Acute motor axonal neuropathy cases in Van region.

Acute motor axonal neuropathy (AMAN) is a form of Guillain Barré Syndrome (GBS) seen in summer months in Northern China to cause epidemics. This form of the disease, which is also sporadically observed in other countries, constitutes less than 5% of GBS in Western countries. It usually develops with motor findings. No sensory findings are observed. In some of the cases, the severe impairments in tissues improve however slowly and inadequately. In the motor conduction studies of cases with AMAN, motor action potential values are lowered. On needle electromyography (EMG), motor unit potential (MUP) activity is diminished with spontaneous denervation findings. Investigations were conducted on nerve conduction of patients with GBS aged from 1 to 77 years. AMAN was detected in 25 of these patients. In our investigation, AMAN as a GBS variant was detected in 39.7% of the patients. The conduction velocities of motor nerves were in normal ranges whereas combined muscle action potentials were significantly lower. No F response could be obtained. Although AMAN is a rare variant of GBS and shows different clinical courses, it has been brought under intense scrutiny since there is high prevalance of acute inflammatory neuropathies in our region (Tab. 1, Ref. 7).

Coronary artery calcifications in hemodialysis patients and their correlation with the prevalence of erectile dysfunction.

INTRODUCTION: Our aim in this study was to investigate the prevalence and correlation with coronary artery calcium scores (CACS) and erectile dysfunction (ED) among hemodialysis patients. PATIENTS AND METHODS: Thirty-five male patients with chronic renal failure were selected to participate in this study. All patients underwent examinations for CACS using 16-channel multidetector computed tomography. The presence and severity of ED were determined by calculating the erectile function domain of the self-administered International Index of Erectile Function (IIEF). RESULTS: The patients' ages ranged from 22 to 78 with a mean of 51.6 years. The mean duration of hemodialysis was 75.7 months (range = 12 to 232). Twenty-six patients had a history of one or more systemic diseases. The prevalence of any level of ED was 82.9% for all hemodialysis patients, and severe ED, 40%. The CACS was significantly higher among patients with severe ED (P = .032). The IIEF-5 score was also shown to have a moderate negative correlation with the CACS (r = -.420, P = .012). Age, duration of hemodialysis, body mass index, diabetes mellitus, hypertension, coronary heart diseases, hyperlipidemia, thyroid disease, depression, tobacco consumption, and medication were not associated with the presence of ED (P > .05). CONCLUSION: ED is prevalent in hemodialysis patients. Although many possible factors contribute to ED, the severity of ED increases with greater CACS.

A review of 35 cases of asymmetric crying facies.

A review of 35 cases of asymmetric crying facies: Congenital asymmetric crying facies (ACF) is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle (DAOM) on one side of the mouth. It is well known that this anomaly is frequently associated with cardiovascular, head and neck, musculoskeletal, respiratory, gastrointestinal, central nervous system, and genitourinary anomalies. In this article we report 35 ACF patients (28 children and 7 adults) and found additional abnormalities in 16 of them (i.e. 45%). The abnormalities were cerebral and cerebellar atrophy, mega-cisterna magna, mental motor retardation, convulsions, corpus callosum dysgenesis, cranial bone defect, dermoid cyst, spina bifida occulta, hypertelorism, micrognatia, retrognatia, hemangioma on the lower lip, short frenulum, cleft palate, low-set ears, preauricular tag, mild facial hypoplasia, sternal cleft, congenital heart defect, renal hypoplasia, vesicoureteral reflux, hypertrophic osteoarthropathy, congenital joint contractures, congenital hip dislocation, polydactyly, and umbilical and inguinal hernia. Besides these, one infant was born to a diabetic mother, and had atrial septal defect and the four other children had 4p deletion, Klinefelter syndrome, isolated CD4 deficiency and Treacher-Collins like facial appearance, respectively Although many of these abnormalities were reported in association with ACF, cerebellar atrophy, sternal cleft, cranial bone defect, infant of diabetic mother, 4p deletion, Klinefelter syndrome, isolated CD4 deficiency and Treacher-Collins like facial appearance were not previously published.

Peripheral sensory and motor abnormalities in patients with multiple sclerosis.

OBJECTIVES: Peripheral nerve abnormalities are uncommon in multiple sclerosis (MS). When present, they are usually attributed to factors associated with advanced disease, such as malnutrition or cytotoxic drugs or hereditary factors. However, a combination of MS and neuropathy has been reported. The aim of this study is to evaluate the question of possible peripheral involvement in MS. METHODS: We studied sensory and motor nerve conduction velocities (NCV) and amplitudes values in some sensory and motor nerves in upper and lower extremities in 20 definite multiple sclerosis patients diagnosed according to the criteria of Poser Scale and in 15 healthy subjects. The total number of studied nerves were 91 in patients group and 69 in control group. RESULTS: The most frequent electrophysiological abnormalities noted in patients group were low amplitude of the ulnar and sural nerve and slow NCV of the tibial and sural nerves. Electrophysiological abnormalities were found in 15 of 91 nerves examined (16.5%). The neurological disability was not associated with the presence of electrophysiological abnormalities. The electrophysiological abnormalities in control group subjects were a slight slow conduction of sural nerve in 2 of 69 nerves examined (2.9%). CONCLUSIONS: Our findings indicate a high frequency of sensory-motor electrophysiological nerve abnormalities in a selected group of multiple sclerosis patients. Electromyographers must be aware of these abnormalities when performing routine electromyography examinations in these patients.

Food-borne botulism cases in Van region in eastern Turkey: importance of electromyography in the diagnosis.

OBJECTIVES: Food-borne botulism is an acute form of poisoning that results from ingestion of a toxin produced by Clostridium botulinum. Botulism toxin causes its major effect by blocking neuromuscular transmission in autonomic and motor nerve terminals. METHODS: In this study, we present the features of eleven cases of food-borne botulism admitted to our hospital in 2001. All of the cases were caused by home-prepared foods; green beans. In these cases, the main symptoms and signs were generalized muscular weakness, dry mouth, dysphagia, disponea and diplopia. Electrophysiological studies were performed on four patients. RESULTS: Motor conduction studies showed that compound muscle action potentials were decreased with normal latencies and conduction velocities. The needle electromyography showed signs of denervation potentials like fibrillation and positive waves in four patients. Repetitive nerve stimulation with high frequency (20 Hz) induced an increment close to 100% in the amplitudes in 2 of 4 patients. CONCLUSION: Although toxin could not be detected in the patients, the electromyographic findings supported our diagnosis. We concluded that electromyography has an important role in diagnosis of botulism, especially in the condition that serologic tests are negative or cannot be performed.

Report of five children with Guillain-Barré syndrome following a nationwide oral polio vaccine campaign in Turkey.

Five children with Guillain-Barré syndrome (GBS), following a national oral polio vaccination campaign to eradicate disease, are reported. Clinical examination, cerebrospinal fluid and electromyographic findings conformed to the classical description of GBS. Four of them received therapeutic dose of intravenous immunoglobulin G. Two children succumbed to the disease. It was observed that the number of cases of GBS in children increased during the period of the oral polio vaccination campaign in Turkey, suggesting a causal relationship.

A case of Melkersson-Rosenthal syndrome associated with Ehlers-Danlos syndrome.

Melkersson-Rosenthal syndrome (MRS) is characterized by the triad of recurrent facial palsy, lingua plicata, and facial edema. Herein, we report a case of MRS associated with Ehlers-Danlos syndrome due to rare presentation. To the best of our knowledge only one case of MRS associated with Ehlers-Danlos syndrome has been reported in the literature until now.

Dural arteriovenous malformation: a rare cause of epilepsy in childhood.

A 3 year and 6 month old girl with epilepsy associated with dural arteriovenous malformation (DAVM), diagnosed on the MRI, is presented to emphasise the importance of DAVM in the aetiology of childhood epilepsy.

Cardiac involvement in Sydenham's chorea: clinical and Doppler echocardiographic findings.

Sydenham's chorea is often encountered in pure form, but it may also occur in association with carditis. Recently, it has been reported that silent, mild valvular regurgitation has been encountered in patients with rheumatic fever manifested by isolated polyarthritis and pure chorea. We studied 22 children with Sydenham's chorea to determine clinical and subclinical heart involvement at the time of the initial diagnosis. Ages of the patients ranged from 6-19 y (mean 13.14+/-3.0), and the female/male ratio was 3.4:1. All patients were evaluated by clinical and echocardiographic examination with respect to heart involvement. Carditis was detected in only 5 of 22 patients (23%) by auscultation. Clinical valvular involvement was determined: mitral regurgitation in three and combined mitral and aortic regurgitation in two. The frequency of valvular involvement increased significantly to 63% 2(14/22) after adding silent mitral regurgitation detected by colour Doppler echocardiography (chi2=7.50, p=0.0062). We found that clinically inaudible, but echocardiographically significant, mild valvular regurgitation was frequent in patients with pure chorea. It is concluded that colour Doppler echocardiography may be useful in detecting silent valvular regurgitation and in deciding the duration of prophylaxis.