Neurological Disorders

Pseudotumor cerebri has been described after exposure to steroids as well as following steroid withdrawal, causing adrenocortical insufficiency resulting in reduction of cerebrospinal fluid absorption and leading to intracranial hypertension. In our case, the patient had increased intracranial pressure caused by hypocortisolism. Cases of pseudotumor cerebri after resection of an ACTH-secreting adenoma has been reported in the literature, more often in children than in adults, and mostly occurring after steroid taper compared to steroid excess states. Our case highlights the importance of considering recurrence of Cushing’s syndrome in adults presenting with isolated cranial nerve palsy and pseudotumor cerebri.

A 65 year-old female patient with past medical history of diabetes mellitus, hypertension and ischemic heart disease presented with acute onset of right hemiplegia, right facial palsy and dysphasia. Urgent CT brain showed hypodensity involving the territory of the lower division of left middle cerebral artery denoting recent infarction. On admission the patient was found to have Atrial Fibrillation (AF) and LMW Heparin was started followed by Warfarin. One week after initiation of Warfarin the patient started to have headache, increased weakness with deterioration of the level of consciousness. Urgent NECT brain was done and showed hemorrhagic transformation, mass effect and “Blood-fluid level” (Figure 1). Her international normalized ratio (INR) was 3.5.

Metastasis forms the most common brain malignancy. Solitary or multiple ring enhancing intra-axial lesions bordered on the gray-white matter junction with disproportionate edema is the usual pattern on imaging. No reports of high protein content parenchymal metastatic lesion can be found in literature till now. Authors here describe a case of 42 year old female presenting with features of intracranial hypertension and imaging suggestive of a high protein containing lesion. Gross total resection was performed and histopathological features were suggestive of metastatic papillary adenocarcinoma.

Objective: To describe a case series of patients with pituitary adenomas with endoscopic endonasal transsphenoidal approach, the technique performed and complications in our centre.
Methods: The technique performed in a series of 98 consecutive patients, and description of their complications and the protocol followed to treat these complications.
Results: 120 patients where submitted to Skull Base Endoscopic Surgery, of which 98 (81.66%) had diagnosis of non-functioning pituitary adenoma in a two year period. Complications described are postoperative cerebrospinal fluid leaks (6.12%), vascular injury (3.06%) and cranial nerve injury (2.04%).
Conclusion: A specific protocol is followed in our institution to treat different complications in pituitary adenoma transnasal endoscopic surgery in order to improve outcomes. Prompt and diligent identification of complications is of utmost importance for their treatment.

Hemangiopericytoma (HPC) is a rare tumor, accounting for less than 1% of intracranial tumors. Though it’s notoriously difficult to distinguish HPC from meningioma in pre-op images, HPC presents bone and vascular invasion, high local recurrence and distal metastasis clinically, as a surgically challenging malignancy. High-flow extracranial-intracranial (EC-IC) bypass is a skill demanding surgery and is usually performed as a wellprepared elective surgery. However, emergency EC-IC bypass is indicated for unplanned major artery loss during surgery and sometimes as the final rescue for the patient. We present a case of 46 year-old man, who was diagnosed of a left anterior clinoid process (ACP) tumor with presentation of gradually visual loss. As the dura tail sign and MRI signal, we performed left pterional approach tumor excision for the impression of ACP meningioma. However, due to the vascular invasion of tumor, left ICA perforation was noted intraoperatively and finally the bleeding was controlled with ICA clipping. Emergency EC-IC bypass from common carotid artery to middle cerebral artery was performed. The patient recovered to oriented consciousness, independent ambulation but mild aphasia in our outpatient department follow-up.

Stroke is the third leading cause of death in world. According to the Centers for Disease Control and Prevention (CDC), the incidence is around 1 in every 40 seconds and the death rate is in every 4 minutes in USA. People under the age of 65 are prone to 255 of stroke attacks. Today, approximately 6.2 million people are living with the consequences of stroke. Incidence is nearly double that of Caucasians and African Americans suffer more extensive physical impairments. This module provides trainers with an outline for the teaching of the basic care for stroke patients. Participants will learn about stroke as a disease and its nature then basic care for stroke patients will be demonstrated.

Sialic acid supports nerve cell regeneration, differentiation and neuronal plasticity. Especially, polysialic acid (polySia) chains which are built up by α2,8-linked Neu5Ac Neu5Ac residues influence by their specific interactions with polySia receptors neuronal processes related to tumor spread and differentiation processes. With a combination of biophysical and biochemical methods including molecular modeling as described here it is possible to support cell biological experiments and in vivo studies on a nanoscale level. The submolecular analytical approaches which are directed to crucial functional groups focus on the potential therapeutic impact of sialic acids and in particular polySia. Such results are helpful for the development of new drugs which might have a high clinical relevance in respect to the therapy of various diseases correlated to neuronal regeneration, tumor spread and infections. It is not surprising that several diseases belonging to these different clinical fields (e.g. oncology, infection diseases, neuronal disorder) can be treated as indicated because sialic acids represent essential contact structures on numerous cell surfaces in dependence to their state of differentiation.

Background: Achieving haemostasis during surgical procedures within the brain is of paramount importance. Bipolar cauterization is most effectively used to occlude identifiable vessels but it has minimal efficacy in controlling the oozing bleeding that characterises most neurological pathologies. In such cases chemical haemostatic agents are often preferable to bipolar cautery. Rarely these chemical agents may produce a foreign-body reaction that resembles infectious or malignant processes. On imaging significant oedema and ring-like enhancement on postcontrast studies may falsely confirm the suspect of such processes, requiring new surgical intervention.
Methods: In this report a reaction to a chemical haemostatic agent in a patient operated on for a low grade glioma causing focal epilepsy is discussed.
Results: After a two year follow-up the patient is seizure free without neurological deficits, and MRI scans do not show any significant focal alteration except for surgical scar.
Conclusions: The literature reports sporadic cases of foreign-body reaction to haemostatic agents that may mimic neoplastic recurrency or post-surgical infectious complications on imaging studies, but this complication is still considered exceedingly rare. Our case, however, confirms this possibility, raising the suspicion that it may be overlooked when imaging studies are not performed soon after surgery or if the reaction happens in a silent cerebral area.

Fourteen year old female patient referred in June 2013 to the Emergency Department due to seizures of recent onset. The patient had a six-month history of behavioral changes and impaired academic performance; also, days before the seizure episodes she developed vomiting. The physical examination documented bradypsychia and papilledema. The results of hormone testing showed panhypopituitarism, a condition of the hypothalamic-hypophysis axis. An MRI was performed and it showed a heterogeneous tumor that compromises the frontal periventricular white matter extending through the rostrum of the corpus callosum, infiltrates the ependyma, it is heterogeneous with cystic areas; after contrast, it presents heterogeneous enhancement. In univoxel MR spectroscopy, it was observed an increase of choline, lipid and lactate, and a decrease in N-acetyl aspartate. It associates with the existence of edema in the bifrontal white matter, there is mass effect given by the compression of the frontal recesses of the lateral ventricles and effacement of the frontal anterior sulci. There is a second lesion that compromises the tuber cinereum, pituitary stalk and optic chiasm, it enhances after contrast administration and presents cystic areas. For the morphological characteristics and changes in spectroscopy, differential diagnosis as primitive neuroectodermal tumor (PNET) and glioblastoma were planted. The frozen biopsy reports a small celled tumor: PNET vs. Lymphoma. Due to the latter, a complete macroscopic resection is performed via a bifrontal craniotomy with an interhemispheric approach. Diabetes insipidus is developed immediately during the postoperative period. The full section biopsy reported a germinoma. Managed through sequential chemotherapy and radiation therapy.

Neurological complications are a potential risk after spine surgery with various reported rates. This a case series of a single surgeon’s experience of neurological complications following spine surgery between the 2007-2010 with review of the current recommendations regarding the use of intra-operative neuro monitoring during spine surgery. In the period between 2007-2010, 215 spine procedures were performed by single surgeon in all regions of the spine for various pathological conditions (deformity, tumor, infection, trauma, and degenerative), 61% were female patients with an average age of 37 years. Patients with preoperative complete neurological loss where excluded from this review. Intra operative neuro monitoring was used in 27 cases. Three patients (1.4%) wake up from surgery with new neurological deficit and after urgent appropriate imaging studies, 2 were taking back to the operating rooms within few hours of their index operation. Both made full recovery of their deficit in the postoperative period. The 3rd patient’ s lower ext paralysis was observed and over time. He was able to regain his ambulation ability. The incidence of neurological complication in this series is very similar to other reports, higher risk procedures should be done under intra operative neuro monitoring. This report could serve as guide for counseling of patients pre operatively regarding the potential for such adverse effects.

Background: Reliable, quantitative progression markers for Parkinson’s disease (PD) are needed. We aimed to determine whether the quantitative analyses of the cerebrovascular, cardiovascular autonomic and small fiber polyneuropathy features correlate with PD severity. Methods: This was a single center, retrospective study. PD patients were evaluated using standardized cardiovascular autonomic reflex testing including deep breathing, Valsalva maneuver, tilt test and skin biopsies for the quantification of epidermal sensory and sweat gland sudomotor fibers. Parkinsonism severity was quantified by the Unified Parkinson’sDisease Rating Scale (UPDRS). Severity of non-motor symptoms was evaluated using QASAT (Quantitative scale for grading of cardiovascular reflex tests, QSART and small fibers densities from skin biopsies) adjusted for age and gender. Correlations were obtained using the Pearson’s correlation coefficient (r). Results: We tested 109 PD patients (age 70.14 ± 11.2, disease duration 4.8 ± 4.06 years). UPDRS-II was correlated with the following QASAT scores: total (r=0.62, p<0.0001), adrenergic function (r=0.71, p<0.001), sensory fiber analysis (epidermal nerve fiber density or ENFD, r=0.34, p<0.001), cerebral blood flow scores (r=0.43, p<0.007) and sudomotor function (r=0.42, p<0.0001). UPDRS-III correlated with the following QASAT scores: total (r=0.46, p<0.0017), adrenergic function (r=0.48, p<0.0001), ENFD (r=0.31, p<0.001) and sudomotor function (r=0.34, p<0.05). Conclusions: Autonomic, cerebrovascular and sensory abnormalities coexist in PD and they progress along with disease severity. The adrenergic score, that primarily measures the severity and duration of orthostatic hypotension during tilt test, had the best correlation with disease severity. QASAT may be a feasible instrument for the objective monitoring of PD progression presenting with autonomic nervous system involvement. These findings need to be validated in a prospective study.

Background: Deep brain stimulation (DBS) of the subthalamic nucleus is an effective treatment option for patients with Parkinson’s disease (PD). The risk factors associated with the occurrence of psychiatric symptoms have not been completely elucidated.
Methods: We conducted a naturalistic observational study on patients with PD who would be subjected to DBS. Clinical data, including motor functions, cognitive functions, mental status, and daily dosage of anti-PD drugs were monitored. A psychiatrist evaluated their psychiatric symptoms at the initial assessment, three months, and one year after DBS.
Results: We evaluated 44 participants with PD, of which 32 were subjected to DBS. Thirteen participants were diagnosed with mental disorders at the initial assessment. Twenty-six patients were reassessed at three months after surgery, and 19 participants were reassessed at one year. At three months, the motor function of the participants was significantly improved, and the mean anti-PD drug dose was significantly decreased. Sixteen participants were experiencing some psychiatric symptoms, of which 12 were considered as worsened due to DBS, whereas 6 participants experienced improved mental state. At one year, 6 participants were suffering from some psychiatric symptoms caused by DBS, whereas improvement was observed in 6 participants. An exploratory analysis revealed that participants without dopamine dysregulation syndrome (DDS) at the screening were likely to improve their psychiatric symptoms.
Conclusion: Although DBS caused some psychiatric complications, mental status was improved in some patients in a longitudinal course. DDS is possible to predict poor outcome in psychiatric complication after DBS in PD patients.

We are facing and extremely exciting era after knowing the results of the recently finished clinical trials on the effectiveness of endovascular thrombectomy in acute stroke patients with large vessel occlusion [1-3]. The MRCLEAN, ESCAPE, EXTEND-IA and SWIFT-PRIME trials showed that a significant proportion of patients with acute stroke may greatly benefit from endovascular thrombectomy with the current generation of stentriever devices and these results are expected to be confirmed by the REVASCAT trial [4] to be presented in the upcoming European Stroke Organization Conference in Glasgow next April. One of the most interesting aspects of these studies is that patients with poor prognostic factors such as advanced age or tandem occlusions also benefit from endovascular thrombectomy, because their functional outcome is terrible when only medical therapy is used.

Hydatid disease is a rare parasitic disease caused by the larval stage of Echinococcus granulosus. It is endemic in many areas including the Middle-East and the Mediterranean countries. It mainly affects the liver and the lungs but can rarely affect other organs including the brain (Neurohydatidosis). Intracranial hydatid cysts account for 0.5-3% of all the cases of hydatid disease and contribute to 1-2% of all the intracranial space occupying lesions. We report a case of a 27 year-old female patient who presented with hemiparesis and seizures and was found to have a giant primary intracranial hydatid cyst arising in the right parietal lobe.

Introduction: Intravenous Tissue Plasminogen Activator (rTPA) is used to treat acute ischemic stroke (AIS). Early recanalization from this leads to better stroke outcomes, but the exact mechanism remains unknown. To clarify this, we correlated tissue perfusion and functional outcomes in acute stroke patients (AIS) who received rTPA to resting state default mode network (DMN) and task-positive (TPN) activities measured with fMRI. Following NIH stroke scale (NIHSS) assessments, patients underwent magnetic resonance imaging (MRI) scans during rTPA infusion (baseline), six hours post stroke and at 30d follow up visit.
Results: Paired t-tests revealed that NIHSS at 6 hrs post stroke and at 30-days follow up significantly better compared to baseline, indicating improved functional outcomes. Changes in NIHSS were associated by significant changes in resting connectivity in TPN and DMN. In the TPN, both the undamaged Frontal Eye Field (FEF) and the undamaged intraparietal sulcus (IPS) node at the 6 hrs time point improved in connectivity with other TPN nodes compared to baseline. 30 days follow up resting connectivity of the DMN on the medial-prefrontal (MPF) node, and undamaged lateral parietal (LP) node, along with the damaged medial-temporalnode of TPN showed more robust correlations from baseline to 30 days follow up. The damaged IPS connectivity was the only measure that significantly correlated with NIHSS at the 6 hrs time point. No correlations with NIHSS were found at baseline or the 30 days time points with resting state or perfusion data.
Conclusion: In this pilot study, we found that patients who received rTPA showed changes in resting state networks and functional outcomes over time. These findings point to an intriguing possibility, that the improvement of resting state networks may reflect improved efficiency of brain activity that is potentially related to functional outcomes in AIS patients who receive rTPA. As such, the improved functional connectivity measured with rsBOLD fMRI should be further explored as a potential predictive biomarker for rTPA response. Larger studies are needed to verify these findings.

Management of complications in case of rupture of the superior sagittal sinus (SSS), during parasagittal meningiomas surgery represents a critical issue for neurosurgeons. Various techniques have been proposed to repair the sinus since the first reports. Nowadays, SSS reparation is achieved with haemostatic tools as Gelfoam or Surgicel, ligature, and through mechanic compression with cottonoids. Our paper is the first report of reparation of the SSS with an autologous fibrin glue system (Vivostat®). We also describe our experience and the management of a common complication in the surgical treatment of lesions involving the sinus.

The treatment of meningiomas is still at the borderline of two or even three disciplines. Surgery, radiation and interventional neuroradiology. Even when it seems confusing, reviewing the literature allows us to draw a clear line between the therapeutic modalities. There are cases where surgery is the first line treatment and others where radiation should be first line and again others where pre-operative embolization, surgery and postsurgical radiation are the treatment options of choice. In the present short review we present four cases of different meningiomas treated surgically in our department and we review the literature to provide a flow chart, indicating which treatment modality would fit to different meningiomas. Of course the flow chart is based on the present literature and cannot be followed without restrictions and reservations. Randomized multicenter studies will help to clear this field more effectively.

Two rare cases of split cord malformation with neurenteric cyst are presented. The clinical, radiological and surgical findings are presented with brief review of literature. Co-existent neurenteric cysts along with SCM type 1 have been described very rarely in the literature.

Basal ganglia hemorrhage, one of the most devastating forms of cerebrovascular disease, is often as a result of poorly controlled long standing hypertension. Hypertension often leads to development of solitary intracerebral hematoma. The occurrence of multiple simultaneous intracerebral hemorrhages due to hypertension in same or different vascular territories is rare. We report the occurrence of simultaneous bilateral hypertensive putaminal hemorrhage appearing as mirror image (Owl eye appearance) in a 60 years old gentleman and discuss the probable pathophysiological mechanisms

Background: Orthostatic intolerance including dizziness and syncope is common and may reflect autonomic dysfunction. Cardiovascular reflex tests (deep breathing, Valsalva maneuver and tilt test) are established diagnostic methods for evaluation of orthostatic and autonomic symptoms. Small fiber neuropathy, a frequent underlying mechanism, is evaluated by the quantitative sudomotor axonal reflex test (QSART) and skin biopsies. The comprehensive quantitative scale to grade abnormalities in these tests is lacking.
Methods: This study defines the QASAT - Quantitative scale for grading of cardiovascular reflex tests using heart rate, blood pressure, n transcranial Doppler, QSART and small fibers (intrapidermal sensory and sweat gland) densities from skin biopsies. The QASAT has three main categories: cardiovascular, cerebral blood flow (includes cerebral autoregulation/vasoreactivity score) and small fiber neuropathy. QASAT was validated in 612 participants with diabetes mellitus (92), Parkinson’s disease (88), multiple system atrophy (23) and other diagnoses (409). The QASAT was compared with the Composite Autonomic Severity Score using ANOVA, correlations and sensitivity/specificity analysis.
Results: Scores of heart rate variability from deep breathing, orthostatic hypotension, orthostatic cerebral blood flow, sensory and sweat gland small fiber densities were disease specific (p<0.0001) and were correlated with the severity of autonomic failure (r=0.84, p<0.0001). Autonomic, sensory and cerebral blood flow abnormalities coexist and are correlated. Initial response to slow and fast tilt, baroreflex gain, QT interval, catecholamines and spectral analysis of the heart rate failed to correlate with severity of autonomic failure and therefore were not included in QASAT.
Conclusion: QASAT is an objective and validated instrument for grading of dysautonomia, associated small fiber neuropathy and cerebral blood flow. QASAT quantifies both below (bradycardia, supine/orthostatic hypotension) and above (tachycardia, supine/orthostatic hypertension) normal values. Cardiovascular tests are enhanced by scoring the cerebral hypoperfusion which underlies orthostatic symptoms. Inclusion of epidermal and sweat gland fiber density scoring improves lesion localization.

Vitiligo is a common acquired disorder that has an estimated incidence of 1-4% [1]. It is inherited either polygenic or autosomal dominant, with incomplete penetrance and variable expression. Approximately in half of the patients vitiligo developed before the age of 20 [2]. It is characterized by a fairly symmetric pattern of circumscribed macules and patches of amelanosis. Vitiligo has a considerable social and psychological effect on patients because of alteration of appearance [3,4]. On the other hand chronic urticaria not only affect the appearance but involves physical discomfort and others view skin lesions as stigmatizing [5,6].

Background: Arachnoid cysts are intra-arachnoid fluid collections covered by a thin membrane that may develop throughout the cerebrospinal axis. Although the precise causative mechanism is unknown, arachnoid cyst (AC) are now generally accepted to be developmental anomalies of arachnoid. These lesions have commonly been described in the literature; however the presence of familial arachnoid cysts is quite rare. Most genetically related AC have been documented in patients with a known genetic syndrome. The current case report describes a family with four members affected by an arachnoid cyst in the same region.
Methods: In addition to reviewing the current case, a literature search was conducted using National Library of Medicine and National Institutes of Health databases to identify articles pertaining to familial Arachnoid cysts. Overall, 32 published articles fit the established review criteria
Results: We describe a family whose members (father and three siblings) present an arachnoid cyst in the same region (the left middle cranial fossa). The general physical findings in the four members were normal and no clinical suggestion of a genetic syndrome. None of the members had an increased head size or abnormal cutaneous findings. Histories of prenatal and perinatal periods were unremarkable. All were born normally at term and none had any history of intrauterine exposure to infection, drugs, teratogens or trauma. Karyotyping failed to reveal abnormalities.
Conclusion: This report extends previous observations that AC can be familial and supports the hypothesis that some cases have a genetic aetiology. The lack of chromosomal or genetic studies in these patients supports the need for additional research into the mechanism of AC formation.

It is suspected that the pathophysiology of migraine has a relation to the autonomic dysfunction. We studied to assess the autonomic function in migraine using the spectral analysis of heart rate variability (HRV) by accelerated plethysmography (APG). Five-hundred-twenty-four patients received the health check-ups in an affiliated hospital since September, 2011 to April, 2013. Eighty-three patients with headache and 38 controls were enrolled in this study. The patients were subdivided into 19 with migraine (19 female), 26 with tension-type headache (TTH) (7 male, 19 female) and 31 of other headaches using a questionnaire based on the International Classification of Headache Disorders 2nd Edition. Patients with migraine or tension-type headache underwent examinations using APG with an orthostatic load. Twenty-four controls without headache received same examinations. As a result, the standing/sitting ratio of spectral power of high-frequency (HF) in the migraine group was higher than those of TTH (p=0.0277) and normal controls (p=0.0104). The cut-off value of 1.058 could separate migraine from TTH with sensitivity of 0.632 and specificity of 0.846. The results suggested that the parasympathetic activity accentuated by orthostatic load in patients with migraine, on the contrarily the parasympathetic activities diminished by orthostatic load in patients with TTH or normal controls. This study showed that spectral analysis of HRV by APG is a supportive tool to distinguish migraine from TTH.