Cluster Analysis Revealed Two Hidden Phenotypes of Cluster Headache.

Objective: To investigate the possible subgroups of patients with Cluster Headache (CH) by using K-means cluster analysis. Methods: A total of 209 individuals (mean (SD) age: 39.8 (11.3) years), diagnosed with CH by headache experts, participated in this cross-sectional multi-center study. All patients completed a semi-structured survey either face to face, preferably, or through phone interviews with a physician. The survey was composed of questions that addressed sociodemographic characteristics as well as detailed clinical features and treatment experiences. Results: Cluster analysis revealed two subgroups. Cluster one patients (n = 81) had younger age at diagnosis (31.04 (9.68) vs. 35.05 (11.02) years; p = 0.009), a higher number of autonomic symptoms (3.28 (1.16) vs. 1.99(0.95); p < 0.001), and showed a better response to triptans (50.00% vs. 28.00; p < 0.001) during attacks, compared with the cluster two subgroup (n = 122). Cluster two patients had higher rates of current smoking (76.0 vs. 33.0%; p=0.002), higher rates of smoking at diagnosis (78.0 vs. 32.0%; p=0.006), higher rates of parental smoking/tobacco exposure during childhood (72.0 vs. 33.0%; p = 0.010), longer duration of attacks with (44.21 (34.44) min. vs. 34.51 (24.97) min; p=0.005) and without (97.50 (63.58) min. vs. (83.95 (49.07) min; p = 0.035) treatment and higher rates of emergency department visits in the last year (81.0 vs. 26.0%; p< 0.001). Conclusions: Cluster one and cluster two patients had different phenotypic features, possibly indicating different underlying genetic mechanisms. The cluster 1 phenotype may suggest a genetic or biology-based etiology, whereas the cluster two phenotype may be related to epigenetic mechanisms. Toxic exposure to cigarettes, either personally or secondarily, seems to be an important factor in the cluster two subgroup, inducing drug resistance and longer attacks. We need more studies to elaborate the causal relationship and the missing links of neurobiological pathways of cigarette smoking regarding the identified distinct phenotypic classes of patients with CH.

Reduced expression of inflammasome complex components in cluster headache.

BACKGROUND: The involvement of inflammation in the pathophysiology of cluster headache (CH) has been suggested, with a role implied for interleukin (IL)-1ß. We aimed to measure peripheral blood expression levels of IL-1ß-inducing systems, the inflammasome complex, and nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) signaling, and investigate their values as putative biomarkers in CH. METHODS: In this cross-sectional study conducted in the Headache Unit of Istanbul University, Turkey, blood mononuclear cells (PBMCs) and sera were collected from 30 patients with episodic migraine, 4 with chronic CH, and 47 healthy individuals. Levels of inflammasome complex components (NLRP1, NLRP3, caspase 1, and ASC), end products of inflammasome complex activity (IL-1ß, IL-18, and nitric oxide synthase isoforms), neuron-specific enolase, other inflammatory factors (NF-κB, HMGB1, and s100b), and anti-inflammatory IL-4 were measured by real-time quantitative polymerase chain reaction and/or enzyme-linked immunosorbent assay. RESULTS: NLRP3 expression levels were significantly reduced in PBMC samples of patients with CH, obtained during CH attacks (n = 24) or headache-free (out of cycle) episodes (n = 10). CH-attack patients showed greater expression levels of IL-1ß (2-ΔΔCT median [25th-75th percentile], 0.96 [0.66-1.29 vs. 0.52 [0.43-0.73]) and NF-κB (1.06 [0.66-3.00] vs. 0.62 [0.43-1.19]) in PBMCs but not in sera compared with headache-free CH patients. However, these differences did not attain statistical significance (p = 0.058 and p = 0.072, respectively). Moreover, NLRP1 (52.52 [35.48-67.91] vs. 78.66 [54.92-213.25]; p = 0.017), HMGB1 (11.51 [5.20-15.50] vs. 13.33 [8.08-18.13]; p = 0.038), S100b (569.90 [524.10-783.80] vs. 763.40 [590.15-2713.00]; p = 0.013), NSE (11.15 [6.26-14.91] vs. 13.93 [10.82-19.04]; p = 0.021), nNOS (4.24 [3.34-12.85] vs. 12.82 [4.52-15.44]; p = 0.028), and eNOS (64.83 [54.59-91.14] vs. 89.42 [61.19-228.40]; p = 0.034) levels were lower in patients with three or more autonomic manifestations (n = 9). No correlation was found between inflammation factors and clinical parameters of CH. CONCLUSION: Our results support the involvement of the IL-1ß system in attacks of CH. However, the components of the inflammasome complex are suppressed in the peripheral blood and do not appear to play a role in the pathophysiology of CH. These findings argue against a potential biomarker value of the inflammasome complex in CH.

Near-tendon single-fiber electromyography in patients with myopathy.

We investigated how properties of single-fiber action potentials (SFAP) and jitter changed in different recording sites in patients with myopathy and controls. SFAP and jitter were recorded using a disposable single-fiber electrode in the biceps brachii muscle of three patients and controls in two sites: i) near the end-plate zone (EPZ), ii) near the tendon. SFAP and spike duration were longer in patients than in controls. Moving the needle away from the EPZ did not change the electrophysiologic parameters. Longer SFAP duration may reflect slow velocity in muscle fibers and serve as an indicator for small fiber size in myopathy.

Bi-allelic variants in neuronal cell adhesion molecule cause a neurodevelopmental disorder characterized by developmental delay, hypotonia, neuropathy/spasticity.

Cell adhesion molecules are membrane-bound proteins predominantly expressed in the central nervous system along principal axonal pathways with key roles in nervous system development, neural cell differentiation and migration, axonal growth and guidance, myelination, and synapse formation. Here, we describe ten affected individuals with bi-allelic variants in the neuronal cell adhesion molecule NRCAM that lead to a neurodevelopmental syndrome of varying severity; the individuals are from eight families. This syndrome is characterized by developmental delay/intellectual disability, hypotonia, peripheral neuropathy, and/or spasticity. Computational analyses of NRCAM variants, many of which cluster in the third fibronectin type III (Fn-III) domain, strongly suggest a deleterious effect on NRCAM structure and function, including possible disruption of its interactions with other proteins. These findings are corroborated by previous in vitro studies of murine Nrcam-deficient cells, revealing abnormal neurite outgrowth, synaptogenesis, and formation of nodes of Ranvier on myelinated axons. Our studies on zebrafish nrcamaΔ mutants lacking the third Fn-III domain revealed that mutant larvae displayed significantly altered swimming behavior compared to wild-type larvae (p < 0.03). Moreover, nrcamaΔ mutants displayed a trend toward increased amounts of α-tubulin fibers in the dorsal telencephalon, demonstrating an alteration in white matter tracts and projections. Taken together, our study provides evidence that NRCAM disruption causes a variable form of a neurodevelopmental disorder and broadens the knowledge on the growing role of the cell adhesion molecule family in the nervous system.

Automatic detection of F waves and F-MUNE in two types of motor neuron diseases.

INTRODUCTION/AIMS: Motor unit number estimation by F waves (F-MUNE) is an uncommonly used MUNE technique. Our aim in this study was to assess the sensitivity of F-MUNE values elicited with newly developed software in motor neuron diseases. METHODS: F waves were recorded by 300 submaximal stimuli from abductor digiti minimi and abductor pollicis brevis muscles of 35 patients with amyotrophic lateral sclerosis, 18 with previous poliomyelitis, and 20 controls. The software determined the surface motor unit action potentials (sMUAPs) and calculated the F-MUNE values. Compound muscle action potential scans were also recorded to obtain MScanFit. RESULTS: The sMUAP amplitudes were higher and F-MUNE values were lower in both muscles of the patients when compared with controls. F-MUNE values could distinguish patients from controls. Significant correlations were found between F-MUNE and MScanFit in the patient groups. DISCUSSION: The new F-MUNE software offered promising results in revealing motor unit loss caused by motor neuron diseases.

Evaluation of Patients with Post-Traumatic Hearing Loss: A Retrospective Review of 506 Cases.

OBJECTIVES: The purpose of the study is to evaluate the audiological, radiological, and examination findings of patients who have been treated for hearing loss (HL) due to head trauma and evaluated in terms of causality to reveal current data, and to highlight the steps to be taken. METHODS: We retrospectively reviewed the reports of cases that had applied for disability with HL due to head injury and had been evaluated by the Forensic Medicine Institute between January 01, 2009 and January 01, 2019. RESULTS: Of the total cases of head trauma, 52.42% were not vehicle-related, and cases were observed to be concentrated in the age range of 19-40 (55.92%; n = 283). Although otorrhagia/otorrhea was the most common finding in all types of trauma, TM perforation was the most common finding in blast-type injuries. While the rate of newly developed unilateral HL was 84.2%, 72.7% of the patients had sensorineural hearing loss (SNHL) . Temporal bone fractures were detected in 59.3% of the cases, and 60.2% of them were of the longitudinal type. Facial paralysis (FP) was detected in 28.6% of the cases, and there was no statistically significant difference between the groups in terms of HL compared to those without FP. Other nerve palsy was detected in 4.9% of the cases, and N. Abducens paralysis was the most common. CONCLUSION: Accompanying intracranial nerve injury, temporal fracture, and intracranial pathologies are considerably high in patients who develop HL following head trauma. The first examination requires a multidisciplinary approach to guide future disability applications.

Olfactory dysfunction in patients with cluster headache.

OBJECTIVE: Cluster headache (CH) is a primary headache characterized by strictly unilateral, short-lasting severe headache attacks accompanied by at least one ipsilateral autonomic symptom. Our study aimed to determine whether CH patients had olfactory dysfunction and to correlate it with clinical characteristics. MATERIALS AND METHODS: Twenty patients and 57 healthy volunteers were included in the study. All participants were examined in the otorhinolaryngology outpatient clinics to exclude other clinical problems causing olfactory dysfunction. The Sniffin' Sticks test was performed, and threshold (T), discrimination (D), identification (I) scores, and TDI global olfactory score were evaluated. RESULTS: The CH patients had significantly lower threshold scores than healthy controls (6.9 ± 1.70 vs. 7.8 ± 1.08, p = 0.007). The mean threshold scores of CH patients during in-bout (n = 9) were significantly lower than CH patients during out-of-bout (n = 11) in subgroup analysis (5.9 ± 1.16 vs. 7.6 ± 1.76, p = 0.038). CH patients with left-sided headache had significantly lower discrimination scores compared to CH patients with right-sided headache (12.8 ± 1.24 vs. 14.4 ± 1.51, p = 0.03). CONCLUSION: There is marked impairment in olfactory function in CH patients compared to healthy controls.

Corticomotor Excitability in Two Kinds of Motor Neuron Diseases: A Study on the Patients With Amyotrophic Lateral Sclerosis and Poliomyelitis Survivors.

PURPOSE: To examine upper motor neuron functions comparatively in patients with amyotrophic lateral sclerosis (ALS) and poliomyelitis survivors using transcranial magnetic stimulation (TMS) methods. METHODS: Single- and paired-pulse TMS with conventional methods and the triple-stimulation technique were performed by recording from the abductor digiti minimi and abductor pollicis brevis muscles in 31 patients with ALS, 18 patients with poliomyelitis survivors, and 21 controls. Nine patients were diagnosed as having postpoliomyelitis syndrome after a 6-month follow-up. RESULTS: Triple-stimulation technique and some of conventional TMS studies were able to distinguish ALS from both poliomyelitis survivors and controls. A reduced ipsilateral silent period in abductor pollicis brevis muscles was the only parameter to show a significant difference when comparing thenar and hypothenar muscles in ALS. No significant difference was present in any TMS parameters between the postpoliomyelitis syndrome and non-postpoliomyelitis syndrome groups. CONCLUSIONS: Conventional TMS and particularly triple-stimulation technique studies are helpful in disclosing upper motor neuron dysfunction in ALS. The results of this study might favor the cortical hypothesis for split hand in ALS, but they revealed no significant indication for upper motor neuron dysfunction in postpoliomyelitis syndrome.

Headache characteristics in COVID-19 pandemic-a survey study.

BACKGROUND: Headache is the most common COVID-19-related neurological symptom. We aimed to reveal diagnostic clues of headache for COVID-19 infection and to investigate the course of primary headaches during the pandemic. METHODS: We developed a detailed web-based questionnaire screening the characteristics and course of headaches besides clinical COVID-19 features. The participants were grouped according to being diagnosed with COVID-19 infection or not, and having previous or new-onset headaches. The COVID-19 related headache features and their associations with other clinical features were investigated. A binary logistic regression model was performed to differentiate the characteristics of headache related to COVID-19. FINDINGS: A total of 3458 participants (2341 females;67.7%, 1495 healthcare workers;43.2%) with a mean age of 43.21 ± 11.2 years contributed to the survey. Among them, 262 participants had COVID-19 diagnosis and 126 (48.1%) were male. The rate of males in the group without COVID-19 was 31% (991 out of 3196 participants) showing significant gender difference between groups (p < 0.000). COVID-19 related headaches were more closely associated with anosmia/ageusia and gastrointestinal complaints (p < 0.000 and p < 0.000), and showed different characteristics like pulsating, pressing, and even stabbing quality. Logistic regression analyses showed that bilateral headache, duration over 72 h, analgesic resistance and having male gender were significant variables to differentiate COVID-19 positive patients from those without COVID-19 (p = 0.04 for long duration and p < 0.000 for others). A worsening of previous primary headaches due to the pandemic-related problems was not reported in the majority of patients. INTERPRETATION: Bilateral, long-lasting headaches, resistance to analgesics and having male gender were more frequent in people with COVID-19 in conjunction with anosmia/ageusia and gastrointestinal complaints. These features may be helpful for diagnosing the headache related to COVID-19 during the pandemic.

Electrophysiological Findings of Subclinical Lower Motor Neuron Involvement in Degenerative Upper Motor Neuron Diseases.

INTRODUCTION: The present study is an examination of possible subclinical involvement of lower motor neuron (LMN) in patients with primary lateral sclerosis (PLS) and hereditary spastic paraparesis (HSP) electrophysiologically. METHODS: Nine PLS patients and 5 HSP patients were prospectively analyzed. Jitter measurement with concentric needle electrode (25 mm, 30 G) (CN-jitter) recorded from right extensor digitorum muscle during voluntary contraction with 1 kHz high-pass frequency filter set. European Myelopathy Score (EMS) was used to evaluate disability. The relationship between disability score and jitter values was investigated. RESULTS: HSP patients had suffered from the disease for longer period of time (p<0.001). Mean jitter values of patients with PLS and HSP were 26.5±12.1 µs and 30.8±34.8 µs, and the number of individual high jitters (>43 microseconds) observed in the PLS and HSP groups was 16/180 and 9/100, respectively without a significant intergroup difference. The ratio of patients with an abnormal jitter study were higher in HSP group (60%) compared to PLS (22%) (p<0.05). Potential pairs with blocking were present in HSP group (7 of 100 potential pairs) but not seen in PLS patients. EMS values were significantly lower in patients having potential pairs with high jitter and blocking compared to those without high jitter and blocking. CONCLUSION: The present study has demonstrated that early signs of LMN dysfunction can be detected electrophysiologically by CN-jitter in patients with UMN involvement. These electrophysiological findings in these patients with longer disease duration and lower clinical scores may be explained by spreading of the disease to LMNs or transsynaptic degeneration and its contribution in disease progression.

Sex Differences of Migraine: Results of a Nationwide Home-based Study in Turkey.

INTRODUCTION: The prevalence of migraine was found to be more than three-fold higher in women as compared with men, and in addition to differences in prevalence rates, the characteristics and associated features might also differ between the sexes. The aim of this study was to compare sex-specific features of migraine and demographic parameters in a nationwide population-based study in Turkey. METHODS: Among 5323 subjects, a total of 871 patients who were diagnosed as having definite migraine according to the diagnostic criteria of the International Classification of Headache Disorders-III (ICHD-III) were included in our study. The demographic characteristics, associated features, and triggers of migraine were examined with regard to sex. RESULTS: The study group comprised 640 women (73.5%) and 231 men (26.5%), with a female to male ratio of 2.8:1. Attack duration, mean migraine disability assessment scores (MIDAS), frequencies of nausea, vomiting, osmophobia, vertigo/dizziness, and allodynia were found significantly different between women and men. When we compared these parameters between men and postmenopausal women, all these parameters were still significant except nausea. Odor was statistically more frequent as a reported trigger in women, whereas excessive sleep was a statistically more frequent triggering factor in men. The rates of depression and allergy were significantly higher in women when compared with men. CONCLUSION: Longer attack duration, higher MIDAS scores, and the frequencies of nausea, vomiting, osmophobia, vertigo/dizziness, and allodynia were more significant in women and this variance in sex persisted after menopause. Also, some trigger factors and co-morbidities differed between the sexes. These findings might result from complex genetic factors besides sociocultural influences, biologic, and sociocultural roles. Future studies should continue to explore biologic and genetic factors with respect to sex in migraine.

Compound muscle action potential scan and MScanFit motor unit number estimation during Wallerian degeneration after nerve transections.

BACKGROUND: Compound muscle action potential (CMAP) scan and MScanFit have been used to understand the consequences of denervation and reinnervation. This study aimed to monitor these parameters during Wallerian degeneration (WD) after acute nerve transections (ANT). METHODS: Beginning after urgent surgery, CMAP scans were recorded at 1-2 day intervals in 12 patients with ANT of the ulnar or median nerves, by stimulating the distal stump (DS). Stimulus intensities (SI), steps, returners, and MScanFit were calculated. Studies were grouped according to the examination time after ANT. Results were compared with those of 27 controls. RESULTS: CMAP amplitudes and MScanFit progressively declined, revealing a positive correlation with one another. SIs were higher in WD groups than controls. Steps appeared or disappeared in follow-up scans. The late WD group had higher returner% than the early WD and control groups. CONCLUSIONS: MScanFit can monitor neuromuscular dysfunction during WD. SIs revealed excitability changes in DS.

Changes in motor unit bioelectrical activity recorded at two different sites in a muscle.

INTRODUCTION: The objective of this study was to compare the properties of bioelectrical signals of motor units recorded at different sites in the muscles of controls, patients with myopathy and patients with motor neuron disease (MND). METHODS: Five controls, 10 patients with myopathy and 11 patients with MND were included. Electrophysiologic tests were performed in the biceps brachii (BB) muscle from two recording sites. Site 1 was near the belly of the muscle and Site 2 was 5cm distal from Site 1, near the tendon. Multi-motor unit potential (MUP) analysis, jitter analysis, and peak number count were calculated from the signals recorded using a concentric needle electrode (CN). RESULTS: At Site 2, duration was longer, number of phases was higher and amplitudes were smaller in MUPs compared with those recorded at Site 1. This significant difference between recording site and patient groups was related to neurogenic muscles. Jitter analysis showed no significant difference except an intergroup difference between the patient groups and controls. The peak number calculated using the CN was greater when recorded from Site 1 in concordance with MUP analysis. CONCLUSION: Duration of MUP was longer and amplitude was smaller when the recording electrode was placed distally along the muscle near the tendon in neurogenic muscles, probably related to increased temporal dispersion. However, changing the position of the needle did not provide further information in distinguishing myogenic muscles.

Repeater F-waves in amyotrophic lateral sclerosis: Electrophysiologic indicators of upper or lower motor neuron involvement?

OBJECTIVE: To extract insight about the mechanism of repeater F-waves (Frep) by exploring their correlation with electrophysiologic markers of upper and lower motor neuron dysfunction in amyotrophic lateral sclerosis (ALS). METHODS: The correlations of Frep parameters with clinical scores and the results of neurophysiological index (NI), MScanfit MUNE, F/M amplitude ratio (F/M%), single and paired-pulse transcranial magnetic stimulation (TMS), and triple stimulation technique (TST) studies, recorded from abductor digiti minimi (ADM) and abductor pollicis brevis (APB) muscles of 35 patients with ALS were investigated. RESULTS: Frep parameters were correlated with NI and MScanfit MUNE in ADM muscle and F/M% in both muscles. None of the Frep parameters were correlated with clinical scores or TST and TMS measures. While the CMAP amplitudes were similar in the two recording muscles, there was a more pronounced decrease of F-wave persistence in APB, probably heralding the subsequent split hand phenomenon. CONCLUSION: Our findings suggest that the presence and density of Freps are primarily related to the degree of lower motor neuron loss and show no correlation with any of the relatively extensive set of parameters for upper motor neuron dysfunction. SIGNIFICANCE: Freps are primarily related to lower motor neuron loss in ALS.

Parameters derived from compound muscle action potential scan for discriminating amyotrophic lateral sclerosis-related denervation.

INTRODUCTION: The objective of this study was to determine compound muscle action potential (CMAP) scan parameters and MScanFit motor unit number estimation (MUNE) in patients with amyotrophic lateral sclerosis (ALS) and to compare the results in the abductor pollicis brevis (APB) to those in the abductor digiti minimi (ADM). METHODS: CMAP scans were recorded from the APB and ADM in 35 patients with ALS and 21 controls. MScanFit MUNE, neurophysiological index (NI), step%, returner%, and D50 were calculated. RESULTS: CMAP scan parameters including the returner%, MScanFit MUNE, and NI can distinguish ALS with high sensitivity and specificity. The electrophysiological parameters, with the exception of D50 (the number of largest consecutive differences of recorded responses generating 50% of maximum CMAP), showed more pronounced changes in the APB than in the ADM, even though most of the patients had normal APB/ADM amplitude ratios. DISCUSSION: CMAP scan parameters and MScanFit MUNE can be used in the evaluation of denervation and reinnervation and may herald the "split hand" in ALS.

Assessment of the Olfactory Function in Patients With Idiopathic Intracranial Hypertension Using the Sniffin' Sticks Test: A Case-Control Study.

OBJECTIVE: Despite the lack of recognition in clinical practice, there is increasing evidence that patients with idiopathic intracranial hypertension may suffer from hyposmia. The current case-control study aims to evaluate olfactory dysfunction in a large series of patients with idiopathic intracranial hypertension. METHODS: All subjects, 44 idiopathic intracranial hypertension patients and 57 healthy controls, underwent olfactory function assessment using standardized "Sniffin' Sticks" test at a tertiary referral center of a university hospital. Threshold, discrimination, identification, and total threshold-discrimination-identification scores have been determined and analyzed statistically. RESULTS: Idiopathic intracranial hypertension patients had significantly lower threshold (6.5 [3.69] vs 8 [1.88], P < .001, 95% CI [-2.250, -0.750]) and threshold-discrimination-identification scores (29.75 [5.56] vs 32.5 [5.25], P = .003, 95% CI [-4.250, -0.750]). Twenty-five patients (57%) were diagnosed with hyposmia. Test scores of patients with active idiopathic intracranial hypertension (n = 18) were not statistically different from patients with inactive disease (n = 26), except for discrimination score (14 [2.50] vs 11 [2.25], P = .005, 95% CI [-3.000, -1.000]). Although idiopathic intracranial hypertension patients with a cerebrospinal fluid opening pressure of ≥330 mmH2 O had lower test scores, the difference was significant only for total threshold-discrimination-identification scores (28.5 [5.50] vs 30.5 [4.38], P = .044, 95% CI [0.750, 5.500]). Multiple regression analysis revealed that test scores were related to disease activity, cerebrospinal fluid opening pressure, papilledema, headache, and medication. CONCLUSION: Our clinical study revealed significant olfactory dysfunction in patients with idiopathic intracranial hypertension compared with healthy controls. Future research should employ larger samples to search for usability of olfactory testing in clinical management of patients with idiopathic intracranial hypertension.

Trigeminal Hyperexcitability in Idiopathic Intracranial Hypertension: A Blink Reflex Study.

PURPOSE: To compare blink reflex examination findings in patients with idiopathic intracranial hypertension (IIH) with migraineurs and controls and to investigate the role of blink reflex data in the follow-up by evaluating their relationship with clinical symptoms. METHODS: We included 23 patients with IIH, 15 migraineurs, and 16 controls. Blink reflex was studied with bilateral paired stimulations, and the 2R2/1R2 and 2R2c/1R2c area ratios, 1R2, 2R2, 1R2c, and 2R2c latencies were compared (R2:ipsilateral R2 response, R2c:contralateral R2 response). RESULTS: 2R2/1R2 area ratios at 200, 300, and 500 ms interstimulus intervals were significantly higher in the IIH group compared with the controls, and significantly higher 2R2/1R2 area ratio at 200 ms interstimulus interval was also detected in the IIH group compared with the migraine group. Significantly shorter 2R2 latencies were found at 500 and 800 ms interstimulus intervals in IIH group compared with the migraine group and controls. Significantly higher 2R2/1R2 area ratios at 500, 800 ms, and 2R2c/1R2c area ratios at 300, 500, and 800 ms were detected in patients with IIH in remission compared with patients with attacks. CONCLUSIONS: The significant increase in R2 recovery rates in patients with IIH in remission suggests that there is an increased excitability in the trigeminal pathways. Our findings may provide a new perspective for IIH mechanism related to trigeminal hyperexcitability and evaluating new management strategies.

Epilepsia partialis continua: Correlation of semiology, outcome and electrophysiologic features.

OBJECTIVE: Epilepsia partialis continua (EPC) is a special form of cortical epilepsy. Several studies have described the ictal and interictal electroencephalography (EEG) findings in patients with EPC; however, lateralizing and localizing values of these findings have been evaluated rarely. This study investigated the correlation of semiologic and EEG findings, and outcomes in patients with EPC. PATIENTS AND METHODS: Clinical and EEG findings and outcomes, and their correlations were studied prospectively in 15 patients who were diagnosed as having EPC upon presentation to Istanbul Medical Faculty Hospital between January 2010 and April 2014, and retrospectively in 5 previously evaluated patients. RESULTS: EEG findings were lateralizing in 11 (47.8%) of the overall 23 EEG recordings, 7 (30.4%) of which were also localizing. Eleven (55%) of the 20 patients had poor prognosis. Patients with interictal lateralizing EEG findings had better outcomes compared with patients who had interictal non-lateralizing EEG findings (P = 0.016). Periodic epileptiform discharges (PEDs) were noted in the 6 EEGs (3 ictal EEGs and 3 interictal EEGs) of 5 patients, all of whom had poor outcomes (P = 0.04). All four patients with false lateralizing EEG findings had poor outcomes. CONCLUSION: EEG has low lateralizing and localizing value in EPC but patients with interictal lateralizing EEG findings have better outcomes, which may be used as a prognostic tool in EPC. The presence of PEDs and false lateralizing findings in EEG might be associated with poor prognosis in EPC.

Repetitive nerve stimulation and jitter measurement with disposable concentric needle electrode in newly diagnosed myasthenia gravis patients.

INTRODUCTION: The aim of this study was to define the diagnostic accuracy of concentric needle (CN)-jitter in newly diagnosed myasthenia gravis (MG) patients and to compare CN-jitter with repetitive nerve stimulation. METHODS: In 30 MG patients, repetitive nerve stimulation in 4 muscles (orbicularis oculi, nasalis, trapezius and abductor digiti minimi) and CN-jitter of extensor digitorum (ED) and frontalis muscles were evaluated. RESULTS: Twenty-eight of 30 patients (93%) had high jitter in at least one muscle. Repetitive nerve stimulation was abnormal in 23 of the patients (77%). Eighty-six percent of the patients in whom repetitive nerve stimulation test was negative could be diagnosed with CN-jitter. The most frequent muscle showing abnormal decrement was orbicularis oculi. The results of CN-jitter were similar between patients with different serological groups. Of 13 patients with generalized weakness, all had high jitter in both muscles studied whereas of 17 patients only with ocular weakness, 15 had high jitter in at least one muscle studied. CONCLUSION: Abnormal RNS was present in 77% of newly diagnosed MG patients, being less than CN-jitter (93%) but more than antibody positivity (73.3%).