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).

Effect of leukemia inhibitory factor in experimental cisplatin neuropathy in mice.

In this study, the effect of leukemia inhibitory factor (LIF) on cisplatin (CDDP)-induced neuropathy was evaluated. Mice were treated with CDDP, 2 mg/kg i.p. twice a week nine times. During the last week some of the mice were also injected with LIF, 2 mug/kg s.c. every other day for a total of four injections. Development of neuropathy was evaluated with changes in tail flick latency and sensory nerve conduction velocity (NCV). At the end of the treatment period dorsal root ganglia (DRG) were microscopically examined. Some of the DRGs were explanted into extracellular matrix, covered with culture medium and incubated for 3 days. During and at the end of the incubation, cellular migration and axonal outgrowth from the DRGs were quantified. LIF proved effective in reversing the increase in tail flick latency (p<0.05) and improving the reduction in NCV induced by CDDP. CDDP led to smaller nuclear and somatic size in neurons, while with LIF, the latter was restored to control values (p<0.01). No apoptotic nucleus was observed among DRG neurons while very few and moderate numbers detected among satellite and Schwann cells, respectively. With LIF, none of the cells had apoptosis. CDDP caused a decrease in the number of migrating cells and in the length of outgrowing axons while LIF treatment restored both capacities (p<0.05) In conclusion, in CDDP-induced neuropathy, LIF was found to be effective in correcting some functional and morphological deteriorations related with major involvement of Schwann cells.

Severe peripheral polyneuropathy in a child with infective endocarditis caused by Staphylococcus aureus.

Although central nervous system complications such as stroke, encephalopathy and meningitis are commonly described in Staphylococcus aureus endocarditis, peripheral nervous system involvement is rarely reported in the literature. In this article we report on a 13-year-old boy with infective endocarditis caused by Staphylococcus aureus in whom severe polyneuropathy developed during hospitalization. To the best of our knowledge this is the first child case with infective endocarditis associated with peripheral polyneuropathy in the literature.

A case of metastatic spinal Ewing's sarcoma misdiagnosed as brucellosis and transverse myelitis.

An 11-year-old girl was admitted with back pain for 2 months, inability to walk for 15 days, and enuresis and encopresis for 2 days. She had been hospitalized with the diagnosis of brucellosis in another hospital. At presentation, she had paraplegia, sphincter dysfunction, and bilateral sensory loss below the T6 level, and was initially diagnosed with transverse myelitis caused by brucellosis. On the third day of hospitalization, however, agglutination test for brucella was negative, but it was positive for Salmonella. Therefore, transverse myelitis was considered to be due to salmonellosis. Thoracic spine magnetic resonance imaging showed an extradural, paraspinal mass at the level of T6-T7. The mass was totally extracted, and histopathological examination revealed Ewing's sarcoma. During follow-up, no improvement in paraplegia was noted and an enlarged presacral decubital ulcer developed. Aside from supportive care, local radiotherapy was applied. Unfortunately, the patient died from probable infection 9 months after the diagnosis. We emphasize that metastatic spinal Ewing's sarcoma may mimic brucellosis and transverse myelitis in childhood.

A whole genome screen for linkage in Turkish multiple sclerosis.

Factors exerting recessive effects on susceptibility to complex traits are expected to be over-represented in communities having a higher frequency of consanguineous marriage. Multiple sclerosis, a typical complex trait, is relatively common in Turkey where cultural factors also determine a high rate of consanguineous marriage. Previous genetic studies of multiple sclerosis in Turkey have been confined to the search for associations with candidate genes. In order to exploit the special genetic features of the Turkish population, we performed a whole genome screen for linkage in 43 Turkish multiplex families employing 392 microsatellite markers. Two genomic regions where maximum lod score (MLS) values were suggestive of linkage were identified (chromosomes 13q and 18q23) along with a further 14 regions of potential linkage. Parametric analysis of these data using a recessive model, appropriate for populations with a high frequency of consanguinity, increased the LOD scores in four regions.

A genome screen for linkage disequilibrium in Turkish multiple sclerosis.

In order to screen the Turkish population for evidence of association with multiple sclerosis, we typed 6000 microsatellite markers in separately pooled DNA samples from 197 cases and 199 controls following the Genetic Analysis of Multiple sclerosis in EuropeanS (GAMES) protocol. Twelve markers showing evidence for association were identified. One of these markers lying directly in a region which is also implicated in the Turkish linkage screen (chromosome 5p15) and thus shows evidence for both linkage and association in independent data sets.

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.

Disposable concentric needle electromyography electrodes artifact discharge mimicking positive sharp waves.

OBJECTIVES: To determine a new artifact discharge produced from the disposable concentric needle (DCN) electromyography (EMG) electrodes. METHODS: We have recorded the activity obtained after the first insertion of 41 DCN (37 mm) and 36 DCN (50 mm) in several muscles during the rest. The number of the patients was 77 (26 males, 51 female). RESULTS: We observed an artifact discharge (AD) resembling positive sharp waves (PSWs) in 31 patients of 77 insertions. The AD occurred with 18 of 41 insertions of DCN 37 mm and 13 of DCN 50 mm. The artifact resolved when the needle has been moved in the muscle or in the adipose subcutaneous tissue. CONCLUSION: This AD should be recognized when using DCN electrodes to avoid confusion with PSWs. Recognizing these artifacts during needle EMG is important to avoid false positive results.

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.

Central nervous system brucellosis: presentation, diagnosis and treatment.

Although neurological symptoms in brucellosis are frequent, central nervous system (CNS) involvement is uncommon. Five patients with neurobrucellosis are presented. Three patients presented with meningoencephalitis, one with polyradiculoneuritis and one with myelitis and an eighth nerve palsy. All patients had lymphocytic pleocytosis, low glucose and elevated levels of protein in the cerebrospinal fluid (CSF). Gamma-globulin and IgG values in the CSF were also increased. Results of agglutination tests for Brucella in serum and CSF were positive for all patients, although titres were less in the CSF. Brucella melitensis was cultured from two patients. Treatment included concurrent administration of three of the following drugs: doxycycline, rifampicin, streptomycin, co-trimoxazole, ceftriaxone or ciprofloxacin. The three patients with meningoencephalitis fully recovered; the other two patients were left with minor disability.