Fahr's syndrome presenting with epileptic seizure: Two case reports

Fahr's syndrome is a neuropsychiatric syndrome characterized by symmetrical and bilateral intracerebral calcifications located in the basal ganglia and usually associated with a phosphorus and calcium metabolism disorder. Clinical manifestations of Fahr's syndrome vary; it may start at different ages and have a variety of presentations. This article discusses rare presentation of Fahr's syndrome with epileptic seizure. These cases are important because they appear to be the first cases in the literature of Fahr's syndrome presenting with generalized tonic clonic seizure

Akut allerjik rinitli hastalarda lokal sempatik sistem disfonksiyonunu gösteren lokal sempatik deri yanıtları testi’nin elektrofizyolojik çalışması

Introduction: In this study, we aimed to investigate sympathetic nervous system functions by local sympathetic skin responses of the nasal septum in patients with acute allergic rhinitis.Material and Methods: Eighty-five patients who were diagnosed as acute allergic rhinitis according to medical history and otorhinolaryngological examination with positive allergy evaluations via skin prick testing and 50 healthy subjects were included to the study. Sympathetic skin responses of the nasal septum were recorded in patients and in the control groups, and sympathetic skin response latencies and amplitudes were compared between groups.Results: The mean value of sympathetic skin response latencies was significantly longer in the patient group than that of the control group (p<0.001). In addition, mean value of sympathetic skin response amplitudes was significantly lower in the patient group than the control group (p<0.001).Conclusion: Our study is the first which electrophysiologically evaluated the local sympathetic nervous functions that shows objective evidence of local sympathetic nervous system dysfunction. This way to access local sympathetic nervous system dysfunction would be helpful in deciding patients' treatment

Conduction in ulnar nerve bundles that innervate the proximal and distal muscles: a clinical trial

<p>Abstract</p> <p>Background</p> <p>This study aims to investigate and compare the conduction parameters of nerve bundles in the ulnar nerve that innervates the forearm muscles and hand muscles; routine electromyography study merely evaluates the nerve segment of distal (hand) muscles.</p> <p>Methods</p> <p>An electrophysiological evaluation, consisting of velocities, amplitudes, and durations of ulnar nerve bundles to 2 forearm muscles and the hypothenar muscles was performed on the same humeral segment.</p> <p>Results</p> <p>The velocities and durations of the compound muscle action potential (CMAP) of the ulnar nerve bundle to the proximal muscles were greater than to distal muscles, but the amplitudes were smaller.</p> <p>Conclusions</p> <p>Bundles in the ulnar nerve of proximal muscles have larger neuronal bodies and thicker nerve fibers than those in the same nerve in distal muscles, and their conduction velocities are higher. The CMAPs of proximal muscles also have smaller amplitudes and greater durations. These findings can be attributed to the desynchronization that is caused by a wider range of distribution in nerve fiber diameters.</p> <p>Conduction parameters of nerve fibers with different diameters in the same peripheral nerve can be estimated.</p

Clinical and electronystagmographic evaluation of vestibular symptoms ın relapsing remitting multiple sclerosis patients

Relapsing Remitting Multipl Skleroz Hastalarında Vestibüler Semptomlar Ve Elektronistagmografi Bulguları Dr. Eylem Değirmenci MS'de ENG ile vestibüler sistem tutulumunu incelen çalışmalar yetmişli yılların sonrasından bu yana yapıla gelmekle birlikte, yeterli sayıda hasta ile sakkad test, trekking test, optokinetik test, Dix-Hallpike testi, pozisyonel test, gaze test ve kalorik testi kapsayan ayrıntılı çalışmalar oldukça az sayıdadır. Bu çalışmada relapsing-remitting multiple skleroz hastalarında ayrıntılı ENG testlerini kullanarak vestibüler tutuluma ait klinik ve elektronistagmografik özellikleri incelenmesi ve klinik ve subklinik vestibüler tutulumda ENG'nin tanısal değerinin araştırılması amaçlanmıştır. Aynı zamanda klinik özellikler ile ENG arasındaki ilişki de incelenmiştir. Bilgimiz dahilinde, çalışmamız hastalığın erken döneminde olan RRMS hastalarından oluşan bir popülasyonda ENG'nin tüm alt testlerinin değerlendirilip, elde edilen bulguların klinik parametreler ile korelasyonlarının araştırıldığı ilk prospektif çalışmadır. Çalışmaya Mc-Donald tanı kriterlerine göre kesin RRMS tanısı alan 30 hasta ve 30 sağlıklı kişi kabul edilmiştir. Hastalar Eylül 2003 ve Mart 2005 tarihleri arasında Pamukkale Üniversitesi Tıp Fakültesi, Nöroloji Bölümü'nde değerlendirilmiştir. Sakkadik test, trekking test, optokinetik test, Dix-Hallpike, pozisyonel test ve gaze testi içeren detaylı ENG çalışmaları hem hasta hem de kontrol grubunda yapılmıştır ancak kalorik test rahatsızlık verici bir tetkik olduğu için sadece hasta grubunda yapılmıştır. RRMS hastalarında en sık bozukluk saptanan test trekking testti (% 57,1) ve bunu sakkadik test (% 36,7) izliyordu. Hastaların % 33,3 `ünde kalorik test bozukluğu, % 17,9'unda optokinetik test bozukluğu, % 16,7'sinde pozisyonel test bozukluğu, %10,0'unda gaze test bozukluğu ve % 7,7'sinde Dix Hallpike testinde bozukluk saptanmıştır. Hasta grubunda testlerde (kalorik test hariç) herhangi bir patolojinin varlığını gösteren ENG bulgusu % 90 (n=27), kontrol grubunda ise % 6,7 (n=2)oranında saptanmıştır ve her iki grup arasında istatistiksel olarak anlamlı fark saptanmıştır (p< 0,001). Santral patolojinin varlığını gösteren ENG patolojisi hasta grubunda % 83,3 (n=25), kontrol grubunda % 6,7 (n=2) oranında saptanmıştır ve her iki grup arasında istatistiksel olarak anlamlı fark saptanmıştır (p<0,001). ENG'de bozuk olan toplam test adedi testlerin yapıldığı dönemde herhangi bir vestibüler semptomu olanlarda (p<0,001), nörolojik muayenesinde vestibüler sistem tutuluş bulgusu, nistagmus ve koordinasyon bozukluğu olanlarda (sırasıyla p<0,001, p=0,006 ve p<0,001), vestibüler semptomuna eşlik eden otolojik, otonomik ve nörolojik semptomu olanlarda (sırasıyla p<0,001, p=0,004 ve p<0,001) olmayanlara göre istatistiksel olarak farklıydı. ENG'de bozuk olan test adedi ile yaş, ilk atak yaşı, hastalık süresi ve toplam atak sayısı arasında anlamlı korelasyon saptanmazken (sırası ile p=0,624, p=0,268, p=0,845 ve p=0,137), ENG'de bozuk olan test adedi ile EDSS puanı arasında pozitif yönde, istatistiksel olarak önemli bir ilişkinin olduğu saptanmıştır (p= 0,039). Bu korelasyonlar aynı zamanda tüm diğer alt testler için de araştırılmış ancak anlamlı korelasyon saptanmamıştır. Ayrıca her iki grup arasında sakkad test (p< 0,001), trekking test (p< 0,001) ve pozisyonel test (p=0,026) sonuçları arasında istatistiksel olarak anlamlı fark saptanmıştır. Tüm istatistiksel kıyaslamalar testlerin yapıldığı dönemde immün- modülatör kullanmayan 22 RRMS hastasına ait verilerle tekrar edilmiştir ve farklı her hangi bir sonuç saptanmamıştır. Çalışmamız, literatüre MS'de ilk atakta (% 70) veya hastalığın seyri sırasında (% 80) ortaya çıkan vestibüler semptomunların daha fazla sıklıkla izlenmesi, hastalığın seyri sırasında herhangi bir vestibüler semptomu olmayan olguların oranının sadece % 13,3 olduğu gibi yeni bilgiler katmaktadır. Aynı zamanda ataklarda gerçek baş dönmesi deneyimi yaşayan olguların bu yakınmalarının ilk atakta ortaya çıktığını gözlenmedik. lk atak veya herhangi bir atakta vertigo tanımlayan olgularda sakkadik test patolojinin anlamlı olarak daha fazla saptanabileceği ve EDSS ile ENG'de bozuk olan test adedi arasında anlamlı bir ilişki olduğu da MS için yeni bilgiler arasındadır. Sonuç olarak çalışmamız, ENG'nin tüm alt testleriyle detaylı olarak değerlendirilirse asemptomatik multipl skleroz olgularında bile yüksek duyarlılığa sahip olduğunu ortaya koymuştur. MS hastalarında vestibüler sistem ile ilişkili bilgi edinilmek istendiğinde, ya da farklı bir alt sistemin tutulumu ortaya koyarak destekleyici paraklinik ek bir kanıt isteniyorsa, ENG kuvvetle önerilir.Clınıcal And Electronystagmographıc Evaluatıon of Vestıbular Symptoms In Relapsıng Remıttıng Multıple Sclerosıs Patıents Dr Eylem Degirmenci Studies of electronystagmographic investigation in MS patients has been done since seventies, but detailed ENG test including saccade test, tracking test, optokinetic test, Dix-Hallpike test, gaze test, positional test and caloric test had not been done among adequate number of MS patient. In this study we aimed to evaluate clinical and electronystagmographical characteristics of vestibular system involvement in RRMS patients by performing detailed ENG test, and to investigate the diagnostic value of ENG in clinical and subclinical vestibular involvement. We also investigated the association of ENG and clinical characteristics. To the best of our knowlegde, this study is the first prospective study that contains all of the sub-tests in ENG, and correlates the datas with clinical parameters in a population of RRMS patients who are in the early states of the disease. Thirty patients who were diagnosed as definite relapsing remitting multiple sclerosis due to Mc-Donald?s diagnostic criteria and 30 healthy individuals included in this study. The entire population of patients was examined at the Pamukkale University Hospital, Neurology Department during the period from September 2003 and March 2005. ENG test including saccade test, tracking test, optokinetic test, Dix-Hallpike test, gaze test, and positional test were performed in all patient and control group, but caloric test which is an uncomfortable test was only performed in patient group. The most common abnormal test in RRMS patients in this study was tracking test (57.1 %), and saccade test (36.7 %) follows it. Caloric test abnormalities was found in 33.3 %, optokinetic test abnormalities was found 17.9 %, positional test abnormalities was found in 16.7 % patients, gaze test abnormalities was found in 10.0 %, and abnormalities of Dix-Hallpike test was found 7,7 % of the patients. Any abnormality in ENG subtests (except caloric test) was found in 90.0 % (n=27) of the patients and in 6.7 % (n=2) of the individuals in the control group. Difference of existence of any ENG abnormality between two groups was statistically significant (p< 0,001). Abnormalities showing central pathology in ENG subtests was found in 83.3 % (n=25) of the patients and in 6,7 % (n=2) of the controls. Statistically significant difference was found between central pathology in ENG subtests between groups (p<0,001). Total number of abnormal tests in ENG were statistically different between patients who have any vestibular symptom during the test (p<0,001), vestibular system involvement?s findings (p<0,001), nystagmus (p=0,006), coordination difficulty (p<0,001) in neurological examination, concomitant otological symptoms (p<0,001), concomitant autonomic symptoms (p=0,004) and concomitant neurological symptoms (p<0,001) or who have not. Correlation of total number of abnormal tests in ENG with patient age, age of the patient during first attack, number of the years after the diagnosis of MS, and number of total attacks was not statistically significant, but correlation of total number of abnormal tests in ENG with EDSS score was statistically significant (p= 0,039). These correlations were also investigated for all subtests, but any significant correlation was not found. Statistically significant difference was found between patients and controls in saccade test (p< 0,001), tracking test (p< 0,001), and positional test (p=0,026). These statistical analyses were also repeated in 22 RRMS patients who were not using any immune-modulating drug during the time that we performed ENG and we did not find any different result in the statistically analyses. Our study adds new knowledge to the literature as higher frequencies of non-vertiginous dizziness in first attack (70.0 %) and any time during the disease course (80.0 %); very low frequency (13.3 %) of patients without any vestibular symptom during the course of disease. In addition we observed that the patients who experience true vertigo during attacks have the history of true vertigo also in their first attacks. We also found that saccade test pathology is statistically different between the patients who have any vestibular symptom during the first or any attack and who have not. Existence of significant correlation between EDSS score and total number of abnormal subtests in ENG is also a new knowlegde. As a result ENG has high levels of sensitivity if all subtests evaluated in details even if in asymptomatic patients in RRMS. ENG is strongly suggested for both vestibular evaluation of MS patients and detection of involvement of a different sub-system when additional paraclinical evidence is needed

Multiple sclerosis coexisting with spinal cord ependymoma: A case report

Background - In this paper, we report a coexistence of multiple sclerosis and an intradural spinal cord tumor. Case report - A 34-year-old woman who had a history of relapsing-remitting multiple sclerosis for the last 15-years presented with acute sensory loss and spasticity in her left lower limb and her spinal magnetic resonance imaging study revealed an intradural spinal cord tumor in the lumbar spine, further diagnosed as ependymoma. Conclusion - We call attention to this rare association of MS and a spinal cord tumor, emphasizing the need for investigation of new symptoms during the evolution of MS

Panik ataklarını taklit eden epileptik nöbetleri olan bir hastada serebral kortikal migrasyon defektleri

Nöronal migrasyon anomalileri serebral neokorteks malformasyonlarına neden olabilir ve bu hastaların ilk başvuruları ve klinik gidişi değişkenlik gösterebilir. Bu yazıda, başlangıçta epizodik panik atakları olduğu düşünülen, ancak ileri incelemelerden sonra bunların serebral kortikal migrasyon anomalileri ile ilişkili epileptik nöbetler olduğu anlaşılan bir olgu sunulacaktır.Disorders of neuronal migration can lead to malformations of the cerebral neocortex and presentation and clinical course of patients these patients are variable. In this paper, we report a patient with episodic attacks initially thought to be panic attacks which on further investigation were identified to be seizure episodes to be associated with cerebral cortical migration abnormalities

Ciddi hipersomni ile prezente olan tek taraflı talamodienfalik sendrom

Talamus ve beyin sapı inmeleri organik hipersomniye neden olabilirler. Paramedian talamusun uyku regülasyonunda önemli rol oynadığına inanılır ve uyku regülasyon bozukluklarının paramedian talamus inmelerinde (PTİ) ortaya çıkabileceği bilinmektedir. Tek taraflı ve sınırlı lezyonu olan PTİ'li hastalarda hafif uyku hali daha önceden bildirilmiştir. Bu yazıda manyetik rezonans görüntülemede (MRG) sınırlı, tek taraflı talamodiensefalik iskemik lezyonu olan ancak ciddi hipesomni ile kendini gösteren bir olgu sunulmaktadır. 54 yaşında, erkek hasta hastanemize gün içi aşırı uyku hali yakınması ile başvurdu. Çekilen kranial MRG'de solda talamus seviyesinden başlayarak nükleus Ruber ve Edinger Westpall nükleusuna uzanan iskemik lezyon saptandı. İlk hafta hastanın gün boyu toplam uyku saati 23-24 saat olarak seyretti. Hastaya dopamin agonisti tedavisi başlandı (bromokriptin 2.5mg/gün başlanarak 7.5mg/gün dozuna çıkıldı). Bu tedavi ile hastanın gün boyu uyku saati basamaklı olarak azaldı ve iki ay sonra hasta normal vijilansına geri döndü. Talamus ve mezensefalik veya pontin tegmental retiküler formasyonu etkileyen lezyonlar hipersomninin nedenlerinden biridir fakat klinik prezentasyon ve hipersomninin derecesi beynin etkilenen bölgesine bağlı olarak oldukça değişken olabilmektedir. Uyku ve uyanıklığın farklı beyin yapılarınca regülasyonunun hala anlaşılması zor olduğu ve yeni olgu bildirileri ve klinik çalışmaların bu durumun daha iyi anlaşılabilmesi için faydalı olacağı görüşündeyiz.Thalamic and brainstem strokes can cause organic hypersomnia. Paramedian thalamus is believed to play an important role in the regulation of sleep, and disturbances of sleep regulation are known to occur in paramedian thalamic stroke (PTS). In patients with PTS, slight sleepiness is reported with unilateral and limited lesions. In this paper we report on a patient presenting with severe hypersomnia who has a limited, unilateral thalamodiencephalic ischemic lesion on magnetic resonance imaging (MRI). A 54 year-old man referred to our hospital with the complaint of excessive day time sleepiness. His cranial MRI revealed a unilateral ischaemic lesion beginning at the level of left thalamus and extending to nucleus Ruber and Edinger Westpall. His total day time sleep was about 23-24 hours in the first week. He had been put on dopamine agonist therapy (bromocriptine 2.5mg/day up to dose 7.5mg/day). During this medication his total day time sleep decreased gradually and after two months he returned to his normal vigilance. Lesions affecting thalamus and mesencephalic or pontine tegmental reticular formation are a cause of hypersomnia but clinical presentation and degree of hypersomnia can be various due to the affected region of the brain. We think that regulation of sleep and wakefulness by different brain structures are still elusive and new case reports and clinical trials would be helpful about clarification of this condition

Acquired immune deficiency syndrome showing its first symptom with nervous system involvement

Human immunodeficiency virus (HIV) infection is increasingly common today and the diversity of its clinical presentation makes the diagnosis difficult. The virus can cause neurologic involvement in the entire nervous system through direct action or opportunistic infections. HIV-associated neurocognitive disease, HIV encephalopathy, HIV-associated vacuolar myelopathy, and distal symmetric polyneuropathy are some of the neurologic involvements. The aim of this article was to present patients who were admitted to Pamukkale University Hospital, Neurology Clinic with neurologic symptoms as the first clinical evidence of HIV infection, and to compare their patterns of neurologic involvement with patterns in the literature. © 2020 by Turkish Neurological Society