Eylem Degirmenci

Acute Transverse Myelitis at the Conus Medullaris Level After Rabies Vaccination in a Patient With Behçet's Disease

Abstract Case report: A 25-year-old man with BehÇets disease was admitted because of weakness of the lower limbs and difficulty in urination. He had received a rabies vaccination 2 months previous because he had been bitten by a dog. Findings: Clinical and laboratory findings supported acute transverse myelitis. A hyperintense lesion and expansion at the level of conus medullaris was detected on spinal magnetic resonance imaging. Conclusion: Although neurologic involvement is one of the main causes of mortality and morbidity in BehÇets disease, the factors that aggravate the involvement of the nervous system are still unclear. Vaccination may have been the factor that had activated autoimmune mechanisms in this case. To our knowledge, involvement of the conus medullaris in BehÇets disease after rabies vaccination has not been reported.

Correlation between blink reflex abnormalities and magnetic resonance imaging findings in patients with multiple sclerosis

This study investigates the correlation between brain magnetic resonance imaging findings and blink reflex abnormalities in patients with relapsing remitting multiple sclerosis. Twenty-six patients and 17 healthy subjects were included in this study. Blink reflex test (BRT) results were obtained using right and left stimulations; thus, 52 BRT results were recorded for the patient group, and 34 BRT results were recorded for the control group. The magnetic resonance imaging (MRI) findings were classified based on the existence of brainstem lesions (hyperintense lesion on T2 weighted (W) and fast fluid-attenuated inversion recovery MRI or contrast-enhancing lesion on T1W MRI). Correlation analysis was performed for the BRT and MRI findings. The percentage of individuals with abnormal BRT results (including R1 latency, ipsilateral R2 latency, and contralateral R2 latency) was significantly higher in the patient group as compared to the control group (p values: 0.015, 0.001, and 0.002, respectively). Correlation analysis revealed significant correlations between contralateral R2 latency abnormalities and brainstem lesions (p value: 0.011). Our results showed significant correlation correlations between contralateral R2 latency abnormalities and brainstem lesions and these results may be explained the effects of multiple demyelinating lesions of the brain stem of patients with relapsing remitting multiple sclerosis.

Clinical and electronystagmographical evaluation of vestibular symptoms in relapsing remitting multiple sclerosis

Abstract Objective: Multiple sclerosis (MS) may give rise to a variety of clinical signs and symptoms including vertigo and/or other problems related with equilibrium. In this study, we aimed to evaluate clinical and electronystagmographical (ENG) characteristics of relapsing remitting MS (RRMS) patients. Design: This is a prospective controlled study consisting of 30 patients who were diagnosed as definite RRMS according to McDonalds diagnostic criteria and 30 healthy individuals. Setting: Entire population of patients were examined and followed up at the same tertiary centre during the period of September 2003 and March 2005. Clinical examination and detailed electronystagmographic investigations were performed in each group. Methods: Vestibular laboratory testing was carried out by a computerized ENG system. All ENG subtests including tracking, saccade, optokinetic, gaze, positional and Dix-Hallpike tests were performed in each group but caloric, which is relatively an invasive test, was performed only in the patient group. Main outcome measures: We aimed to find the ratio of abnormal tests indicating, central and/or peripheral pathology in ENG. We also analyzed the correlation of total number of abnormal tests in ENG with clinical parameters. Results: Differences of ENG abnormality indicating central and/or peripheral pathology and ENG abnormality indicating only central pathology between the two groups were statistically significant. Correlation of total number of abnormal tests in ENG with EDSS score was statistically significant. Conclusion: ENG is sensitive in detecting the vestibular system involvement in RRMS patients if all subtests are performed and evaluated in detail with clinical symptoms and signs.

Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) in a patient presenting with papilledema

MERS is a clinically mild encephalitis/encephalopathy characterized by the MRI finding of a reversible lesion with transiently reduced diffusion in the corpus callosum at least involving the splenium of the corpus callosum (SCC) [1]. The most common neurological symptom is delirious behavior, followed by consciousness disturbance, and seizures, all of which completely recover within a month. Takanashi et al. [2] first described a solitary lesion in the splenium of the corpus callosum on magnetic resonance imaging (MRI) in two patients with influenza-associated encephalitis/encephalopathy in 2004. Such lesions of the SCC are reported various types of viral infections [3, 4]. In this paper, we report on an adult patient who presented with papilledema diagnosed as MERS due to viral encephalitis/encephalopathy. A previously healthy 27-year-old man referred to our hospital from a psychiatry clinic with the complaints of headache, apathy, indifference, and rarely agitation for 10 days. The patient reported that he had complained about nausea and vomited two times in last 2 days. There was bilateral papilledema and mildly altered mental status in his neurological examination. There was no neck stiffness and the meningeal irritation signs were negative. His routine blood tests, initial cranial imaging findings including the conventional MR, MR-venography, computed tomography (CT) angiography images of cerebral vessels and electroencephalography were unremarkable. Further diagnostic tests to evaluate papilledema and mild encephalopathy such a young, male patient including vasculitis markers, paraneoplastic markers and autoimmune encephalitis’ markers (Potassium channel complex antibody, NMDA antibody and thyroid auto-antibodies) were negative. Lumbar puncture showed elevated cerebrospinal fluid (CSF) pressure of 31 cm H2O and elevated CSF protein of 114.9 mg/dL. Glucose concentration of CSF was 62 mg/dL (Blood glucose 96 mg/dL) and direct microscopic evaluation showed no cells. Microscopic and serological examination of CSF for mycobacterium tuberculosis, neurobrucellosis, syphilis, and Lyme disease was negative. In addition, Mycobacterium tuberculosis Real-Time PCR and Herpes virus Real-Time PCR were unremarkable. The patient was put on acetazolamide (1,000 mg/day) and empirical antibiotherapy (Ceftriaxone 2 9 2 g IV) and his headache and papilledema resolved in the follow-up, but his control LP showed 150 leukocytes/mm with a normal opening CSF pressure of 15 cm H2O. In addition, CSF protein was found to be increased at a level of 365.2 mg/dL. All repeated microbiological analyses were again negative. The control brain MR images demonstrated a solitary, non-enhancing, ovoid lesion (10 9 5 mm in E. Degirmenci (&) Department of Neurology, School of Medicine, Pamukkale University, Denizli, Turkey e-mail: eylemteke@yahoo.com

Cognitive Insight in Alzheimer’s Disease

Background/rationale: We investigated the cognitive insight profile of patients with Alzheimer’s disease (AD) using the Beck Cognitive Insight Scale (BCIS). Methods: This study involved 30 patients with probable AD and 15 healthy participants (ie, the controls). All individuals completed the BCIS, the Hamilton Rating Scale for Depression (HAMD), and the Hospital Anxiety and Depression Scale (HADS). Results: Mean scores of the HADS-depression subscale, HAMD, BCIS-self-reflectiveness (BCIS-R), and BCIS-self-certainty (BCIS-C) subscales were significantly different between the patients and the controls. However, there was no significant difference in BCIS reflectiveness–certainty index scores between the patients and the control groups. Regression analyses showed a moderately positive correlation between hallucinations and BCIS-C scores. Conclusion: This study is the first to investigate cognitive insight in patients with probable AD. The BCIS-R and BCIS-C scores were significantly lower in patients than in control group.

Nerve excitability properties in early preclinical diabetic neuropathy

Diabetic polyneuropathy can be easily diagnosed when the nerve conduction studies are affected. Strength Duration Time (SDTc) reflects nerve excitability properties and was previously used several times to demonstrate the excitability properties of the nerves in the existence of electrophysiologically developed diabetic polyneuropathy. But as we all know, diabetic patients may experience neuropathic symptoms even though their routine nerve conduction studies are normal. SDTc may be useful in this early stages of developing neuropathy. In this study we aimed to evaluate the SDTc properties of diabetic patients in this early preclinic stage. Recently SDTc was commonly studied in the upper extremities but most of the diabetic neuropathies are predominant in the lower extremities. So here we also studied both upper and lower extremities to demonstrate a possible difference.

Moyamoya syndrome or Behçet's disease?

A 28-year-old woman presented with involuntary movements on her right arm and leg that started 4 years ago and were periodical, recurring each autumn and lasting about 10 days. Her medical history revealed that she had recurrent (4–5 times/year) painful, oral ulcers and skin lesions for the last 3 years and physical examination showed the scar of a genital ulcer (Fig. 1a). She did not report any uveitis attack. The pathergy test was negative. The only abnormality on her neurological examination was cerebellar signs on the right. Brain magnetic resonance imaging (MRI) revealed multiple hyperintense punctate lesions predominantly located at the level of the cortico-subcortical junction in T2-weighted and fluid-attenuated inversion recovery (FLAIR) images. There also was a dense contrast enhancement in the meningo-vasculer structures (Fig. 1b,c). Digital subtraction angiography demonstrated occlusion of the bilateral distal carotid arteries and basilar artery but a large basal vascular collateral circulation resulting in the smoke-like appearance suggestive of Moyamoya disease (Fig. 1d,e). Perfusion MRI showed relative diminished perfusion of the vascular area of the right posterior cerebral artery (Fig. 1e,f). Extensive laboratory tests, including studies for hypercoagulable states and infectious diseases, anti-cardiolipin antibodies, double-stranded DNA antibodies, SM antibodies, ribonucleoprotein antibodies, Sj€ ogren’s syndrome antigen (SSA) and SSB antibodies were unremarkable. Examination of cerebrospinal fluid (CSF) was normal, including opening pressure of 18 cm H2O, CSF protein (18.9 mg/dL), CSF glucose (65 mg/dL), immunoglobulin index (0.6), CSF microscopic evaluations and CSF cultures. The patient was consulted by a rheumatologist to exclude other vasculotides and autoimmune diseases. Complement levels, erythrocyte sedimentation rate and C-reactive protein levels were also normal. Antinuclear antibodies were positive with low titration (1/100), and human leukocyte antigen (HLA)B51 was positive, consistent with Behc et’s disease. The patient was hospitalized during the diagnostic evaluations and the neurological complaints were totally resolved after bed rest and intravenous hydration of 2500 mL/day. WHAT IS YOUR DIAGNOSIS? Moyamoya syndrome associated with Behc ets disease

Transient electromyographic findings in serotonergic toxicity due to combination of essitalopram and isoniazid

Here, we report a case of serotonergic toxicity due to combination of essitalopram and isoniazid, which was rarely reported before. Moreover, we observed transient neurogenic denervation potentials in needle electromyography, which disappeared with the treatment of serotonergic toxicity. As to our best knowledge, this is the first case, reporting transient electromyographic changes probably due to serotonergic toxicity.

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

BackgroundThis 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.MethodsAn 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.ResultsThe 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.ConclusionsBundles 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.Conduction parameters of nerve fibers with different diameters in the same peripheral nerve can be estimated.

Remarkable effect of benzodiazepine in a patient with anti-NMDA receptor encephalitis.

Here, we report a case of anti-NMDA receptor encephalitis with a history of vaccination in which peroral low dosages of benzodiazepines caused reversible episodes of wakefulness. A 42-year-old male patient presented with a reversible attack of blurred vision, paresthesia in his right hand followed by psychiatric symptoms, visual hallucinations, and episodes of disorientation. In the end of the first month, he experienced seizures. He had a history of vaccination just 3 weeks before the symptoms started. Cranial imaging was within normal limits. EEG demonstrated persistent, repeated sharp waves localized to left parietal cortex. Cerebrospinal fluid (CSF) examination revealed an elevated protein level without any cells or microorganism. All infectious markers were negative. Antinuclear antibody profile, lupus anticoagulants were also negative. Whole body 18F-fluorodeoxyglycose positron emission tomograpghy (FDG-PET) demonstrated hyperactivity in bilateral temporoparietal cortex. Treatment with acyclovir and meropenem was initiated. Seizures were under control with levetiracetam treatment (2,000 mg daily). The sharp waves previously observed in the left hemisphere were no longer present after treatment. In his follow-up, the patient progressively became unresponsive and the clinical picture deteriorated to a catatonic state. With the suspicious of nonconvulsive status epilepticus, he received intravenous benzodiazepine (Bz) which caused a period of wakefulness lasting for 15 min. Phenytoin was added to the treatment, but despite an adequate free phenytoin serum level of 22 mg/ml, no beneficial clinical effect was seen, and the patient was still unresponsive. Twenty-four hour EEG monitoring demonstrated mild diffuse slowing (6–7 Hz) without any epileptic discharges. This remarkable clinical effect was observed after diazepam injection but not with other antiepileptic drugs, and the absence of obvious EEG findings suggested a pathology rather than nonconvulsant status epilepticus. Low dosage of peroral lorazepam (1 mg) also caused a 2-h period of wakefulness which also supported another diagnosis rather than status epilepticus. Then, the dosage was increased to 4 9 1 mg daily which provided a day time wakefulness. On the other hand, he returned to his unresponsive status when the drug was delayed. Further investigation demonstrated antibodies to NMDA receptors in CSF. No tumor was found by whole body FDG-PET, testicular ultrasound, or chest tomography. At the end of the second month from symptom onset, all anti-infectious agents were stopped step by step and the patient was put on high dosage (1,000 mg) IV pulse corticosteroid treatment for 5 days followed by a 1-mg/kg oral dose regimen. After 2 weeks of treatment, he was awake but still experienced hallucinations and episodes of disorientation so he received seven cycles of plasma exchange (Pex) on alternate days. After Pex, oral benzodiazepine was gradually stopped, and the patient was still awake and was able to perform daily tasks. Ç. Erdoğan (&) E. Değirmenci A. Oğuzhanoğlu Department of Neurology, Pamukkale University, Kinikli, Denizli, Turkey e-mail: drcagdaserdogan@gmail.com