Yüksel Kaplan

Chronic migraine associated with the Chiari type 1 malformation

OBJECTIVE Our objective was to assess the frequency and clinical characteristics of migraine in the patients with CM-1. METHODS We analyzed migraine in 73 patients with CM-1. Migraine was classified according to the new International Headache Society criteria. We did not include patients who had intracranial, parenchymal, or cervical lesions other than CM-1 on brain and cervical magnetic resonance imaging. RESULTS Of the 73 patients diagnosed as having CM-1, 11 (15.06%) had migraines; of them, 8 (10.95%) had chronic migraines, 2 (2.73%) had migraines with auras, and 1 (1.36%) had migraines without auras. The patients who had both migraines and CM-1 (group 1) were compared regarding clinical characteristics and demographic features to the control group having chronic migraines. The control group comprised subjects free of CM-1. Onset age of pain was earlier and the frequency of headache days per month, baseline pain intensity, exacerbation of pain intensity, nausea, vomiting, and pain aggravated by physical activity were significantly higher in group 1. CONCLUSIONS Although we found the frequency of migraine to be similar to that in population-based studies, we detected a threefold increased frequency of chronic migraine in this special population. We believe that CM-1 may be a factor associated with chronic migraine.

Combination of myotonic dystrophy and hereditary motor and sensory neuropathy

Myotonic Dystrophy Type 1 (DM1) in combination with demyelinating neuropathy is very rare in literature. In this study, DM1 and demyelinating neuropathy were demonstrated clinically and electromyographically in a 43-year-old female patient from Turkey. In the patient an expanded CTG repeat in the Myotonic Dystrophy Protein Kinase (DMPK) gene was confirmed in combination with a duplication in the Charcot-Marie-Tooth Disease (CMT1A) gene. DM1 was also determined in her 25-year-old son.

Phenotypic differences in a large family with Kennedy's disease from the Middle Black Sea region of Turkey.

We report the clinical and electrophysiological features of a large Turkish family with genetically confirmed X-linked spinal and bulbar muscular atrophy (SBMA). Family members were identified by field work. A detailed history was obtained from each subject, and each subject received a detailed neurological examination. To confirm the CAG repeat expansion in the AR gene, genomic DNA was extracted from the peripheral blood of patients. The family consisted of 128 individuals over five generations, with two consanguineous parents, one slightly affected female, and 12 affected males with SBMA. We studied the five surviving male patients and one surviving female carrier. The age at disease onset, phenotypic features, and disease severity varied among the family members. DNA analysis was performed on five individuals, belonging to five generations of the family. Four affected males and a slightly affected female carrier were shown to carry an expanded CAG repeat in the androgen receptor gene. This family report is consistent with previous studies suggesting that SBMA may be present with a wide clinical spectrum in affected family members. Further descriptions of SBMA affected families with different ethnic backgrounds may assist in identifying possible phenotypic and genetic features of the disease.

Femoral nerve involvement in diabetics.

Background and purpose:  In this study, the conduction of the femoral nerve has been evaluated in diabetic patients without clinical signs of femoral nerve involvement and in a group of healthy subjects.

Neuromuscular transmission in hypoxemic patients with chronic obstructive pulmonary disease

Many studies have focused on the systemic effects of chronic obstructive pulmonary disease (COPD), but none has examined neuromuscular junction transmission (NMT). We evaluated NMT dysfunction using single-fiber electromyography (SFEMG) in patients with COPD. Twenty patients with COPD and 20 age-matched healthy controls were included in the study. All patients and controls underwent SFEMG. Abnormal NMT was found in seven of 20 patients (35%), but in none of the control subjects. The COPD patients were subgrouped according to the presence of hypoxemia. The patients with normoxemia were classified as Group 1, and the patients with hypoxemia were classified as Group 2. Abnormal NMT was found in six patients in Group 2 and in one in Group 1. While there was significant difference in terms of abnormal NMT between Group 2 and the controls, there was none between Group 1 and the controls. Our results show that NMT abnormalities can be present in hypoxemic patients with COPD.

What are the Predictors of Death in Patients With Cranio-Cervical Artery Dissection?

INTRODUCTION Few studies have reported the predictive factors related to mortality in patients with cranio-cervical artery dissections (CCAD). Our aim was to investigate the predictors related to in-hospital mortality in patients with CCAD and its subgroups. METHODS Sixty-seven patients diagnosed with carotid artery dissection (CAD) or vertebral artery dissection (VAD), admitted to our clinic between 2000 and 2013, were retrospectively reviewed. Age, gender, modified Rankin Scale scores (pre-stroke and at admission), clinical presentation type, location of the dissection, risk factors, and treatments were analyzed as mortality-related prognostic factors. Of the 67 patients, 12 (17.9%) died, five (7.46%) with CAD and seven (10.44%) with VAD. We compared the prognostic characteristics of the surviving versus deceased patients with CCAD and in the subgroups with CAD and VAD. RESULTS Age above 45 years, severe disability at admission, presentation with stroke, and intracranial VAD occurred more frequently in deceased patients and were independent variables related to mortality in patients with CCAD and its subgroup with VAD. Severe disability at admission alone was related to mortality in patients with CAD. Hypertension and hypercholesterolemia were independent variables related to mortality in patients with CCAD. CONCLUSION Severe disability at admission was a mortality predictor in both CAD and VAD. Although the initial severity of stroke is reportedly related to poor outcomes in patients with CCAD, it has not previously been directly identified as a predictor of mortality in patients with CAD or VAD.

Mortality-related factors in ischemic stroke patients 80 years of age and older

OBJECTIVE: The aim of this study was to investigate mortality-related factors in ischemic stroke patients 80 years of age and older. MATERIAL and METHODS: We reviewed all ischemic stroke patients admitted to our clinic between January 2010 and January 2012. The patients’ database information was retrospectively analyzed. One hundred and ten patients aged older than 80 years with ischemic stroke were included in the study. The patients were divided into two groups based on survival. Age, gender, recurrent stroke, risk factors, clinical syndrome, etiology, radiographic localization, duration of hospitalization, and presence of systemic complications were accepted as mortality-related prognostic factors. The groups were compared according to these prognostic factors. RESULTS: In the clinical follow-up, 58 (52.7%) patients died; 65.5% died of neurological causes, and 31% died of systemic complications. No significant differences existed between the two groups in age, gender, risk factors, recurrent stroke, or etiology. The frequency of total anterior circulation infarct syndrome was much higher in deceased than living patients (50% and 36.5% respectively;p<0.05). Deceased patients had a statistically significantly higher incidence of total MCA infarct and systemic complications than did living patients (p<0.05). CONCLUSION: To date, many variables that affect the early and late prognosis in stroke patients have been reported. Many of these factors cannot be changed, such as oldest age. It is important to determine the changeable and non-changeable factors related to disability and death in the oldest age groups. Starting appropriate treatment and care initiatives as soon as possible is also very necessary in the oldest stroke patients.

Semptomatik Chiari Malformasyonu Tip 1 ile İlişkili Küme Baş Ağrısı

Cluster headache (CH) is considered as a primary headache syndrome, thus neuroradiological investigations of the central nervous system should be normal in primary CH patients (1). However, symptomatic cluster-like headaches (CLH) have been reported in association with various disorders such as aneurysms, arteriovenous malformations, cervical trauma, medullary infarcts, inflammatory/infectious diseases, and tumors (2). CLH associated with Chiari type 1 Malformation (CM-1) has been described in only one case report in the relevant literature (3). Here we describe a case diagnosed with CLH associated with symptomatic CM-1. A 28-year-old female patient had admitted with recurrent headaches since the age of 20 years. She had severe, recurrent, pain in the right frontal and periorbital areas and headache, lasting about 20-90 minutes. She also suffered from ipsilateral tearing, rhinorrea and ptosis during headaches. These pain attacks occurred irregularly two to four times per day. Although this attack usually appeared spontaneously, it was precipitated by head or neck movements. Additionally, she also suffered from occasional pain in the occipital-suboccipital area, vertigo, blackout in the eyes, palpitation, sweating; all of which disappearing in 5-10 minutes. Neurological examination revealed vertical and down-beat nystagmus, positive Romberg’s test, and hyperreflexia. Her cardiologic tests were normal. Cranial and cervical MRIs disclosed CM-1 (Figure 1). She responded well to methyl-prednisolone 60 mg/day (10 days), which was tapered in a month period. We believe that CM-1 could be interpreted as symptomatic in this patient. While CM-1 can be asymptomatic, clinical manifestations, which typically begin in young adulthood, can include headaches, visual disturbances, neuro otological complaints, lower cranial nerve dysfunction, and sleep apnea (4). Among the manifestations of CM-1, headache is one of the most common symptoms, occurring in 15-98% of the patients. The known headache spectrum in CM-1 includes cough headaches, exertional headaches, low cerebrospinal fluid pressure headaches, long-lasting headache attacks, suboccipital headaches, and migraine attacks (4). While CM-1 and CH seemed as unrelated diseases, determining the actual cause of CH is obviously difficult when these two pathological processes co-occur. It raises the question whether the pathogenesis of these diseases may be linked. With the current precision of central nervous system imaging, CM-1 can be an incidental finding when MRI is conducted for other reasons. In CM-1, variable caudal displacement of cerebellar 93

Mortality-related factors in ischemic stroke patients 80/INS; years of age and older

OBJECTIVE: The aim of this study was to investigate mortality-related factors in ischemic stroke patients 80 years of age and older. MATERIAL and METHODS: We reviewed all ischemic stroke patients admitted to our clinic between January 2010 and January 2012. The patients’ database information was retrospectively analyzed. One hundred and ten patients aged older than 80 years with ischemic stroke were included in the study. The patients were divided into two groups based on survival. Age, gender, recurrent stroke, risk factors, clinical syndrome, etiology, radiographic localization, duration of hospitalization, and presence of systemic complications were accepted as mortality-related prognostic factors. The groups were compared according to these prognostic factors. RESULTS: In the clinical follow-up, 58 (52.7%) patients died; 65.5% died of neurological causes, and 31% died of systemic complications. No significant differences existed between the two groups in age, gender, risk factors, recurrent stroke, or etiology. The frequency of total anterior circulation infarct syndrome was much higher in deceased than living patients (50% and 36.5% respectively;p<0.05). Deceased patients had a statistically significantly higher incidence of total MCA infarct and systemic complications than did living patients (p<0.05). CONCLUSION: To date, many variables that affect the early and late prognosis in stroke patients have been reported. Many of these factors cannot be changed, such as oldest age. It is important to determine the changeable and non-changeable factors related to disability and death in the oldest age groups. Starting appropriate treatment and care initiatives as soon as possible is also very necessary in the oldest stroke patients.