Elif Kocasoy Orhan

Diet restriction in migraine, based on IgG against foods: A clinical double-blind, randomised, cross-over trial

Introduction: It is well-known that specific foods trigger migraine attacks in some patients. We aimed to investigate the effect of diet restriction, based on IgG antibodies against food antigens on the course of migraine attacks in this randomised, double blind, cross-over, headache-diary based trial on 30 patients diagnosed with migraine without aura. Methods: Following a 6-week baseline, IgG antibodies against 266 food antigens were detected by ELISA. Then, the patients were randomised to a 6-week diet either excluding or including specific foods with raised IgG antibodies, individually. Following a 2-week diet-free interval after the first diet period, the same patients were given the opposite 6-week diet (provocation diet following elimination diet or vice versa). Patients and their physicians were blinded to IgG test results and the type of diet (provocation or elimination). Primary parameters were number of headache days and migraine attack count. Of 30 patients, 28 were female and 2 were male, aged 19–52 years (mean, 35 ± 10 years). Results: The average count of reactions with abnormally high titre was 24 ± 11 against 266 foods. Compared to baseline, there was a statistically significant reduction in the number of headache days (from 10.5 ± 4.4 to 7.5 ± 3.7; P < 0.001) and number of migraine attacks (from 9.0 ± 4.4 to 6.2 ± 3.8; P < 0.001) in the elimination diet period. Conclusion: This is the first randomised, cross-over study in migraineurs, showing that diet restriction based on IgG antibodies is an effective strategy in reducing the frequency of migraine attacks.

A frameshift mutation of ERLIN2 in recessive intellectual disability, motor dysfunction and multiple joint contractures

We present a family afflicted with a novel autosomal recessive disease characterized by progressive intellectual disability, motor dysfunction and multiple joint contractures. No pathology was found by cranial imaging, electromyography and muscle biopsy, but electron microscopy in leukocytes revealed large vacuoles containing flocculent material. We mapped the disease gene by SNP genome scan and linkage analysis to an ∼0.80 cM and 1 Mb region at 8p11.23 with a multipoint logarithm of odds (LOD) score of 12. By candidate gene approach, we identified a homozygous two-nucleotide insertion in ERLIN2, predicted to lead to the truncation of the protein by about 20%. The gene encodes endoplasmic reticulum (ER) lipid raft-associated protein 2 that mediates the ER-associated degradation of activated inositol 1,4,5-trisphosphate receptors and other substrates.

Spinal accessory nerve function after neck dissections.

The aim of this study was to evaluate spinal accessory nerve function after functional neck dissection (FND) and radical neck dissection (RND) by monitoring the nerve with electromyographic (EMG) examinations. A prospective, double-blind, clinical study was undertaken in 21 patients (42 neck side dissections) operated on for head and neck malignant diseases, separated into two groups: 10 neck sides in the RND group and 32 neck sides in the FND group. Electromyographic examinations were performed pre-operatively and post-operatively in the third week and third and ninth months. Additionally, a questionnaire, modified from the neck dissection impairment index, was applied to all the patients in order to assess shoulder function in the ninth post-operative month. All patients had maximum EMG scores pre-operatively. Following the operation, motor amplitudes decreased in both groups. At the third post-operative month, amplitudes decreased to their lowest values. As expected, the decreases in amplitude and EMG score were more prominent in the RND group. Following reinnervation, the amplitudes of the trapezius motor response increased in the FND group but never reached pre-operative values (during the time of follow up). The FND group scores for pain, neck and shoulder stiffness, and disability in heavy object lifting, light object lifting and reaching overhead were significantly lower than those of the RND group. In FND, one aims to preserve anatomically the spinal accessory nerve, and it is presumed to be intact after the procedure. However, using EMG nerve function monitoring, our study revealed that profound spinal nerve injury was detected immediately after FND surgery, which tended to improve over subsequent months but had not regained its original function by the end of the ninth post-operative month.

Intravenous levetiracetam treatment in status epilepticus: A prospective study

OBJECTIVE To assess the efficacy of intravenous (IV) levetiracetam (LEV) in the treatment of status epilepticus (SE) and treatment outcomes. METHODS This study was conducted on patients, who were classified according to the clinical characteristics of their seizures, in the emergency department, neurology, and other services of our hospital. Patients were administrated IV LEV for the treatment of their SE after failing to respond to IV diazepam. RESULTS We prospectively investigated 30 patients, 16 females and 14 males whose ages ranged between 17 and 90 years (55.6 ± 19.6). Fourteen patients had convulsive SE (CSE), 11 had nonconvulsive SE (NCSE), and 5 had epilepsia partialis continua (EPC). The patients were given IV LEV with dosages ranging between 1000 and 4000 mg/day. Twenty-nine of the patients continued to receive LEV orally as maintenance treatment. The most common etiologies were cerebrovascular diseases (n = 7) and brain tumors (n = 6). SE was terminated in 23 (76.6%) patients. In the 12 months that followed SE, 9 of our patients (30%) died and 4 patients could not be contacted. Fifteen patients reported having no adverse effects, whereas three had mild adverse effects. No major adverse effects or complications causing disability were observed in twelve patients who were unconscious. CONCLUSION Treatment with IV LEV is well-tolerated and effective both in focal and generalized SE. IV LEV has the combined advantage of efficacy, safety, and ease of use, which qualifies it to be the first choice after benzodiazepines (BZD) in the treatment of SE. This is the first prospective study of IV LEV treatment in status epilepticus and has the longest follow-up period, one year.

Jitter analysis with concentric needle electrode in the masseter muscle for the diagnosis of generalised myasthenia gravis

OBJECTIVES The purpose of our study was to show neuromuscular transmission abnormality in the masseter muscle of generalised myasthenia gravis (MG) patients and to compare motor end-plate failure of the masseter with the extensor digitorum communis (EDC) and periocular muscles. METHODS Motor end-plate function was evaluated during voluntary contraction of the masseter muscle of 20 generalised MG patients aged between 16 and 63 years, as well as 20 age-matched healthy volunteers. The mean jitter value was calculated for each group and compared. The upper limit of normal jitter was also calculated and the number of jitters exceeding this cut-off value was counted for each group for comparison. In MG patients, jitter analysis was also performed in periocular and EDC muscles along with the masseter and the number of single fibre-like potentials with abnormal jitter was counted for each muscle. All tests were performed during the same session with a concentric needle electrode (CNE). RESULTS For the masseter muscle, the mean jitter of all potential pairs was significantly higher in the patient group (24.7 ± 9.6 μs in healthy volunteers, 71.9 ± 41 μs in patients). The calculated mean jitter for the 18th highest value in healthy volunteers was 33.8 ± 5.9 μs (upper 95% confidence limit was 45.6 μs). The number of abnormal jitters (≥ 46 μs) was significantly higher in the patient group (276 out of 402 jitters) compared to healthy volunteers (10 out of 400 jitters). In the patient group, the number of single fibre-like potentials with abnormal jitter was found to be similar for the masseter, periocular and EDC muscles. CONCLUSION The masseter muscle has diagnostic importance in generalised MG. The ratio of high jitters to all of the calculated jitters in a particular muscle was similar for masseter, periocular and EDC muscles. SIGNIFICANCE Jitter analysis of the masseter muscle during voluntary contraction is easy to perform and it was found as informative as other muscles in patients with generalised MG.

Possible reflex pathway between medial meniscus and semimembranosus muscle: an experimental study in rabbits

Meniscus is a well innervated tissue with four types of receptors. These receptors are mainly concentrated at the anterior and posterior horns. Although they are intended to be a part in reflex arc, this function has not been thoroughly evaluated. We hypothesized that electrical stimulation of the normal meniscus would elicit electromyographic activity of the hamstring muscle via the reflex arc. Five adult domestic male rabbits were used in this study. Under general anesthesia, knee arthrotomy and thigh dissection were done to expose medial meniscus and semimembranosus muscle. Menisci were stimulated by Teflon-coated bipolar needle electrodes. Needles were placed in the posterior horn of the medial menisci. Two Teflon-coated monopolar needle electrodes were placed in semimembranosus muscle. A four-channel electromyograph was used for recording. Two different potentials were recorded from the target muscle. The first response had a very short distal latency and its amplitude was changing in accordance with the strength of the stimulus, suggesting that this response was being elicited by direct muscle stimulation. The second delayed response with less amplitude also appeared in some traces. The latency and the amplitude of this second response were fairly stable stating that this delayed response was being generated by a reflex pathway and seen in all subjects.

Excitability changes at brainstem and cortical levels in blind subjects.

OBJECTIVES This study was designed to search potential changes in trigemino-facial system in blind subjects by the use of relatively well-established electrophysiological methods. Excitability changes in the motor cortex were also investigated by transcranial magnetic stimulation studies (TMS) with the expectation of finding some abnormal interactions between the cortex and brainstem. METHODS Twenty blind (BS) and 13 control subjects (CoS) were included in the study. Blink reflex and its recovery with paired electrical stimulation were studied at 150, 200, 300, 400 and 500 ms interstimulus intervals (ISI). Facial F waves elicited by buccal branch stimulation were recorded from nasalis muscles. Motor cortex excitability with recordings from left first dorsal interosseus muscle was studied by using magnetically elicited silent periods and paired magnetic stimuli, subthreshold conditioning and suprathreshold test, given at ISIs of 2, 3, 4, 10, 12, 15 and 20 ms. RESULTS Blink reflex recovery was significantly reduced in BS group comparing to CoS at 400 and 500 ms ISIs. This difference between the groups was more prominent for the responses evoked by the initial stimulation side and faded away with stimulations on the contralateral side. Facial F wave amplitudes and F/M amplitude ratios were higher in BS group. In TMS studies, the early inhibitions at 2 and 4 ms were found to be significantly less in BS as compared to that of CoS. CONCLUSIONS The reduced blink reflex recovery and its fast restoration with continuing stimulation might be explained by conditioning and extinction processes which have been shown to be mainly carried out by cerebellar-brainstem pathways. Our TMS studies showed reduced intracortical inhibition in the motor cortices of BS cases and facial F wave studies revealed the possible effect of this altered excitability on the facial motor nuclei. SIGNIFICANCE Firing probabilities of facial motor neurons in BS are probably determined by the equilibrium between the low-set excitability of blink reflex interneurons and the enhanced excitability brought on by the descending motor pathways.

Prepubertal anti-Musk positive myasthenia gravis with long remission

Anti-MuSK positive myasthenia gravis (MuSK-MG) is rare prepuberty. We report a female patient with MuSK-MG starting at 3.5years with ptosis as the sole symptom for 2years. A brief period of generalization was followed by complete stable remission for 6years. Prolonged ocular symptoms and long remissions are not features of MuSK-MG, but are often seen in prepubertal onset MG. The patient then presented at age 12 with moderately severe symptoms which were almost confined to oculobulbar muscles and were unresponsive to pyridostigmine. She was dependent on corticosteroids and thymectomy did not seem to be effective. She was later noted to have tongue atrophy after a period without treatment. Our patient thus presented with features seen in many prepubertal patients, but the later course was quite typical of MuSK-MG.

Subclinical neuromuscular transmission abnormality detected by single‐fibre EMG is more pronounced in cluster headache than in migraine with aura

The aim was to investigate neuromuscular transmission (NMT) by single-fibre EMG (SFEMG) in a large series of patients having migraine with aura (MA) or cluster headache (CH). Recent studies using SFEMG have shown subclinical dysfunction of NMT in MA and CH. Forty-three patients having MA, 51 with CH and 38 healthy control subjects underwent nerve conduction studies, EMG and SFEMG during voluntary contraction of the extensor digitorum communis muscle. Twenty different potential pairs were recorded and individual, mean and total abnormal individual jitter values were calculated. The results obtained from MA patients were compared with those from CH patients. In MA patients, 32 of 860 jitters were abnormally high, whereas 73 of 1020 of the jitters showed this abnormality in CH patients. None of the control subjects, five MA patients (11.6%) and 11 CH patients (21.6%) were designated as having subclinical NMT abnormality. Thus, patients having junction dysfunction were significantly more common in the CH group. The subclinical NMT abnormality shown by SFEMG is more common in CH than in MA. These two primary headache syndromes may have some shared functional abnormality of NMT constituents which is more evident in CH.

Investigation of neuromuscular transmission in some rare types of migraine.

The aim of this study was to delineate any dysfunction of neuromuscular transmission (NMT) by single-fibre electromyography (SFEMG) in some rare types of migraine. Recent studies have shown subclinical dysfunction of NMT in migraine with aura and cluster headache by using SFEMG, whereas another recent study has shown NMT to be normal in familial hemiplegic migraine (FHM) with CACNA1A mutations. Thirty patients with rare primary headache syndromes [18 with sporadic hemiplegic migraine (SHM), six with FHM and six with basilar-type migraine (BM)] and 15 healthy control subjects without any headache complaints underwent nerve conduction studies, EMG and SFEMG during voluntary contraction of the extensor digitorum communis muscle. Ten to 20 different potential pairs were recorded and individual jitter values calculated. The results obtained from patient groups were compared with those from the normal subjects. Of 600 individual jitter values of the patients, 27 (4.5%) were abnormally high, whereas only 3/205 (1.5%) jitter values from normal subjects were abnormal. Abnormal NMT was found in 4/30 (13.3%) patients (three SHM and one BM), but in none of the control subjects. Only in SHM patients was the number of individual abnormal jitter values slightly but significantly different from normal controls. The present study demonstrates that subclinical NMT abnormality is slightly present in only SHM and BM patients, but not in FHM patients.