Myoclonus and tremor in chronic inflammatory demyelinating polyneuropathy: a multichannel electromyography analysis.

BACKGROUND: Information regarding involuntary movements in chronic inflammatory polyneuropathy (CIDP) is gradually increasing. Our goal is to identify the types of involuntary movements in CIDP. METHODS: All patients who were followed with the diagnosis of CIDP were invited for clinical and electrophysiological evaluations. Demographic and clinical findings (age, gender, duration of illness, diagnosis, treatments) were noted. Clinical examination and multichannel surface electromyography were done. We also performed routine upper and lower extremity peripheral nerve conduction studies, F-waves, long latency reflexes, blink reflex, mixed nerve silent period and cutaneous silent period in all patients. RESULTS: Twenty-two patients accepted the invitation. Eleven patients with CIDP had involuntary movements. Ten (45.5%) patients with CIDP had tremor and seven (31.8%) had short-duration and high-amplitude myoclonus. Regarding demographic, clinical and electrophysiological features, there was no significant difference between patients with and without tremor. The latencies of R1, R2 and R2c components of BR were longer among CIDP patients without tremor compared to CIDP patients with tremor. Presence of myoclonus (p = 0.007) and delayed F-waves (p = 0.008) were associated with the presence of tremor. CONCLUSION: Tremor and myoclonus were frequent in CIDP. The fact that myoclonus was detected in the majority of patients only by multichannel surface EMG who were clinically evaluated as pure tremor suggests that a more detailed electrophysiological evaluation is required. There was no difference in the medications used or other clinical features between patients with and without tremor.

Cutaneous silent period in patients with spinal muscular atrophy type 2 and type 3.

OBJECTIVE: The aim of this study was to examine cutaneous silent periods (CSPs) in spinal muscular atrophy (SMA) type 2 and type 3 patients. METHODS: Fourteen patients with SMA and 14 healthy subjects were included. CSPs were recorded from thenar muscles after painful stimulation of the index finger during slight thumb abduction. CSP parameters (including onset latency, duration, index of suppression, long-loop reflex, and post-inhibitory rebound activity) were measured. All parameters were compared between SMA patients and healthy subjects using the Mann-Whitney U test. We then correlated CSP parameters to Hammersmith Functional Motor Scale Expanded (HFMSE) scores in SMA patients by Spearman-rho testing. RESULTS: No CSP parameter except E3% (i.e., the post-inhibitory rebound activity relative to pre-stimulus baseline) differed significantly between SMA patients and healthy subjects. E3% was significantly smaller in patients than healthy individuals. HFMSE scores of SMA patients correlated negatively with CSP duration and positively with E3%. DISCUSSION: Smaller E3%, correlating with longer CSP duration, in SMA patients with more severe clinical findings and therefore more marked motoneuron loss suggests that the E3 component following the CSP is directly affected by motoneuron loss.

Modulation of the Somatosensory Blink Reflex in the Peripersonal Space Is Defective in Episodic Migraine.

OBJECTIVE: In migraine, there is an altered behavior of patients during the attack and an altered connectivity in the cortical structures modulating and encoding the sensation and pain. Thus, we hypothesized that the extent of the peripersonal space (PPS) and the responses in the PPS may change during a migraine attack. For this reason, we analyzed the modulation of somatosensory blink reflex (SBR) in the PPS during episodic migraine. DESIGN: Cross-sectional assessment of modulation of SBR in patients with migraine. SETTING: Headache outpatient clinic of a tertiary referral center. SUBJECTS: We included 22 patients with episodic migraine, of whom 13 individuals were in the interictal period and nine were experiencing a headache episode. We also included 14 healthy individuals. The three groups were similar in age and gender. METHODS: SBR was recorded when the participants were sitting with their forearm in the extrapersonal space and also when their hands were in the PPS surrounding the face. Latency, amplitude, and area under the curve (AUC) were measured and compared. RESULTS: The amplitude and AUC of the SBR were significantly higher in patients during the attack compared with healthy subjects. The magnitude of the SBR was increased in the PPS in healthy subjects, whereas the increase was not significant in patients during the attack or in the interictal period. CONCLUSIONS: We think that the modulation in the PPS is defective in patients with migraine both during the acute attack and in the interictal phase, suggesting diminished top-down modulation of the SBR.

Ulnar Neuropathy at Elbow in Patients With Type 2 Diabetes Mellitus.

PURPOSE: In diabetes mellitus (DM), upper extremity entrapment neuropathies are suggested to be a component of polyneuropathy (PNP). Our aim is to examine the presence of ulnar neuropathy at the elbow (UNE) and its relation to other findings including PNP in symptomatic and asymptomatic type-2 DM patients who were admitted for routine examinations. METHODS: The study included all cases referred for electromyography because of type-2 DM between November 2017 and May 2018. Demographic and clinical characteristics were recorded. Routine electromyography examinations in all cases included the following: bilateral motor conduction of the median, ulnar, peroneal, and tibial nerves and sensory conduction of the median, ulnar, and sural nerves. For ulnar nerve examination, stimuli were given at the wrist, below the elbow, and above the elbow. Electrophysiological findings were evaluated according to the American Association of Neuromuscular and Electrodiagnostic Medicine criteria. RESULTS: Eighty-two patients with type-2 DM and 144 upper extremities were included in the study. Of the 82 patients who participated in the study, 3 had findings suggesting ulnar neuropathy, and electrophysiology confirmed UNE in only one. Electrophysiological studies showed UNE in 36 patients. Other diagnoses identified by electrophysiology were carpal tunnel syndrome and PNP. Ulnar neuropathy at the elbow was more commonly associated with PNP compared with carpal tunnel syndrome. Gender and PNP were independent risk factors for the development of UNE. CONCLUSIONS: Although the majority of diabetic patients were asymptomatic for the UNE, approximately one third of all patients with DM were found to have UNE. Ulnar neuropathy at the elbow is closely related with PNP.

Guillain-Barré Syndrome and Its Variants: Clinical Course and Prognostic Factors.

INTRODUCTION: We aimed to analyze the frequency, clinical characteristics, medical treatment options and final functional status of Guillain-Barré syndrome (GBS) and its variants in a population from a tertiary hospital setting. METHODS: All medical records of patients with acute inflammatory polyneuropathy between the years of 1998-2013 were retrospectively screened. Demographic, clinical and laboratory information, treatment options and the rate of recovery of the patients were gathered. RESULTS: A total of 183 patients met the study criteria. Subtypes were typical demyelinating form (n=102, 79.1%), acute motor sensory axonal variant (n=11, 8.5%), acute motor axonal variant (n=10, 7.8%), Miller-Fisher syndrome (n=5, 3.9%), and pure sensory subtype (n=1, 0.8%). Remaining patients had the diagnosis of acute-onset chronic inflammatory demyelinating polynuropathy. The data of treatment option were available for 70 patients. Most of the patients received intravenous immunoglobulin (IVIg) treatment or the combination of IVIg and methylprednisolone. One patient died, there was no improvement in eight patients and rest showed improvement with varying degrees. CONCLUSIONS: We did not observe major change of recovery between different treatment options, however, most of the patients using methylprednisolone required IVIg because of inadequate response.

Reorganization of sensorimotor gating after peripheral facial palsy starts at brainstem.

Background and objective We aimed to analyze the alterations in sensorimotor gating at brainstem after peripheral facial palsy (PFP). To examine sensorimotor gating, we used prepulse modulation (PPM) of blink reflex (BR). We also recorded BR recovery to identify excitability changes in the facial nucleus. Patients and method We included 33 patients and 39 recordings. Control group was composed of 16 healthy subjects. Simultaneous bilateral baseline BR, BR recovery at ISI of 300-ms and BR-PPM at ISI of 100-ms recordings were performed after stimulation of trigeminal nerve on right sides of healthy subjects and on both sides of patients. Severity of PFP and time lapse from the onset was noted. Results Mean R1 amplitude was increased, whereas mean R2 and R2c magnitude were reduced in all groups after prepulse stimulation. However, multivariate ANOVA showed significance at group level (patients and healthy subjects), at prepulse level (no prepulse and 100-ms prepulse) and group and prepulse level. Suppression of R2 or R2c was lower on both sides of patients compared to healthy subjects and the deficit first started on the symptomatic side. Conclusion Suppression of R2 and R2c after prepulse stimulation is reduced in PFP suggesting decreased filtering of facial sensory input at brainstem level. Trigeminal sensitization at brainstem develops early after PFP.

Deficient prepulse inhibition of blink reflex in migraine and its relation to allodynia.

OBJECTIVE: Prepulse inhibition (PPI) of the blink reflex (BR) is a reduction in BR excitability due to a conditioning stimulus, reflecting sensory gating by brainstem structures. We aimed to analyze PPI changes during a painful episode in chronic or episodic migraine and its relation to allodynia, since abnormal brainstem filtering has been hypothesized in migraine pathophysiology. METHODS: We included 20 patients with migraine during headache episode, and age- and gender-matched 22 healthy subjects. We recorded BR after unconditioned and conditioned supraorbital stimuli. For conditioned stimuli, we applied preceding subthreshold stimulus to the median nerve at wrist. The presence of PPI was compared between the two groups, as well as the specific BR parameters (latency, amplitude or area of R1 and R2 components) in unconditioned (test) and conditioned (PPI) paradigms. RESULTS: In the patient group, seven (35%) patients did not have R2-PPI whereas all healthy subjects had R2-PPI (P=0.003). Healthy subjects displayed significantly increased R1 amplitude and reduced R2 amplitude and area after conditioned stimuli. In migraine patients, we observed significant reduction only in R2 amplitude. Logistic regression demonstrated that allodynia was independently related with the presence of PPI (beta: -0.535, P=0.021). CONCLUSIONS: Our study provides evidence for sensory gating impairment at brainstem level in migraine headache, related to the presence of allodynia.

Familial Adult-onset Alexander Disease: Clinical and Neuroradiological Findings of Three Cases.

The adult-onset Alexander disease (AOAD) dramatically differs from the early onset AD with respect to clinical and neuroradiological findings. Herein we report the detailed clinical and neuroradiological findings of a Turkish family with AOAD. In all three cases, magnetic resonance imaging revealed marked atrophy of the mesencephalon, bulbus, and cervical spinal cord accompanied with signal abnormalities in the same regions along with supratentorial white matter. Basal ganglia were affected in two cases. Molecular genetic analysis revealed heterozygous mutation in the 8th exon of the glial fibrillary acidic protein gene M451I (c.1245G>A), leading to the diagnosis of AOAD in all cases.

A novel muscle for electroneurography in peripheral facial palsy: occipitalis.

Electroneurography (ENoG) is one of the most objective tests in grading the damage and prediction of prognosis in peripheral facial palsy (PFP). We aimed to determine temporal changes of ENoG recorded over occipitalis muscle in acute idiopathic PFP. Consecutive 21 patients with unilateral acute idiopathic PFP and age- and sex-matched 15 healthy volunteers were included in the study. Nasal and occipital ENoG values were recorded once in the control group and the same procedure was repeated daily between the second and eight days of the disorder in the PFP group. Occipital ENoG value began to increase on the third day while nasal ENoG value was still within the normal range (27.04 vs 7.69 %, p = 0.0001). In the fourth, fifth and sixth days, occipital ENoG value was significantly high compared to nasal ENoG value (p = 0.0001 for each day) whereas nasal and occipital ENoG values were very similar in the seventh and eighth days (p = 0.181 and p = 0.584, respectively). Our study presents further support for technical possibility of occipital ENoG which may reflect the degree of fiber degeneration earlier than the nasalis muscle in PFP.

Headache in carotid artery stenting and angiography.

INTRODUCTION: Carotid angioplasty headache and diagnostic criteria are based on scarce data and small series. Here, we aimed to determine presence, frequency, and characteristics of headache after carotid artery stenting and angiography headache and speculate on possible mechanisms of head and neck pain emerging during or after the carotid artery stenting procedure. PATIENTS AND METHOD: The total of 64 patients who were admitted to our Neuroradiology Division of Radiology Department for primary percutaneous transluminal carotid interna stenting were included in the study. They had symptomatic or asymptomatic carotid artery disease with stenosis more than 70%. All patients were questioned by a neurologist regarding the presence, side, location, quality, severity, duration, and timing of headache after both angiography and stenting procedures. RESULTS: Frequency of headache after carotid interna stenting was 39.1%, it commonly arose in a short period after the procedure and relieved in 10 minutes. This type of headache was mild, ipsilateral, frontotemporal in location, pressing in nature, and arose frequently within 10 minutes after the procedure, whereas angiography headache had a frequency of 21.9% and it was ipsilateral, mild, burning-like headache. Angiography headache also relieved within 10 minutes. Both types of headache were related to severe stenosis. DISCUSSION: Our study clearly demonstrates that headache is seen after carotid artery stenting (39.1%) and angiography (21.9%). Although both types of headache have similar characteristics, they differ in that it is mostly pressing in the group of carotid artery stenting and burning in angiography group.

Efficacy of nebivolol and amitriptyline in the prophylaxis of cyclic vomiting syndrome: a case report.

BACKGROUND: Cyclic vomiting syndrome (CVS) is an episodic disorder with recurrent attacks of nausea and vomiting. The exact cause of the disorder is still unclear. It was first described in children but may affect patients of any age. The syndrome is frequently misdiagnosed, and patients receive redundant investigations and treatments. Patients are referred finally to a neurologist because of the differential diagnosis of abdominal migraine or abdominal epilepsy. CASE REPORT: We present a 18-year-old girl with episodic nausea and vomiting attacks who was diagnosed as CVS. The attacks regressed with combination treatment with amitriptyline and nebivolol. CONCLUSION: CVS has no specific diagnostic test and the diagnosis is based on history, clinical presentation and exclusion of other possible causes with similar presentation. The syndrome has a strong association with migraine; treatment options may also overlap. Treatment is still based on case series and reports. Here, we aim to present the clinical features as well as treatment response of a patient with CVS.

An unusual case of vascular loop syndrome.

Coexistence of hemifacial spasm (HFS) and trigeminal neuralgia (TN) is a rare entity known as painful tic convulsif (PTC). Here, we present a case of right-sided HFS after which left TN developed, which is an unusual form of PTC. Both disorders were caused by bilateral vascular compression of the cranial nerves and successfully treated with botulinum toxin and carbamazepine. As PTC is benign in nature and can be treated with botulinum toxin, neuroradiological investigations should be performed for an accurate aetiological diagnosis, particularly in young patients with atypical disease manifestations.

EEG correlates of startle reflex with reactivity to eye opening in psychiatric disorders: preliminary results.

Previous studies have shown alterations of eyeblink reflex in patients with various psychiatric disorders. It has previously been demonstrated by our group that EEG measures of the reactivity to eye opening could effectively predict patient-reported startle response in patients with acute stress reaction. In our present study, EEG spectral power analysis and eyeblink electrical startle responses were acquired from a total of 39 patients diagnosed with various psychiatric disorders: 7 patients with schizophrenia, 10 patients with major depressive disorder (MDD), 10 patients with panic disorder, 5 patients with posttraumatic stress disorder (PTSD) and 7 patients with generalized anxiety disorder (GAD). EEG percent power data of each frequency band (delta, theta, alpha, beta) obtained from the 19 leads under open or closed eyelid conditions were used to calculate the arithmetical difference between eyes-open and eyes-closed states as representative of "EEG reactivity to eye opening". Data was analyzed separately for each diagnostic group. For all of the disorders, right-sided R2c (contralateral secondary component) latency was the single eyeblink startle measure that was found to be significantly correlated with EEG reactivity to eye opening. The correlation was most significant for right temporal theta frequency in schizophrenia, right temporal theta frequency in MDD, left central beta frequency in panic disorder, left parietotemporal delta frequency in PTSD and right occipital alpha frequency in GAD. Findings showed a newly identified pattern that has potential scientific and clinical value with respect to psychiatric medicine.

Seizure-related headache in patients with epilepsy.

We investigated the type and frequency of interictal primary headache and peri-ictal headache in 109 patients with partial-onset and 26 patients with generalized onset seizures in this study. Interictal headaches were present in 50 (40.7%) of 135 patients. Comparing the interictal headache on the basis of seizure type, we couldn't find any significant difference between the seizure groups. Seventy-nine (58.51%) patients had peri-ictal headache. Eleven of these patients had pre-ictal headache (PriH), three of all had ictal headache and, 56 of these had post-ictal headache (PoiH). PriH and PoiH were more frequently encountered before and after secondary generalized tonic-clonic seizures (GTCS) compared to other seizure groups. The type of pain in PoiH was 'throbbing' in complex partial seizures and 'steady' in GTCS.