Winged Scapula: Clinical and Electrophysiological Features and Common Causes Based on 20 Years of Experience in a Referral Center in Turkey.

PURPOSE: Winged scapula (WS) is a functionally disabling problem and it occurs because of neurogenic causes frequently. The authors aimed to assess WS patients by physical and electrodiagnostic examinations as well as some further investigations and define the common causes of WS. METHODS: The authors reviewed clinical and neurophysiological findings of 52 patients who were referred for electrodiagnostic examination because of WS in the period of 20 years. RESULTS: The mean age was 39 (range, 11-73) years and 32 were male patients. Right side was involved in 60% of patients (n = 31). According to electrodiagnostic examinations, 44 patients (85%) had neurogenic causes; 29 spinal accessory nerve palsy (17 occurred after surgical procedure), nine long thoracic nerve palsy (four occurred after strenuous activity), two dorsal scapular nerve (both neuralgic amyotrophy), one long thoracic nerve and spinal accessory nerve (relevant with strenuous trauma), one spinal accessory nerve and dorsal scapular nerve palsies (after surgical procedure and radiotherapy), one C5-7 radiculopathy (avulsion), and one brachial plexopathy (obstetric trauma). Five patients (10%) had muscle-related findings (four facio-scapulo-humeral dystrophy and one Duchenne muscular dystrophia) and three patients (5%) had normal findings (bone-joint related). CONCLUSIONS: This study presents a relatively large series of patients with WS because of several causes from a referral tertiary EMG laboratory. The authors found that spinal accessory nerve palsy after neck surgery is the most common cause and long thoracic nerve palsy is the second common cause of unilateral WS. Electrodiagnostic examinations should be performed in WS patients to establish exact diagnosis and reveal some coexistence of WS causes.

The REM-sleep-related characteristics of narcolepsy: a nation-wide multicenter study in Turkey, the REMCON study.

INTRODUCTION: Narcolepsy type 1 (NT1) is caused by hypocretin deficiency, the pathophysiology of narcolepsy type 2 (NT2) has not been delineated. Except for the hypocretin deficiency and cataplexy, all clinical and laboratory features used in the diagnosis of NT2 are identical to those used for NT1. The aim of this study was to assess the rapid eye movement (REM) sleep-related characteristics in the patients with narcolepsy; the characteristics of REM sleep in polysomnography (PSG) and multiple sleep latency test (MSLT) recordings, the quantification of REM sleep without atonia (RSWA) and atonia index, and the analysis of rapid eye movements (REMs) during REM sleep. MATERIALS AND METHODS: This study was planned by the Sleep Medicine Study Group of the Turkish Neurology Society, and conducted in 11 centers in eight cities in Turkey. The analysis of RSWA was analyzed by reviewing all REM sleep periods on nocturnal PSG and MSLT recordings per standard criteria. The total duration of the increased muscle tone during REM sleep in the chin and bilateral leg electromyography (EMG) recordings was calculated as RSWA index. The REMs index was also investigated the relation to the RSWA. RESULTS: A total of 274 patients were involved; 147 patients (53.6%) were males and 127 patients (46.4%) were females; the mean age was 29.1 ± 12.0 years. The diagnosis of NT1 was made in 166 patients (60.6%), and 108 patients (39.4%) were diagnosed as having NT2. The mean Epworth sleepiness scale score was significantly higher in patients with NT1 than the patients with NT2 (P = 0.001). The diagnosis of REM sleep behavior disorder (RBD) was made in 19.3% of the patients with NT1 versus in 2.8% of the patients with NT2 (P < 0.001). The percentage of SOREMP in PSG recordings was significantly higher in patients with NT1 (37.1%) than those with NT2 (18.9%, P = 0.001). MSLT showed that the mean sleep latency was shorter in patients with NT1 compared to those with NT2 (P < 0.001). The total duration of REMs on electrooculography recordings was also significantly higher in patients with RSWA in comparison with the patients without RSWA (P = 0.002). Total duration of REMs was significantly and positively correlated with the duration of RSWA on chin-EMG and leg-EMG recordings (P = 0.001). ROC analyses showed an RSWA index of ≥2% for the RSWA on chin-EMG with a sensitivity of 86.7% and a specificity of 71.3% (P < 0.001). The REMs index ≥20% was associated with the presence of RSWA with a sensitivity of 70.0% and a specificity of 57.1% (P = 0.008). CONCLUSIONS: In this nation-wide study, we identified for the first time that the increase in REMs density during REM sleep may be a major correlate of the RSWA. Significant positive correlations were demonstrated between the total duration of REMs on electrooculography recordings and the mean durations of RSWA in both chin and leg EMG recordings. A REMs index of >20% was demonstrated to have a moderate sensitivity and specificity in the diagnosis of RSWA. As observed in chin RSWA index, REMs index also showed a significantly high association with RBD, in comparison to RSWA per standard criteria.

Nocturnal Rapid Eye Movement Sleep Without Atonia can Be a Diagnostic Parameter in Differentiating Narcolepsy Type 1 From Type 2.

PURPOSE: We aimed to compare rapid eye movement sleep without atonia (RSWA), tonic RSWA, and phasic RSWA indices during polysomnography as a potential biomarker between narcolepsy type 1 and type 2. METHODS: Medical files, polysomnography, and multiple sleep latency tests of patients with narcolepsy were evaluated retrospectively. A total of three adolescents and 31 adult patients were included. We calculated the total number of rapid eye movement (REM) epochs with tonic and phasic activity in accordance with the American Academy of Sleep Medicine manual scoring rules, version 2.4. We defined tonic RSWA index as the ratio of total number of REM sleep stage epochs with only tonic activity to total REM sleep stage epochs, phasic RSWA index as the ratio of total number of REM sleep stage epochs with only phasic activity to total REM sleep stage epochs, and RSWA index as the ratio of total number of REM stage sleep epochs with RSWA to total REM sleep stage epochs on the polysomnography. RESULTS: Clinically and polysomnographically diagnosed 25 patients with narcolepsy type 1 and 9 patients with narcolepsy type 2 were included. The median age of the subjects was 30 (10, 61) and 36 (18, 64), respectively. Eleven narcolepsy type 1 patients (44%) and 4 narcolepsy type 2 patients (44.44%) were women. The RSWA index of ≥ 3% yielded a sensitivity of 76% and specificity of 88.9% (AUC = 0.77 (0.09), 95% confidence interval = 0.58 to 0.97, p = 0.01), and the tonic RSWA index of ≥ 2.2% yielded a sensitivity of 72% and specificity of 77.8% (area under the curve = 0.74 (0.1), 95% confidence interval = 0.54-0.94, p = 0.03). CONCLUSIONS: As an electrophysiological biomarker, RSWA and tonic RSWA indices can be sensitive and specific polysomnography parameters in distinguishing narcolepsy type 1 from narcolepsy type 2.

Aphasic status epilepticus in a tertiary referral center in Turkey: Clinical features, etiology, and outcome.

OBJECTIVE: Aphasic status epilepticus (ASE), although rare, may often remain underdiagnosed. We aimed to report the patients diagnosed with ASE and describe their clinical presentation, etiology, electrophysiological findings, neuroimaging, treatment options, and outcome. METHODS: We retrospectively analyzed a series of 28 patients diagnosed with ASE between January 2010 and August 2019 in our tertiary referral center. We reviewed medical files, patient charts, and short- and long-term intermittent electroencephalogram (EEG) recordings. Demographical, historical, clinical, neuroimaging, electrophysiological parameters, administered antiseizure medications, and prognosis of all patients were recorded. Furthermore, EEGs were re-evaluated according to Salzburg criteria. RESULTS: Most patients presented with tumors (n = 11) and cerebrovascular disease (CVD) (n = 11), while the rest were diagnosed with hyperglycemia (n = 2), autoimmune encephalitis (n = 1), remote intracranial abscess (n = 1), mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) attack (n = 1), or unknown cause (n = 1). Seventy-one percent of patients had prior history of epilepsy. The most common EEG findings were rhythmic delta activity with fluctuation and epileptiform discharges ≤ 2.5 Hz with spatiotemporal evolution (66 %). Magnetic resonance imaging (MRI) and EEG findings indicated dominant hemisphere involvement in all patients. No casualties occurred; 71 % patients exhibited total recovery, while 29 % had mild-moderate sequela aphasia. None of the patients received aggressive treatment for ASE. SIGNIFICANCE: A relatively higher number of ASE patients could be contributed to the literature through this study. ASE should be considered in patients with prolonged unexplained aphasia, especially with pre-existing risk factors, such as prior history of epilepsy, structural lesion, and metabolic disorders accompanied by concordant EEG findings. Although the underlying cause is a determining prognostic factor, this study shows a significant number of patients with complete remission; thus, the prognosis of ASE may be considered more favorable than other types of nonconvulsive status epilepticus.

The coexistence of psychogenic nonepileptic and epileptic seizures in the same patient is more frequent than expected: Is there any clinical feature for defining these patients?

OBJECTIVES: This study aimed to investigate the prevalence of psychogenic nonepileptic seizures (PNES) and PNES-epilepsy coexistence within all video-electroencephalography (EEG) monitoring unit (VEMU) referrals and to identify semiological and electrophysiological features to differentiate patients with PNES-epilepsy coexistence from PNES-only. METHODS: We retrospectively reviewed medical files, VEMU reports, and videos of 1983 adult patients. Demographical, historical, clinical, neuroimaging, and electrophysiological parameters of all patients were recorded. We classified patients into five groups as definite PNES-only, definite PNES-epilepsy coexistence, definite PNES-probable epilepsy coexistence, probable PNES-definite epilepsy coexistence, and probable PNES-only. We defined a "definite" group when we saw the ictal EEG and/or video recording of the seizure. The "probable" term is used when there is strong evidence from the history of a particular seizure type and suggestive interictal EEGs without video recordings. RESULTS: Two hundred and three of 1983 patients (10.23%) had PNES. Sixty-six of patients with PNES (32.51%) had definite PNES-epilepsy coexistence. When probable cases were included, the PNES-epilepsy coexistence ratio was 53.69% within all patients with PNES. The prevalence of PNES-epilepsy coexistence was 3.32% within all our VEMU referrals. Lower high school graduation rate, earlier age of disease onset, history of status epilepticus, febrile convulsion and brain surgery, use of three or more antiepileptic drugs, and abnormal magnetic resonance imaging (MRI) findings supported PNES-epilepsy coexistence (p < 0.05). On the contrary, seizure duration longer than 10 min was in favor of PNES-only (p < 0.05). CONCLUSIONS: The prevalence of PNES-epilepsy coexistence might be more frequent in VEMUs than expected. Some demographic and semiological features and electrophysiological findings might be useful in differentiating patients with PNES-epilepsy coexistence from patients with PNES-only.

[A rarely known headache: Airplane travel headache]. / Az taninan bir basagrisi: Uçak yolculugu basagrisi.

Recently, headache associated with airplane travel has gained importance with case reports and took its place in the classification of headache in 2013. This rare condition has different spesific characteristic from the primary headaches and its pathophysiology is not clear yet. In this case report, a 27-years-old female patient was diagnosed with the headache associated with airplane travel by history, examination and imaging findings. The possible pathophysiology and treatment were discussed.

MRI findings and cognitive functions in a small cohort of myotonic dystrophy type 1: Retrospective analyses.

Myotonic dystrophy type 1 (DM1) is a progressive multisystemic disease with common cognitive deficits and potential brain involvement in addition to the cardinal muscular and systemic symptoms. Impaired mental function associated with nonspecific pathological findings such as white-matter hyperintense lesions (WMHLs), ventricular enlargement and brain atrophy on brain MRI have been previously reported in DM1 patients. While some studies showed correlation of brain morphological changes with neuropsychological and clinical parameters including CTG repeat sizes and disease severity scales in DM1, others failed. The goal of this study was to retrospectively investigate cranial MR abnormalities, predominantly WMHLs, and their effects on clinical and cognitive deficits in a small, phenotypically or genotypically well-characterized cohort of DM1 patients.

Baloon angioplasty for venous sinus stenosis in a idiopathic intracranial hypertension case.

The Idiopathic Intracranial Hypertension (IIH) is a well characterised condition with intractable headaches, visual obscurations, and papilloedema as dominant features, mainly affecting obese women. With the advent of magnetic resonance (MR) venography and increased use of cerebral angiography, there has been recent emphasis on the significant number of patients with IIH found to have associated non-thrombotic dural venous sinus stenosis. This has led to a renewed interest in endovascular stenting and angioplasty as a treatment for IIH in patients non-responsive to medical treatment. We present a patient without known risk factors for IIH and non-responsive to treatment. The 19-year-old woman presented with headache and diplopia. She was diagnosed with IIH since she was five years of age and had been non-responsive to lumbar cerebrospinal fluid (CSF) drainage and acetazolamide treatment. MR venography revealed thin calibration of transverse sinus. Digital subtraction angiography (DSA) venous phase also revealed 50% stenosis of transverse sinus, 50% stenosis of left proximal sigmoid sinus and 90% stenosis of its distal part leading to obstruction of left transverse sinus outflow and forced directed drainage of left hemisphere to the anterior region.