Mecbure Nalbantoglu

A prospective study of Willis–Ekbom disease/restless legs syndrome during and after pregnancy

OBJECTIVES Willis-Ekbom disease/restless legs syndrome (WED/RLS) is the most common sleep-related movement disorder in pregnancy. We designed a prospective longitudinal study to investigate the correlates of WED/RLS during and after pregnancy. DESIGN A total of 138 pregnant women with WED/RLS and a control group of 251 age-matched pregnant women were enrolled prospectively. A questionnaire was administered during a face-to-face interview at first evaluation during pregnancy and three months after delivery. RESULTS Among all women in the first trimester, 15.6% were diagnosed with WED/RLS, whereas 32.8% of those in the second trimester and 38.8% of those in the third trimester were diagnosed with WED/RLS (p = 0.032). In regression analysis, later gestational age [p < 0.001; odds ratio (OR) 1.054] and previous history of WED/RLS (p = 0.001; OR 2.795) were positively correlated with the presence of WED/RLS, while ferritin levels (p = 0.001; OR 0.956) were negatively correlated with the presence of WED/RLS. Ferritin levels were also negatively correlated with the International RLS Study Group severity index (p = 0.041). Forty-eight patients (34.8%) experienced WED/RLS symptomatology after delivery. The ferritin levels were lower, and the mean number of pregnancies was higher, in women with residual WED/RLS (p = 0.008). CONCLUSION Our survey showed that WED/RLS was more common in the second and third trimesters. Emergence of WED/RLS during the second trimester was strongly associated with residual WED/RLS. Lower ferritin levels were associated with both WED/RLS in pregnancy and residual WED/RLS after delivery. A higher number of pregnancies were also associated with a greater likelihood of having residual WED/RLS after delivery.

Evaluation of the Tibial Nerve with Shear-Wave Elastography: A Potential Sonographic Method for the Diagnosis of Diabetic Peripheral Neuropathy

Purpose To evaluate the value of shear-wave elastography (SWE) in the detection of diabetic peripheral neuropathy (DPN) of the tibial nerve. Materials and Methods This study was approved by the institutional review board, and written informed consent was obtained from all study participants. The study included 20 diabetic patients with DPN (10 men, 10 women), 20 diabetic patients without DPN (eight men, 12 women), and 20 healthy control subjects (nine men, 11 women). The tibial nerve was examined at 4 cm proximal to the medial malleolus with gray-scale ultrasonography and SWE. The nerve cross-sectional area (in square centimeters) and the mean nerve stiffness (in kilopascals) within the range of the image were recorded. Inter- and intrareader variability, differences among groups, and correlation of clinical and electrophysiologic evaluation were assessed with intraclass correlation coefficients, the Mann Whitney U test, and the Wilcoxon signed rank test. Results Between diabetic patients with and diabetic patients without DPN, mean age (60 years [range, 38-79 years] vs 61 years [range, 46-75 years], respectively), mean duration of diabetes (10 years [range, 1-25 years] vs 10 years [range, 2-26 years]), and mean body mass index (31.4 kg/m2 [range, 24.7-48.1 kg/m2] vs 29.8 kg/m2 [range, 22.9-44.0 kg/m2]) were not significantly different. Diabetic patients without DPN had significantly higher stiffness values on the right side compared with control subjects (P < .001). Patients with DPN had much higher stiffness values on both sides compared with both diabetic patients without DPN (P < .001) and healthy control subjects (P < .001). A cutoff value of 51.0 kPa at 4 cm proximal to the medial malleolus revealed a sensitivity of 90% (95% confidence interval [CI]: 75.4%, 96.7%) and a specificity of 85.0% (95% CI: 74.9%, 91.7%). Conclusion Tibial nerve stiffness measurements appear to be highly specific in the diagnosis of established DPN. The increased stiffness in subjects without DPN might indicate that the nerve is affected by diabetes.

Percheron artery infarction in the differential diagnosis of acute confusional state with normal initial brain MRI.

The artery of Percheron (AOP) is a solitary trunk representing an uncommon anatomic variant that provides bilateral arterial supply to the paramedian thalami and the rostral midbrain. Occlusion of this artery results in bilateral thalamic and mesencephalic infarctions [1]. AOP prevalence remains unknown, bilateral thalamic infarcts are uncommon. In a recent study, AOP infarction was found in 0.4 % patients with a first-ever stroke in their stroke registry [2]. The clinical spectrum is very variable which makes the clinician consider about inflammatory, infectious, toxic cause of cerebral dysfunction in the differential diagnosis. We report a case without evident cause for acute disturbance of consciousness and normal early imaging of head computed tomography (CT) and brain magnetic resonance imaging (MRI). A 73-year-old women was brought to the emergency department with acute onset of decreased mental status and rapidly progressing disturbance of consciousness after transient bilateral blurred eye vision. There were no focal or lateralising neurological deficits with Glascow Coma Scale of 5/15. Her past medical history was unremarkable except diabetes mellitus. She was on insulin therapy. All blood tests including full blood count, liver and renal function tests, blood glucose, electrolytes, thyroid function tests and antibodies, viral serology, and arterial blood gas were in normal limits. Electrocardiogram showed a normal sinus rhythm. Initial head CT and brain MRI were unremarkable. Electroencephalography demonstrated diffuse mild background slowing without epileptiform activity. A bilateral thalamic and rostral mesencephalic infarction due to occlusion of the AOP was revealed after 12 h by a new Diffusion Weighted-MRI (DW-MRI) (Fig. 1) and head CT (Fig. 2) which were obtained simultaneously. Anticoagulant therapy was administered and consciousness gradually improved within 3 days. Echocardiogram was normal except mild left ventricular hypertrophy. MR Angiography was reported normal. These lesions disappeared in the DW-MRI which was revealed after 13 days from the clinical improvement and so from 16 days after onset. Our case indicates that a normal initial brain MRI cannot formally eliminate the diagnosis of acute stroke of the AOP. The clinician should consider about AOP occlusion in the differential diagnosis of acute confusional state even if the initial brain imaging is normal. In this case, new brain imaging by MRI or CT scan should be performed within the first 48 h [3]. Another point of interest is that the artery is rarely visualized on MR angiography and lack of visualization does not exclude its presence [4], as we could also have not demonstrated in our case.

Lambert-Eaton myasthenic syndrome associated with thymic neuroendocrine carcinoma.

decremental response, and the initial CMAP of the RNS train had improved (Fig. 1C). On discharge at day 44, her MG-ADL score was 2. Although respiratory infection is the most common cause of MG exacerbation, intravenous peramivir may have induced the respiratory failure in the patient described, because it developed within 20 minutes after drug administration and the symptoms recovered rapidly, with improvement in the RNS test. Her improvement may be a result of the discontinuation of peramivir, regardless of immunotherapy. Furthermore, the safety factor for neuromuscular transmission, which decreased subclinically, may have improved because of immunotherapy. MG exacerbation has been reported with various medications. A few neuraminidase inhibitors suppress respiratory function through mechanisms including ion channel/receptor inhibition. Therefore, we speculate that peramivir may inhibit ion channels/receptors in the neuromuscular junctions, leading to respiratory failure and MC. Further studies are necessary to understand the mechanism underlying MC induction by intravenous peramivir. Koji Hayashi, MD

What Does One Sleep-Onset REM Period—During Either Nocturnal Polysomnography or Multiple Sleep Latency Test—Mean in Differential Diagnosis of Central Hypersomnias?

Purpose: The differentiation of narcolepsy without cataplexy from idiopathic hypersomnia is based on the number of sleep-onset rapid eye movement periods (SOREMPs) observed by multiple sleep latency test (MSLT) and nocturnal polysomnography. The main aim of this study was to investigate the utility of SOREMP in differential diagnosis of central hypersomnias. Methods: The authors retrospectively evaluated consecutive 101 patients with a normal polysomnography other than the presence of SOREMP and/or REM without atonia and a latency of ⩽8 minutes in MSLT. Results: The authors classified patients as follows: 52 patients had at least 2 SOREMPs (narcolepsy group), 23 had no SOREMPs (idiopathic hypersomnia group), and 26 patients had only 1 SOREMP (intermediate group). In polysomnographic recordings, both mean sleep latency and REM latency were significantly shorter in the narcolepsy (P = 0.012, P < 0.001, respectively) and intermediate groups (P = 0.005 and P = 0.035, respectively) compared with the idiopathic hypersomnia group. In MSLT recordings, sleep latency was 2.7 ± 2.2 minutes in the narcolepsy group, 3.6 ± 1.4 minutes in the intermediate group, and 5.2 ± 2.7 minutes in the idiopathic hypersomnia group (P < 0.001). The mean REM latency and sleep stages SOREMPs arised from were similar between the narcolepsy and intermediate groups. Conclusions: To date, SOREMPs in MSLT and polysomnography remain the sole electrodiagnostic feature that discriminates narcolepsy without cataplexy from idiopathic hypersomnia. Different parameters or combined criteria are being increasingly investigated to increase the sensitivity and specificity of MSLT. The findings showed an altered instability of REM sleep not only in patients with 2 or more SOREMPs in MSLT but also in patients with one SOREMP.

Stiff Person Syndrome with Pyramidal Signs

Stiff person syndrome (SPS) is a rare disorder characterized by continuous muscle activity causing severe rigidity and episodic spasms in axial and limb muscles (1). According to the diagnostic criteria, normal motor and sensory examination is the rule. Hyperactive deep tendon reflexes may be observed, but extensor plantar reflexes are rare (1,2,3,4). Here, by presenting this case with atypical features like pyramidal signs, we aimed to review the clinical and electrophysiological signs of this rare syndrome.

Natural course of neuromyelitis optica (NMO) in patients with no long-term treatment

OBJECTIVE:To show the natural course of disease in NMO patients with not receiving long term treatment(LTT). BACKGROUND:Neuromyelitis optica(NMO) is an inflammatory disorder of the central nervous system(CNS) presenting with severe optic neuritis(ON) and transverse myelitis(TM).According to current guideline, early diagnosis and aggressive treatment is critical for preventing disability. Some NMO cases in our series didn’t accepted to receive LTT and making comparison between treated and non-treated NMO patients became possible. DESIGN/METHODS:Sixty one patients followed with a diagnosis of NMO/NMO Spectrum Disorder were included in this study. The patients were divided into four subgroups:Group 1:Classical NMO, Group 2:Single or recurrent attacks of transverse myelitis with longitudinally extending spinal cord lesions(LETM), Group 3:Recurrent optic neuritis(ON) with normal brain MRI or cranial MRI lesions not compatible with multiple sclerosis(MS),Group 4:Optico-spinal MS(OSMS).The age at disease onset,gender,type of attacks,clinical presentation,number of attacks,analysis of the cerebrospinal fluid,serum anti-AQP4 antibodies,Expanded Disability Status Scale(EDSS), annual progression index were assessed. RESULTS:Twenty two of the patients were on LTT(19 male,3 male) and 39 patients were not receiving any LTT (32 female,7 male).The mean age at disease onset was 31.58+11.79(between 12-65) in non-treated and was 30.45+12.36(between13-62) in treated group.The mean disease duration was 68.07+64.02 months in non-treated and 58.76+29.59 in treated group.Mean annual progression index (PI) was found to be higher in the non-treated group with respect to treated group (1.27+1.40 and 0.52+0.94 respectively). CONCLUSION:NMO and NMOSD are both considered to be disabling diseases of the central nervous system.Acute clinical relapses are treated with intravenous IV corticosteroids and plasma exchange.However there are still controversies regarding with long term treatment due to lack of randomized double blind studies.Our results verified that immunosuppressive therapy should be started immediately when the diagnosis of NMO is revealed. Disclosure: Dr. Altintas has received research support from The Scientific and Technological Research Council Of Turkey. Dr. Nalbantoglu has nothing to disclose. Dr. Uygunoglu has nothing to disclose. Dr. Gozubatik-Celik has nothing to disclose. Dr. Saip has nothing to disclose. Dr. Siva has nothing to disclose.

The diagnosis of band heterotopia.

Patient 1: A 6-year-old girl was admitted with a 1 year history of atonic seizures (head drops). Her motor and mental development had been normal until age 5 years, but her family had noted behavioral changes about 2 months after the onset of seizures. Her past medical history was remarkable for febrile seizures. Physical and neurological examinations were unremarkable. Electroencephalography (EEG) demonstrated multifocal spike and wave paroxysms generalizing during sleep and bilateral widespread pseudo-periodic slowwave paroxysms with high amplitude (Fig 1). Atonic-myoclonic seizures were recorded. Brain magnetic resonance imaging (MRI) revealed no abnormalities but was performed under suboptimal conditions. Subacute sclerosing panencephalitis was first considered, but no cells or antimeasles antibodies were detected in cerebrospinal fluid. Further testing for metabolic disorders was unremarkable. Her repeat MRI revealed diffuse band heterotopia (Fig 2). Patient 2: A 7-year-old girl was admitted with atonicmyoclonic seizures manifested by dropping of the head, falling, jerking, and dropping objects. Motor and mental development had been normal until the seizure onset at the age of 4 years, but subsequently her family noted behavioral changes. Her EEG findings included spike and wave paroxsyms propagating from both occipital regions, bilateral pseudo-periodic slowwave and sharp wave discharges, and atonic seizures. Subtle MRI changes suggested band heterotopia, and bilateral band heterotopia was confirmed on her second MRI.