± 12.4 years. Length of hospital stay was 10.7 ± 7.5 days. Mortality rate was 20.4% and the mRS score of the patients was 3.2 ± 2.1. The average cost of stroke was US$ 1677 ± 2964 (29.9% medicine, 19.9% laboratory, 12.8% neuroimaging, and 38% beds and staff). Cost per life saved and per life-year saved were US$ 2108 and US$ 1070, respectively. CONCLUSION: This is the first study in order to determine direct medical cost of stroke in Turkey, therefore, it may be guideline for disease-cost management of stroke.

Bilateral basal ganglionic lesions due to transdermal methanol intoxication.

Methanol is a clear, colorless, and highly toxic liquid with a similar smell and taste to ethanol, and is found in many commercial products such as solvents and cleaning fluids. Severe methanol intoxication occurs after suicidal or accidental oral ingestion of solvents. A few patients with methanol intoxication via the transdermal route have been reported. We present a 47-year-old woman with acute transdermal methanol intoxication admitted to the emergency department with weakness, blurred vision, bilateral areactive mydriasis, and deterioration of consciousness.

Stroke due to bee sting.

Although bee stings can cause local reactions, neurovascular complications are rare. A 60-year-old man developed a focal neurologic deficit 2 hours after multiple bee stings, which was confirmed to be acute cerebral infarctions on magnetic resonance imaging scan.

Acute onset myositis associated with brucellosis, quite a rare diagnosis.

Brucellosis is a zoonosis that is transmissible to humans. It is a disease with multi-systemic involvement caused by the genus Brucella. Neurological complications, including meningitis, meningo-encephalitis, myelitis-radiculoneuritis, brain abscess, epidural abscess and meningo-vascular syndromes, are rarely encountered. We present a patient presenting with acute onset myositis. This kind of presentation has not previously been reported in the English language literature. We conclude that the diagnosis of neuro-brucellosis should be considered in patients presenting with muscle weakness.

[Cerebral infarction due to traumatic carotid artery dissection: case report and review of current management]. / Travmatik karotis arter diseksiyonuna bagli serebral enfarkt: Olgu sunumu ve güncel yaklasimin gözden geçirilmesi.

With the advent of improved neuroradiological methods, it has been determined that frequency of traumatic carotid artery dissections is higher than previously observed. Since delayed neurological deficits may develop in some asymptomatic undiagnosed cases, it is essential to consider the possibility of the carotid artery dissection and evaluate it properly in suspicious cases. In this article, a case of internal carotid artery dissection and subsequent cerebral infarction following a motor vehicle accident is presented. Pathogenesis, clinical features, diagnostic method choices and treatments in this rare but severe condition are discussed in light of the relevant literature in order to convey current knowledge.

Hereditary neuropathy with liability to pressure palsies in a Turkish patient (HNPP): a rare cause of entrapment neuropathies in young adults.

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant nerve disease usually caused by 1,5 Mb deletion on chromosome 17p11.2.2-p12, the region where the PMP-22 gene is located. The patients with HNPP usually have relapsing and remitting entrapment neuropathies due to compression. We present a 14-year-old male who had acute onset, right-sided ulnar nerve entrapment at the elbow. He had electrophysiological findings of bilateral ulnar nerve entrapments (more severe at the right side) at the elbow and bilateral median nerve entrapment at the wrist. Genetic tests of the patient demonstrated deletions in the 17p11.2 region. The patient underwent decompressive surgery for ulnar nerve entrapment at the elbow and completely recovered two months after the event. Although HNPP is extremely rare, it should be taken into consideration in young adults with entrapment neuropathies.

Simultaneous onset of hemorrhagic and ischemic strokes.

Primary brain hemorrhage and infarction only very rarely occur simultaneously. We report a patient with the simultaneous onset of hemorrhagic and ischemic strokes who had uncontrolled hypertension and atrial fibrillation. Neuroradiologic investigations revealed a large right thalamic hematoma with ventricular extension and an infarct in the territory of the left internal carotid artery. The patient died at the end of the second day because of herniation in spite of anti-edema and antihypertensive medication.