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Epileptic vertigo is often a diagnostic problem. We aimed to present the clinical and electrophysiological features of patients with epileptic vertigo with a view to addressing the pathophysiology of this rare aura symptom. Nine epileptic vertigo patients were included in the study. All patients were subjected to neuro-otologic examination, interictal electroencephalogram (EEG), audiogram, cervical vestibular evoked myogenic potential testing (cVEMP), video head impulse testing (vHIT) and brain magnetic resonance imaging (MRI). Eight patients described their aura as epileptic vertigo and one as dizziness. In three patients, auditory hallucinations preceded epileptic vertigo. The semiology of epileptic vertigo was true vertigo in five patients, vertigo with nausea in two patients and vertigo with hearing loss in one patient. Two patients suffered from focal seizures, and in seven patients the seizures were evaluated as focal to bilateral tonic-clonic seizures. MRI was normal in all patients. EEG was abnormal in all cases and showed high-voltage spike or spike-slow-wave complexes, or both, located more frequently in the temporal region, more left than right. On vHIT examination, abnormal responses were recorded bilaterally or unilaterally in five patients. Similarly, cVEMP revealed no response bilaterally or unilaterally in five patients. In three patients, the side of no response to cVEMP corresponded to the side of epileptiform pathology based on EEG. Two patients with bilateral abnormalities on EEG showed bilateral abnormalities either on cVEMP or vHIT, or on both. Taken together, these findings support the involvement of the brainstem connections of the peripheral vestibular system in vertiginous epilepsy. The pathological results of vestibular tests in the majority of our patients, combined with the EEG abnormalities, support the hypothesis of system epilepsies which is based on the dysfunction of specific neural systems.

Pseudo-vestibular neuritis is a clinical diagnosis for patients presenting with acute vestibular syndrome due to a central pathology.We reported a case of multiple sclerosis characterized by pseudo-vestibular neuritis. Our case was a 32-year-old male patient. The patient, who was diagnosed with multiple sclerosis in September 2019, came to the emergency clinic in January 2020 with the complaint of severe vertigo, vomiting-nausea. A newly developed demyelinating plaque was detected in the left vestibular nucleus in cranial MRI. The patient had no hearing loss. On examination of the patient, nystagmus findings supporting peripheral vestibular involvement were present on the left side. Neurologic examination showed left-sided hyperactive deep tendon reflexes, Achilles clonus, dysmetria, ataxia to the left and plantar reflex with extensor response on the left. Video head impulse test and cervical evoked myogenic potential tests were performed. Vestibular hypofunction was present on the left side. Steroid pulse therapy was administered as 1000 mg/day, i.v for 7 days. After treatment, his complaints decreased. In addition, there was an improvement in examination findings.Multiple sclerosis is shown to be an etiological factor in patients with pseudo-vestibular neuritis.

Introduction:Vestibular migraine is a clinical entity characterised by dizziness. Additionally, it has no proven treatment options, and the goal is to control vestibular symptoms by reducing migraine attacks. This study aimed to investigate the effect of onabotulinum toxin type A in reducing vestibular migraine symptoms.Methods:Between 2014 and 2019, the charts of 80 patients with migraine who received onabotulinum toxin type A treatment were collected. Among them, 22 patients who met the criteria of vestibular migraine were included in the study. The treatment outcomes of these patients were investigated. Migraine Disability Assessment scale (MIDAS), Visual Analogue scale (VAS) and Dizziness Handicap index (DHI) were used for evaluation. Pre-treatment and post-treatment values were compared.Results:The mean age of the patients was 41.5 years. When MIDAS, DHI and VAS scores were compared, the post-treatment scores were found to be statistically significantly lower than the pre-treatment scores (p

Zusammenfassung Zielsetzung Ziel dieser Studie ist es, die Inzidenz von Large Fibre Neuropathy (LFN) bei Fibromyalgie (FM) -Patienten mit peripheren Neuropathie-Beschwerden zu untersuchen und den Einfluss von LFN und FM auf Schmerzen, Lebensqualität, Schlafqualität, Behinderung, und depressive Symptome. Methoden Zwischen Juni 2018 und Februar 2019 wurden insgesamt 104 Patienten (67 Frauen, 37 Männer; Durchschnittsalter: 52,21±9,53 Jahre; Spanne 31–74 Jahre) mit der Diagnose FM in die Studie eingeschlossen. Die Patienten wurden in 2 Gruppen eingeteilt: Gruppe 1, einschließlich Patienten mit Polyneuropathie (PNP) (n=48) und Gruppe 2, einschließlich Nicht-PNP-Patienten (n=54). Gruppe 1 wurde auch weiter in 2 Untergruppen als sensorische PNP (n=28) und sensomotorische PNP (n=20) unterteilt. Alle Patienten wurden anhand der Visual Analog Scale (VAS), des Fibromyalgia Impact Questionnaire (FIQ), des Short Form-36 (SF-36), des Pittsburgh Sleep Quality Index (PSQI) und des Beck Depression Inventory (BDI) bewertet. Ergebnisse Es gab keinen statistisch signifikanten Unterschied in den demografischen Merkmalen zwischen den Gruppen (p>0,05). Es gab einen statistisch signifikanten Unterschied in den VAS-, SF-36-, BDI-, FIQ- und PSQI-Werten zwischen Gruppe 1 und Gruppe 2 (p0,05). Schlussfolgerungen Unsere Studienergebnisse zeigen, dass FM mit LFN die Schmerzen, die Lebensqualität, die Schlafqualität, die Behinderung und die depressiven Symptome negativ beeinflusst, gegenüber denjenigen ohne LFN, was auf die Bedeutung des Neuropathiemanagements bei FM-Patienten hinweist.

Autoimmune encephalitis (AIE) and paraneoplastic syndromes (PNS) are both rare groups of neurological diseases that are difficult to diagnose.We aimed to determine the common and distinct aspects of these two aetiologies of encephalitis as well as the characteristics of our patient group.We respectively analysed the records of the patients including symptoms, demographic features, neurological examination, cranial-magnetic-resonance-imaging (MRI), electroencephalography (EEG) findings, cerebrospinal fluid results (CSF) findings. Autoimmune/paraneoplastic autoantibodies in blood and/or CSF were all documented.Forty-six patients fulfilled the diagnostic criteria. Thirty-eight of them were diagnosed with AIE, and 8 of them were diagnosed with PNS. The PNS group had higher nonconvulsive status epilepticus than the AIE (2/8 vs 0/38; p=0.027). PNS patients were diagnosed with a malignancy in their follow-ups more than those in the AIE group [4/38 vs 8/8] (p0.001). When the symptoms of antibody-positive and negative patients were compared in the AIE group, the rates of consciousness/memory problems (13/15 vs 11/23; p=0.020) and speech impairment (8/15 vs 2/23; p=0.004) were significantly higher in patients without antibodies (n: 15) than in antibody-positive patients (n: 23). In antibody-negative groups, the rates of memory problems in neurological examination (13/15 vs 12/23 p=0.028) and temporal findings on electroencephalography were more prominent than antibody-positive groups (1/23 vs 5/15; p=0.027). The number of patients with cerebellar signs was higher in antibody-positive patients (6/23 vs 0/15; p=0.038).Although the positivity of autoantibodies is critical in the diagnosis of AIE and PNS, even minor differences in clinical and laboratory findings of patients are helpful in the diagnosis, especially in the autoantibody-negative patients. Comparing the data with other population studies has shown that several inherited and environmental factors may contribute to the pathophysiology of AIE and PNS, as well as clinical and laboratory differences.

The aim of this study was to determine the type, etiology and the rates of epilepsy and to identify accompanying cognitive and behavioral problems in patients with epileptiform abnormalities in the posterior cerebral localization. In this study, 3500 patients with at least one EEG record at the EEG Laboratory of Clinical Child Neurology Department of Cerrahpasa Medical Faculty of Istanbul University were evaluated in 2014-2015. Three hundred forty-six patients were included in the study. Of the 346 patients included in the study, 42.4% were female and 57.5% were male. The age range of the cases was 1-21 (mean: 8.7) years. Epileptiform activities were observed in post TPO region isolatedly in 58,95% (n = 204), post-TPO epileptiform focus with focal epileptiform focus in different localizations in 31.21% (n = 108), generalized epileptiform activity with more than one epileptiform focus in 9.8% (n= 34). In the period of EEG examinations 250 (72.25%) patients had a history of epileptic seizures and / or epilepsy, while 96 (27.74%) had non-epileptic clinical conditions such as behavioral disorder and autism. In the EEG recordings we examined, sharp and spike wave activities were frequently observed in the post TPO region isolatedly. We believe that this study, which investigated the relationship between focal epilptiform activity in post TPO region and different clinical conditions, will serve as an example for other studies.

The rare syndrome of perioral myoclonia with absences (POMA) is described as a specific type of idiopathic generalized epilepsy in which absence seizures are accompanied by prominent perioral myoclonus as a consistent symptom. We present a 52-year-old man who was referred to our department due to treatment-resistant epilepsy. Typical seizures were described as rhythmic twitching of the lips which started at six years old, and his first convulsive seizure occurred at around 20 years old. Based on video-EEG recordings, we present two distinct EEG patterns accompanied by slight differences in clinical manifestations, which appear to be atypical of POMA. Firstly, consciousness was preserved during seizures, with no manifestation of absences. Secondly, regarding the EEG features, in some of the seizures, the perioral motor symptoms were tonic rather than myoclonic. The defining features of POMA are discussed in relation to this case.

Background Vascular dysfunction dominates the clinical picture of peripheral autonomic neuropathy in lower extremity. Patients and Methods We have studied functional changes of leg vasculature in 30 patients with chronic ulceration due to peripheral autonomic neuropathy between clinical stages 1 and 3. They suffered from lower extremity wounds. After sympathetic skin response test, pedal arterial blood flow analysis including peak systolic velocity (PSV) and pulsatility index (PI) was made by duplex ultrasonography (DUS) in involved legs. Vascular anatomy of leg was also examined by magnetic resonance angiography. Results The mean PSV value was found 58.32 cm/s in stage 1, 35.31 cm/s in stage 2, and 15.71 cm/s in stage 3. The mean PI value was observed 1.17 in stage 1, 1.43 in stage 2, and 1.87 in stage 3. In chronic stage 3, three patients had inadequate arterial blood supply and recurrent ulcer. Conclusions We suggest that reduced sympathetic activity due to small fiber neuropathy causes temporal variations in leg blood flow. There was a nonlinear relationship between vascular functional changes and stages of disease with increased, intermediate, and decreased blood flow, respectively. DUS assessment of pedal arteries contributed to differentiation of clinical stages and permitted vascular evaluation in the course of peripheral autonomic neuropathy.

Purpose To investigate the possible relationship between eye closure sensitivity (ECS) and genetic generalized epilepsy (GGE) in terms of epilepsy syndromes, photosensitivity (PS), and prognosis. Method One hundred and twenty-three patients diagnosed with GGE were classified according to epilepsy syndromes. Among them, ECS and PS were detected in repeated video-EEGs. In patients with ECS, the impact of sleep deprived EEG on awakening on ECS was evaluated. To explore the ECS as a possible accurate predictor designating the prognosis in GGEs, we defined the ECS rate in poor prognosis group (n = 21) and in patients without antiseizure drug (ASD) treatment and seizure-free for at least for 2–5 years (n = 20). Results ECS was found in almost all types of GGE but at different rates. ECS was detected in all groups with highest rate in eyelid myoclonia with or without absences (EMA). Sleep deprived EEG on awakening was performed in 44 of 59 patients with ECS and enhanced ECS prominently. In all groups except EMA, PS rate was lower compared with ECS. Both ECS and PS were observed in 15 patients with poor prognosis (71 %) and one patient with good prognosis (5%). Conclusions ECS can occur in all types of the GGEs. We claim that ECS can be asserted as a syndrome-specific feature for EMA among GGEs. Sleep deprived EEG on awakening can enhance ECS prominently in all types of GGE. ECS and PS might overlap but their impact on prognosis is different. In the poor prognosis group, the number of patients having ECS together with PS was high compared to the group without ASD and excellent prognosis. Therefore, we suggest that this combination predicts worse outcome in GGEs.

Introduction Mutations in the C19orf12 gene cause mitochondrial membrane protein associated neurodegeneration (MPAN), an autosomal recessive form of neurodegeneration with brain iron accumulation (NBIA). A limited number of patients with C19orf12 mutations, particularly those with adult onset of symptoms, have been reported. Methods We sequenced the entire coding region of C19orf12 in 15 Turkish adult probands with idiopathic NBIA. We also performed haplotype analysis in families with a recurrent C19orf12 mutation. Clinical features were collected using a standardized form. Results Nine of our 15 probands (60%) carried the homozygous c.32C > T mutation in C19orf12 (predicted protein effect: p.Thr11Met). This homozygous mutation co-segregated with the disease in all affected relatives available for testing (16 homozygous subjects). Haplotypes across the C19orf12 locus were identical for a very small region, closest to the mutation, suggesting an old founder, or, two independent founders. The clinical phenotype was characterized by adult onset in most cases (mean 24.5 years, range 10–36), and broad spectrum, including prominent parkinsonism, pyramidal signs, psychiatric disturbances, cognitive decline, and motor axonal neuropathy, in various combinations. On T2- or susceptibility weighted-MRI images, all patients displayed bilateral hypointensities in globus pallidus and substantia nigra, without an eye-of-the-tiger sign; however, hyperintense streaking of the medial medullary lamina between the external and internal parts of globus pallidus was observed frequently. Conclusion The C19orf12 p.Thr11Met mutation is frequent among adult Turkish patients with MPAN. These findings contribute to the characterization of this important NBIA form, and have direct implications for genetic testing of patients of Turkish origin.

OBJECTIVE Vestibular migraine (VM) is a clinical condition characterized by temporal overlap between vestibular symptoms and migraine. In this study, we aimed to determine the changes in vestibular myogenic potential (cVEMP) and auditory brainstem response (ABR) in patients with VM and migraine. MATERIALS AND METHODS A total of 86 participants with no hearing loss or additional disease between the ages of 18 and 45 were enrolled in three different groups: group 1, VM; group 2, migraine without aura; and group 3, healthy controls. cVEMP and ABR were performed for all participants during attacks and attack-free periods. The differences between the right and left sides were calculated. RESULTS There was no significant difference in cVEMP p13-n23 latencies between any of the groups. There were statistically significant differences related to cVEMP p13-n23 amplitudes between groups 1, 2, and 3. This significant difference originated from group 1 when compared with the other groups (p

Myoclonus-dystonia syndrome (MDS) is a rare disease manifesting myoclonus as the only neurological symptom which may be accompanied by dystonia. It usually starts in the first or second decade of life. It has a benign course with spontaneous remissions but can cause functional disability in some patients. In this paper, we report a patient diagnosed as probable MDS on the basis of clinical and electrophysiological features who showed marked improvement under levetiracetam treatment.