Clinical Neuroscience

[Sturge Weber type 3 presenting with occipital epileptic seizure: case report ]

SERİNDAĞ Cansu Helin1, EREN Fulya1, KARAHAN Gökçen Muazzez1, GUL Gunay, SELCUK Hakan2, KARA Batuhan2, SOYSAL Aysun1

MARCH 30, 2019

Clinical Neuroscience - 2019;72(03-04)

DOI: https://doi.org/10.18071/isz.72.0131

[Sturge Weber syndrome is the third most common neurocutaneous syndrome after neurofibromatosis and tuberous sclerosis. Three distinct types were identified. Type 3 with leptomeningeal involvement alone is the rarest among other types. The reported case is a 21-years-old female patient without any known chronic disease. She admitted to the emergency department after visual symptoms and headache, followed by generalized tonic clonic seizure. EEG of the patient showed left occipital seizure activity. The contrast enhanced magnetic resonance imaging (MRI) showed left occipital leptomeningeal angioma. Digital substraction angiography (DSA) revealed minimal blushed contrast enhancement on late venous phase and lack of superficial cortical veins. Her focal seizures were under control with levatiracetam and lacosamide treatment. The reported case is unique because of the late onset presentation with focal seizure without mental retardation.]

AFFILIATIONS

  1. Bakirkoy Prof. Dr. Mazhar Osman Education and Research Hospital For Neuropsychiatry, Neurology Clinic, İstanbul, Turkey
  2. Bakirkoy Dr. Sadi Konuk Education and Research Hospital, Radiology Clinic, İstanbul, Turkey

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Clinical Neuroscience

EEG-based connectivity in patients with partial seizures with and without generalization

DÖMÖTÖR Johanna, CLEMENS Béla, EMRI Miklós, PUSKÁS Szilvia, FEKETE István

Objective - to investigate the neurophysiological basis of secondary generalization of partial epileptic seizures. Patients and methods - inter-ictal, resting-state EEG functional connectivity (EEGfC) was evaluated and compared: patients with exclusively simple partial seizures (sp group) were compared to patients with simple partial and secondary generalized seizures (spsg group); patients with exclusively complex partial seizures (cp group) were compared to patients with cp and secondary generalized seizures (cpsg group); the collapsed sp+cp group (spcp) was compared to those who had exclusively secondary generalized seizures (sg group). EEGfC was computed from 21-channel waking EEG. 3 minutes of waking EEG background activity was analyzed by the LORETA Source Correlation (LSC) software. Current source density time series were computed for 23 pre-defined cortical regions (ROI) in each hemisphere, for the 1-25 Hz very narrow bands (1 Hz bandwidth). Thereafter Pearson correlation coefficients were calculated between all pairs of ROI time series in the same hemisphere. Z-scored correlation coefficients were compared at the group level (t-tests and correction for multiple comparisons by local false discovery rate, FDR). Results - Statistically significant (corrected p<0.05) EEGfC differences emerged at specific frequencies (spsg > sg; cpsg > cp), and at many frequencies (sg > spcp). The findings indicated increased coupling between motor cortices and several non-motor areas in patients with partial and sg seizures as compared to patients with partial seizures and no sg seizures. Further findings suggested increased coupling between medial parietal-occipital areas (structural core of the cortex) and lateral hemispheric areas. Conclusion - increased inter-ictal EEGfC is associated with habitual occurrence of secondary generalized seizures.

Clinical Neuroscience

The yield of electroencephalography in syncope

NALBANTOGLU Mecbure, TAN Ozturk Ozlem

Introduction - Syncope is defined as a brief transient loss of consciousness due to cerebral hypoperfusion. Although the diagnosis of syncope is based on a thorough history and examination, electroencaphalography (EEG) is also an important investigational tool in the differential diagnosis in this group of patients. In this study we aimed to identify the diagnostic value of EEG in patients with syncope. Methods - We retrospectively examined EEG recordings of 288 patients with the diagnosis of syncope referred to the Cankiri State Hospital EEG laboratory, from January 2014 to January 2016. The EEG findings were classified into 6 groups as normal, epileptiform discharges (spike and sharp waves), generalized background slowing, focal slowing, hemispherical asymmetries, and low amplitude EEG tracing. The EEGs were separated according to gender and age. Results - Total of 288 patients were included in this study, 148 were females (51.4%) and 140 (48.6%) were males. Among all the EEG reports, 203 (70.5%) were normal, 8 of them (2.8%) showed generalized background slowing and 7 (2.4%) demonstrated focal slow waves. Epileptiform discharges occured among 13 patients (4.5%). Hemispherical asymmetries were detected in 10 patients (3.5%) and low amplitude EEG tracing in 47 patients (16.3%). There was no significant difference between age groups in EEG findings (p=0.3). Also no significant difference was detected in EEG results by gender (p=0.2). Discussion - Although the diagnosis of syncope, epilepsy and non-epileptic seizures is clinical diagnosis, EEG still remains additional method

Clinical Neuroscience

[Rehabilitation possibilities and results after neurosurgical intervention of brain tumors ]

DÉNES Zoltán, TARJÁNYI Szilvia, NAGY Helga

[Objectives - Authors examined the rehabilitation possi­bi­lities, necessities, and results of patients after operation with brain tumor, and report their experiences. Method - Retrospective, descriptive study at the Brain Injury Rehabilitation Unit, in National Institute for Medical Rehabilitation. Patients - Patients were admitted consecutively after rehabilitation consultation, from different hospitals, following surgical intervention of brain tumors, between 01 January 2001 and 31 December 2016. Patients participated in a postacute inpatient rehabilitation program, in multidisciplinary team-work, leaded by Physical and Rehabilitation Medicine specialist included the following activities: rehabilitation nursing, physical, occupational, speech, psychological and neuropsychological therapy. Results - At the rehabilitation unit, in the sixteen-year period 84 patients were treated after operation with brain tumor. Patients arrived at the unit after an average of 41 days to the time of the surgical intervention (range: 10-139 days), and the mean length of rehabilitation stay was 49 days (range: 2-193 days). The mean age of patients was 58 years (20-91), who were 34 men and 50 women. The main symptoms were hemiparesis (64), cognitive problems (26), dysphagia (23), aphasia (16), ataxia (15), tetraparesis (5), and paraparesis (1). The mean Barthel Index at the time of admission was 35 points, whereas this value was 75 points at discharge. After the inpatient rehabilitation, 73 patients improved functionally, the status of 9 patients did not show clinically relevant changes, and 2 patients deteriorated. During the rehabilitation 10 patients required urgent interhospital transfer to brain surgery units, 9 patients continued their oncological treatment, two patients continued rehabilitation treatment at another rehabilitation unit, and after rehabilitation 73 patients were discharged to their homes. Conclusions - Inpatient rehabilitation treatment could be necessary after operation of patients with brain tumor especially when functional disorders (disability) are present. Consultation is obligatory among the neurosurgeon, rehabilitation physician and the patient to set realistic rehabilitation goals and determine place and method of rehabilitation treatment, but even at malignancies cooperation with oncological specialist also needed. Authors’ experience shows benefits of multidisciplinary rehabilitation for patients after brain tumor surgery. ]

Clinical Neuroscience

Secretory meningioma with bone infiltration and orbital spreading

KÁLOVITS Ferenc, TAKÁTS Lajos, SOMOGYI Katalin, GARZULY Ferenc, TOMPA Márton, KÁLMÁN Bernadette

Secretory meningioma is a rare form of meningiomas which differentiates from the meningothelial subtype. It is characterized by significant peritumor edema and distinct immunohistochemical and molecular genetic profiles. We present a middle aged female patient with secretory meningioma infiltrating the orbital bone from the primary cranial base location and causing exophthalmos, features rarely described with this tumor. Surgical resection was challenging because of the associated brain swelling and rich vascularization of the tumor. Imaging and immunohistochemical studies revealed characteristic hallmarks of secretory meningioma. While histologically it was a benign tumor, due to the orbital bone and soft tissue infiltration, postoperative management of neurological sequelae was challenging. This case highlights distinctive clinical, imaging and histological features along with individual characteristics of a rare form of meningiomas.

Clinical Neuroscience

Incidental intracranial lipomas: Assessment of 163 patients

TÜRK Okan, YALDIZ Can

Intracranial lipomas (ILs) are rare congenital lesions which consist 0.1-0.5% of intracranial lesions. They are usually asymptomatic. Our serial of 163 patients is the largest IL serial in literature. Files of the patients who were diagnosed with IL at outpatient clinic of neurosurgery between 2009 and 2018 were screened retrospectively. A total of 163 patients were detected to have been diagnosed with IL according to radiologic findings between 2009 and 2018. Of the patients, 96 were female and 67 were male. Intracranial lipomas are self-limited, slowly growing benign lesions which do not cause a mass effect. We believe that the present study would be a main source due to the currently available insufficient number of studies in literature.

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Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

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Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

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[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

Clinical Neuroscience

Creutzfeldt-Jakob Disease: A single center experience and systemic analysis of cases in Turkey

USLU Ilgen Ferda, ELIF Gökçal, GÜRSOY Esra Azize, KOLUKISA Mehmet, YILDIZ Babacan Gulsen

We aimed to analyze the clinical, laboratory and neuroimaging findings in patients with sporadic Creutzfeldt-Jakob disease (CJD) in a single center as well as to review other published cases in Turkey. Between January 1st, 2014 and June 31st, 2017, all CJD cases were evaluated based on clinical findings, differential diagnosis, the previous misdiagnosis, electroencephalography (EEG), cerebrospinal fluid and cranial magnetic resonance imaging (MRI) findings in our center. All published cases in Turkey between 2005-2018 were also reviewed. In a total of 13 patients, progressive cognitive decline was the most common presenting symptom. Two patients had a diagnosis of Heidenhain variant, 1 patient had a diagnosis of Oppenheimer-Brownell variant. Seven patients (53.3%) had been misdiagnosed with depression, vascular dementia, normal pressure hydrocephalus or encephalitis. Eleven patients (87%) had typical MRI findings but only 5 of these were present at baseline. Asymmetrical high signal abnormalities on MRI were observed in 4 patients. Five patients (45.4%) had periodic spike wave complexes on EEG, all appeared during the follow-up. There were 74 published cases in Turkey bet­ween 2005 and 2018, with various clinical presentations. CJD has a variety of clinical features in our patient series as well as in cases reported in Turkey. Although progressive cognitive decline is the most common presenting symptom, unusual manifestations in early stages of the disease might cause misdiagnosis. Variant forms should be kept in mind in patients with isolated visual or cerebellar symptoms. MRI and EEG should be repeated during follow-up period if the clinical suspicion still exists.

Clinical Neuroscience

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Clinical Neuroscience

Simultaneous subdural, subarachnoideal and intracerebral haemorrhage after rupture of a peripheral middle cerebral artery aneurysm

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The cause of intracerebral, subarachnoid and subdural haemorrhage is different, and the simultaneous appearance in the same case is extremely rare. We describe the case of a patient with a ruptured aneurysm on the distal segment of the middle cerebral artery, with a concomitant subdural and intracerebral haemorrhage, and a subsequent secondary brainstem (Duret) haemorrhage. The 59-year-old woman had hypertension and diabetes in her medical history. She experienced anomic aphasia and left-sided headache starting one day before admission. She had no trauma. A few minutes after admission she suddenly became comatose, her breathing became superficial. Non-contrast CT revealed left sided fronto-parietal subdural and subarachnoid and intracerebral haemorrhage, and bleeding was also observed in the right pontine region. The patient had leucocytosis and hyperglycemia but normal hemostasis. After the subdural haemorrhage had been evacuated, the patient was transferred to intensive care unit. Sepsis developed. Echocardiography did not detect endocarditis. Neurological status, vigilance gradually improved. The rehabilitation process was interrupted by epileptic status. Control CT and CT angiography proved an aneurysm in the peripheral part of the left middle cerebral artery, which was later clipped. Histolo­gical examination excluded mycotic etiology of the aneu­rysm and “normal aneurysm wall” was described. The brain stem haemorrhage – Duret bleeding – was presumably caused by a sudden increase in intracranial pressure due to the supratentorial space occupying process and consequential trans-tentorial herniation. This case is a rarity, as the patient not only survived, but lives an active life with some residual symptoms.