Case Reports in Neurological Medicine The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . All rights reserved. Heading Disorientation after Right Posteromedial Infarction Wed, 07 Oct 2015 11:25:18 +0000 We report the case of heading disorientation following ischemic stroke involving the right posteromedial areas. The patient was administered a new test named the Card Placing Test during which a subject was required to recreate an array of three cards, each of which was randomly placed on eight grids around the subject, before and after the subject’s rotation. Qualitative analysis of his performance after rotation revealed that over half of the errors comprised transposition and rotational offset. His score on the Card Placing Test was compared with those of normal controls . The results showed that his score on Card Placing Test after rotation was significantly lower than those of controls, whereas there was no significant difference between the case and controls in profile of error types. We infer that the heading disorientation observed in the present case was a result of a derangement of a short-term buffer that integrated information on spatial locations of objects with changes in body directions. Ritsuo Hashimoto, Noriyo Komori, and Masako Abe Copyright © 2015 Ritsuo Hashimoto et al. All rights reserved. Acquired Hemophilia A with a Rare Presentation of Acute Subdural Hematoma Tue, 29 Sep 2015 06:43:50 +0000 An 80-year-old man was admitted for acute subdural hematoma caused by a mild brain injury. His coagulation test showed an isolated prolongation of activated partial thromboplastin time (aPTT). Though the subdural hematoma did not progress, oozing bleed from the wound of tracheostomy continued. Failure of correction on aPTT mixing test supported the presence of an inhibitor to a coagulation factor. Once the diagnosis of acquired hemophilia A (AHA) was made, steroid therapy was performed, which leads him to complete remission of AHA. Isolated prolongation of aPTT can be the key to diagnose a rare coagulopathy, such as AHA. Yoshihide Sehara, Yuka Hayashi, and Jun Mimuro Copyright © 2015 Yoshihide Sehara et al. All rights reserved. Extraintracranial Bypass as a Rescue Therapy for Symptomatic Flow Diverter Thrombosis Thu, 17 Sep 2015 08:09:20 +0000 A 56-year-old female with a giant partially thrombosed unruptured carotid-ophthalmic aneurysm was treated with a Pipeline flow diverter. Three months after the procedure, in concomitance with the discontinuation of one of the antiplatelet medications, the patient suffered from a minor stroke and relapsing transient ischemic attacks. The angiography demonstrated the occlusion of the internal carotid artery, and a perfusion-weighted CT scan showed a condition of hypoperfusion. The patient underwent a double-barrel extraintracranial bypass. The postoperative course was uneventful and she has experienced no further ischemic events to date. Luigi A. Lanterna, Alessandro Lunghi, Carlo Brembilla, Paolo Gritti, and Claudio Bernucci Copyright © 2015 Luigi A. Lanterna et al. All rights reserved. Systemic Sarcoidosis Mimicking a Behavioural Variant of Frontotemporal Dementia Wed, 09 Sep 2015 11:11:05 +0000 Among rare neurological manifestations, a progressive dementia may exceptionally be the revealing clinical feature of a sarcoidosis. Diagnosis may then be difficult, especially when systemic signs are missing or latent, with a risk of therapeutic delay. We report the first case of sarcoidosis mimicking a frontotemporal dementia. A 53-year-old man presented with a dementia clinically suggestive of frontotemporal dementia, progressing slowly for about 2 years. However, MRI revealed unusual aspects, mainly large areas of T2/FLAIR hypersignal within temporal regions and cerebellum, with nodular leptomeningeal and juxtacortical Gadolinium enhancement. The patient was finally diagnosed with a systemic sarcoidosis. We discuss the differential diagnosis based on MRI aspects and review the literature on the clinical, biological, and imaging features of sarcoidosis presenting with dementia. This case demonstrates that brain imaging remains mandatory in the exploration process of a patient with dementia. Although the patient presented with rather typical features of a behavioural variant of frontotemporal dementia, the MRI aspect was the key exploration that leaded to the diagnosis. Anne De Maindreville, Line Bedos, and Serge Bakchine Copyright © 2015 Anne De Maindreville et al. All rights reserved. A Case of Subacute Combined Degeneration of the Spinal Cord with Infective Endocarditis Mon, 07 Sep 2015 11:04:02 +0000 Background. Subacute combined degeneration (SCD) is a rare cause of demyelination of the dorsal and lateral columns of spinal cord and is a neurogenic complication due to cobalamin deficiency. Anemia of chronic disease (ACD) occurs in patients with acute or chronic immune activation, including infective endocarditis. It remains to be elucidated whether ACD patients are more sensitive to suffer from SCD. Little cases about SCD patients accompanied with ACD have been reported till now. Here we reported a 36-year-old man with SCD with a medical history of mitral inadequacy over 20 years, who was admitted and transported from another hospital to our hospital due to an 8-month history of gait disturbance, lower limb weakness and paresthesia, and loss of proprioception. Significant laboratory results and echocardiography suggest iron deficiency anemia and infective endocarditis (IE). The SCD diagnosis was confirmed by MRI, which showed selective demyelination in the dorsal and lateral columns of spinal cord. In conclusion, the ACD patients may suffer from SCD, which can be diagnosed by 3 Tesla magnetic resonance imaging. Xiao-Jiang Huang, Jia He, Wen-sheng Qu, and Dai-Shi Tian Copyright © 2015 Xiao-Jiang Huang et al. All rights reserved. STA-MCA Bypass as a “Bridge” to Pituitary Surgery in a Patient with an Adenoma Occluding the Internal Carotid Artery: Case Report and Review of the Literature Sun, 06 Sep 2015 11:52:19 +0000 Occlusion of the intracranial internal carotid artery (ICA) by a pituitary adenoma with resulting cerebral ischemia is a very rare but devastating occurrence. The authors present a case in which a condition of symptomatic ICA occlusion due to a giant pituitary adenoma was successfully treated using a preliminary extraintracranial bypass as a “bridge” to the tumor removal. A 52-year-old patient presented with a minor stroke followed by pressure-dependent transient ischemic attacks consistent with a condition of hypoperfusion. MR imaging and a digital subtraction angiography revealed a pituitary adenoma occluding the ICA on the right side. He underwent a superficial temporal artery to middle cerebral artery (STA-MCA) bypass with the aim of revascularizing the ischemic hemisphere and reducing the risk of perioperative stroke or stroke evolution. The patient was subsequently operated on to remove the adenoma through a transsphenoidal approach. The postoperative course was uneventful and the patient has suffered no further ischemic events. When there are no emergency indications to decompress the optical pathways but the patient is at risk of impending stroke because of ICA occlusion, a two-step strategy consisting of a bypass and subsequent removal of the pituitary adenoma may be a valuable option. Luigi A. Lanterna, Carlo Brembilla, Antonio Signorelli, Paolo Gritti, Emanuele Costi, Gianluigi Dorelli, and Claudio Bernucci Copyright © 2015 Luigi A. Lanterna et al. All rights reserved. Modulation of the Left Prefrontal Cortex with High Frequency Repetitive Transcranial Magnetic Stimulation Facilitates Gait in Multiple Sclerosis Wed, 02 Sep 2015 08:38:32 +0000 Multiple Sclerosis (MS) is a chronic central nervous system (CNS) demyelinating disease. Gait abnormalities are common and disabling in patients with MS with limited treatment options available. Emerging evidence suggests a role of prefrontal attention networks in modulating gait. High-frequency repetitive transcranial magnetic stimulation (rTMS) is known to enhance cortical excitability in stimulated cortex and its correlates. We investigated the effect of high-frequency left prefrontal rTMS on gait parameters in a 51-year-old Caucasian male with chronic relapsing/remitting MS with residual disabling attention and gait symptoms. Patient received 6 Hz, rTMS at 90% motor threshold using figure of eight coil centered on location (using 10-20 electroencephalography (EEG) lead localization system). GAITRite gait analysis system was used to collect objective gait measures before and after one session and in another occasion three consecutive daily sessions of rTMS. Two-tailed within subject repeated measure t-test showed significant enhancement in ambulation time, gait velocity, and cadence after three consecutive daily sessions of rTMS. Modulating left prefrontal cortex excitability using rTMS resulted in significant change in gait parameters after three sessions. To our knowledge, this is the first report that demonstrates the effect of rTMS applied to the prefrontal cortex on gait in MS patients. Amer M. Burhan, Priya Subramanian, Luljeta Pallaveshi, Brittany Barnes, and Manuel Montero-Odasso Copyright © 2015 Amer M. Burhan et al. All rights reserved. Bilateral Intracranial Vertebral Artery Stenosis Presenting as Recurrent Prolonged Presyncopal Episodes Tue, 01 Sep 2015 11:47:44 +0000 Amongst various mechanisms of presyncopal events, posterior circulation disease needs to be considered. This particular mechanism has been underrecognized. We describe a case of a 76-year-old patient with recurrent posterior circulation TIAs, presenting as recurrent prolonged presyncopal events. Banu Sundar, Majaz Moonis, Ajay Wakhloo, and Ajit Puri Copyright © 2015 Banu Sundar et al. All rights reserved. Atypical Miller Fisher Syndrome with Anisocoria and Rapidly Fluctuating Pupillary Diameter Mon, 24 Aug 2015 11:25:26 +0000 Miller Fisher syndrome is a variant of Guillain-Barre syndrome characterized by the classic triad of ophthalmoplegia, ataxia, and areflexia. Pupillary involvement is common in MFS and has been reported in 35–42% of MFS patients. Although case reports have discussed isolated ophthalmoplegia as a presentation of MFS, anisocoria and rapid fluctuation of pupillary diameter have not been reported in anti-GQ1b antibody positive individuals. Here we describe an individual who presented with diplopia and was found to have progressive internal and external ophthalmoplegia with frequent fluctuations in pupillary diameter and anisocoria. These exam findings are not commonly described even in atypical presentations of MFS. The onset of symptoms was preceded by an upper respiratory infection but no gastrointestinal symptoms. Imaging and CSF studies were unremarkable; however serum levels of immunoglobulin G anti-GQ1b antibody and anti-GAD antibody were elevated confirming the diagnosis of MFS. The patient was treated with IVIG and intravenous steroids with mild resolution of external ophthalmoplegia. He did not go on to develop more typical features of MFS such as ataxia or areflexia. This demonstrates that isolated external and internal ophthalmoparesis with rapidly fluctuating pupillary diameter and associated anisocoria can be the sole manifestation of atypical MFS. Garima Gupta and Antonio Liu Copyright © 2015 Garima Gupta and Antonio Liu. All rights reserved. Gabapentin-Induced Urinary Incontinence: A Rare Side Effect in Patients with Neuropathic Pain Sun, 16 Aug 2015 14:04:40 +0000 Gabapentin is a first-line agent for neuropathic pain management and has a favorable safety profile. The literature includes a few cases of gabapentin-induced incontinence, and most of them involved patients with epilepsy who were between the ages of 12 and 43 years. Herein, we present three patients with neuropathic pain due to different diagnoses, and, to our knowledge, these are the oldest reported cases of urinary incontinence caused by gabapentin therapy. A 56-year-old female patient who underwent hip arthroplasty developed a sciatic nerve injury and neuropathic pain postoperatively. Ten days after she began taking gabapentin to relieve her pain, she experienced daily urinary incontinence. In another instance, a 63-year-old female patient was diagnosed with complex regional pain syndrome, and seven days after the initiation of gabapentin therapy, urinary incontinence developed. In addition, a 66-year-old male patient with neuropathic pain due to cervical disc pathology complained of urinary incontinence after the onset of gabapentin therapy. After discontinuing this drug, the incontinence symptoms resolved in these patients on the seventh, the first, and the second days, respectively. Physicians who administer gabapentin should inform their patients about the potential risk of gabapentin-induced incontinence and its negative impact on quality of life. Sibel Kibar, Sibel Demir, Nebahat Sezer, Belma Füsun Köseoğlu, Meltem Dalyan Aras, and Bilge Kesikburun Copyright © 2015 Sibel Kibar et al. All rights reserved. Cerebral Amyloid Angiopathy-Related Inflammation: Report of a Case with Very Difficult Therapeutic Management Sun, 16 Aug 2015 08:49:30 +0000 Background. Cerebral amyloid angiopathy-related inflammation (CAA-ri) results from autoimmune response to beta-amyloid deposits in cerebral vessels. Its clinical course and complications have seldom been described in literature. Case Report. In a patient presenting with delirium and left hemiparesis the diagnosis of CAA-ri was supported by the finding of elevated anti-amyloid autoantibodies in the cerebrospinal fluid (CSF). Steroid therapy produced significant improvements in clinical and investigational assessments, but after two months, it caused Acute Respiratory Distress Syndrome. After steroid therapy discontinuation the patient presented a rapidly progressive dementia, Guillain-Barré syndrome, new cerebral ischemic lesions, and thrombosis of the right cephalic and subclavian veins that were treated with subcutaneous heparin. After a week the patient died because of brain hemorrhage. Conclusion. This case suggests caution in steroid therapy discontinuation and antithrombotic therapy administration in patients with CAA-ri. The CSF search of anti-amyloid autoantibodies could be helpful to support the diagnosis. Francesca Crosta, Berardino Orlandi, Federica De Santis, Gianni Passalacqua, Jacopo C. DiFrancesco, Fabrizio Piazza, Alessia Catalucci, Giovambattista Desideri, and Carmine Marini Copyright © 2015 Francesca Crosta et al. All rights reserved. Isolated Hyperreligiosity in a Patient with Temporal Lobe Epilepsy Thu, 13 Aug 2015 13:33:42 +0000 A 40-year-old man with history of temporal lobe epilepsy presented to the emergency department with hyperreligiosity after medication noncompliance. After medications were resumed, he returned to baseline. Many famous prophets are believed to have suffered epilepsy. Waxman and Geschwind described a group of traits in patients with temporal lobe epilepsy consisting of hyperreligiosity, hypergraphia, altered sexual behavior, aggressiveness, preoccupation with details, and circumstantiality. The incidence of religious experiences ranges from 0.3 to 3.1 percent in patients with epilepsy. Religious experiences can be ictal, interictal, or postictal. Treatment is aimed at the underlying seizure etiology. Rocio Garcia-Santibanez and Harini Sarva Copyright © 2015 Rocio Garcia-Santibanez and Harini Sarva. All rights reserved. Diagnostic Dilemma in a Young Woman with Acute Headache: Delayed Diagnosis of Third Ventricular Colloid Cyst with Hydrocephalus Sun, 09 Aug 2015 11:41:59 +0000 Objectives. To highlight the importance of early diagnosis of colloid cyst of the third ventricle and its early management. Clinical Presentation and Intervention. This is a young lady who presented with sudden onset headache. She attended a local clinic and also her area hospital. Her diagnosis was delayed several hours due to a diagnostic dilemma initially. No surgical intervention was tried since the patient developed early signs of brainstem coning by the time she was seen by neurosurgeon. Patient died after few days in spite of intensive ICU measures. Conclusion. Sudden onset headache in young adults should be looked at carefully. Early imaging is mandatory to prevent mortality. Jasem Y. Al-Hashel, Azza A. H. Rady, Doaa Y. Soliman, and Periasamy Vembu Copyright © 2015 Jasem Y. Al-Hashel et al. All rights reserved. Lhermitte Sign as a Presenting Symptom of Thoracic Spinal Pathology: A Case Study Mon, 03 Aug 2015 06:11:10 +0000 A 54-year-old male with ankylosing spondylitis presented with complaints of progressively worsening bilateral leg weakness and difficulty ambulating of 2-week duration. He also felt a sharp, electric, shock-like sensation radiating from his lower back into his legs upon flexing the trunk. There was no history of trauma or other inciting events within the 2 weeks prior to presentation. Thoracic MRI at this visit showed a three-column fracture at T11-T12. He underwent spinal fusion surgery and within 2 days after surgery the radiating electrical sensation with spinal flexion had completely resolved. Adam Hills and Mazen Al-Hakim Copyright © 2015 Adam Hills and Mazen Al-Hakim. All rights reserved. Endoscopic Transnasal Approach for Cholesterol Granuloma of the Petrous Apex Tue, 21 Jul 2015 09:56:23 +0000 Cholesterol granulomas are rare round or ovoid cysts. They contain cholesterol crystals surrounded by foreign bodies of giant cells and are characterized by chronic inflammation. Large cholesterol granuloma can compress surrounding tissue especially cranial nerves. There are several types of surgery for the resection of cholesterol granuloma. We describe 4 cases of cholesterol granuloma operated on via transnasal endoscopic approach. In this report, we describe radiologic and pathologic features of this lesion and explain the advantages and disadvantages of transsphenoidal endoscopic approach for these rare lesions. Mohammad Samadian, Nader Akbari Dilmaghani, Navid Ahmady Roozbahany, Navid Farzin, and Mohammad Bahadoram Copyright © 2015 Mohammad Samadian et al. All rights reserved. Transient Global Amnesia as the First Clinical Symptom for Malignant B-Cell Lymphoma with Central Nervous System Involvement Tue, 07 Jul 2015 05:50:45 +0000 We present the case of an elderly male who was diagnosed with transient global amnesia (TGA), only to be diagnosed with B-cell lymphoma with central nervous system involvement a few weeks later. This is the first ever case reported in literature with lymphoma presenting as TGA. Literature review and pertinent points regarding high-yield imaging protocol for presumed TGA patients are discussed. Atif Zafar, Ghulam Ishaq Khan, Sahrish Abdin, and Muhammad Taimoor Khan Copyright © 2015 Atif Zafar et al. All rights reserved. Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Polyneuropathy Mimicking Guillain-Barre Syndrome Tue, 23 Jun 2015 13:04:35 +0000 Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) which commonly affects the peripheral nervous system. A 38-year-old female with a history of asthma presented with a 2-week history of bilateral lower extremity paresthesias that progressed to symmetric ascending paralysis. Nerve conduction studies could not rule out Guillain-Barre syndrome (GBS) and plasmapheresis was considered. Her blood work revealed marked eosinophilia (>50%), she had purpuric lesions in her legs, and a head magnetic resonance image showed evidence of pansinusitis. Coupled with a history of asthma we suspected EGPA-associated neuropathy and started steroid treatment. The patient showed rapid and significant improvement. ANCAs were later reported positive. ANCA-associated vasculitides present most often as mononeuritis multiplex, but they can mimic GBS and should always be considered in the differential diagnosis, since the treatment strategies for these conditions are radically different. Carlos R. Camara-Lemarroy, Adrian Infante-Valenzuela, Hector J. Villareal-Montemayor, Carlos A. Soto-Rincon, Javier A. Davila-Olalde, and Hector J. Villareal-Velazquez Copyright © 2015 Carlos R. Camara-Lemarroy et al. All rights reserved. Propranolol for Paroxysmal Sympathetic Hyperactivity with Lateralizing Hyperhidrosis after Stroke Thu, 18 Jun 2015 11:26:40 +0000 Brain injury can lead to impaired cortical inhibition of the hypothalamus, resulting in increased sympathetic nervous system activation. Symptoms of paroxysmal sympathetic hyperactivity may include hyperthermia, tachycardia, tachypnea, vasodilation, and hyperhidrosis. We report the case of a 41-year-old man who suffered from a left middle cerebral artery stroke and subsequently developed central fever, contralateral temperature change, and hyperhidrosis. His symptoms abated with low-dose propranolol and then returned upon discontinuation. Restarting propranolol again stopped his symptoms. This represents the first report of propranolol being used for unilateral dysautonomia after stroke. Propranolol is a lipophilic nonselective beta-blocker which easily crosses the blood-brain barrier and may be used to treat paroxysmal sympathetic hyperactivity. Jason W. Siefferman and George Lai Copyright © 2015 Jason W. Siefferman and George Lai. All rights reserved. Carotid Endarterectomy in a Patient with Posterior Cerebral Artery Infarction: Influence of Fetal Type PCA on Atypical Clinical Course Wed, 10 Jun 2015 12:23:32 +0000 Fetal type PCA may positively affect clinical outcome in patients with ischemic stroke such as protection of a reserve blood flow capacity as well as negative effect on clinical outcome such as certain atypical pathological manifestations. We presented a patient with a posterior cerebral artery territory infarction due to an unexpected etiology as stenosis of internal carotid artery (ICA) with existence of fetal type PCA. Mehmet Kolukısa, Azize Esra Gürsoy, Gülşen Kocaman, Hümeyra Dürüyen, Hüseyin Toprak, and Talip Asil Copyright © 2015 Mehmet Kolukısa et al. All rights reserved. A Case of Acute Disseminated Encephalomyelitis in a Middle-Aged Adult Tue, 09 Jun 2015 16:29:10 +0000 Objectives. Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder that is often preceded by infection or recent vaccination. Encephalopathy and focal neurological deficits are usually manifest several weeks after a prodromal illness with rapidly progressive neurologic decline. ADEM is most commonly seen in children and young adults, in which prognosis is favorable, but very few cases have been reported of older adults with ADEM and thus their clinical course is unknown. Methods. Here we present a case of ADEM in a middle-aged adult that recovered well after treatment. Results. A 62-year-old man presented with encephalopathy and rapid neurological decline following a gastrointestinal illness. A brain MRI revealed extensive supratentorial white matter hyperintensities consistent with ADEM and thus he was started on high dose intravenous methylprednisolone. He underwent a brain biopsy showing widespread white matter inflammation secondary to demyelination. At discharge, his neurological exam had significantly improved with continued steroid treatment and four months later, he was able to perform his ADLs. Conclusions. This case of ADEM in a middle-aged adult represents an excellent response to high dose steroid treatment with a remarkable neurological recovery. Thus it behooves one to treat suspected cases of ADEM in an adult patient aggressively, as outcome can be favorable. Nicole Mahdi, Peter A. Abdelmalik, Mark Curtis, and Barak Bar Copyright © 2015 Nicole Mahdi et al. All rights reserved. Is It a Pathogenic ATP7A Variation and Is It Menkes Disease? Sun, 07 Jun 2015 08:04:23 +0000 Zeynep Tümer Copyright © 2015 Zeynep Tümer. All rights reserved. Progressive Intracranial Vertebral Artery Dissection Presenting with Isolated Trigeminal Neuralgia-Like Facial Pain Wed, 03 Jun 2015 11:13:44 +0000 Intracranial vertebral artery dissection (IVAD) is a potentially life-threatening disease, which usually presents with ischemic stroke or subarachnoid hemorrhage. IVAD presenting with isolated facial pain is rare, and no case with isolated trigeminal neuralgia- (TN-) like facial pain has been reported. Here, we report the case of a 57-year-old male with IVAD who presented with acute isolated TN-like facial pain that extended from his left cheek to his left forehead and auricle. He felt a brief stabbing pain when his face was touched in the territory of the first and second divisions of the left trigeminal nerve. There were no other neurological signs. Magnetic resonance imaging (MRI) of the brain 7 days after onset revealed dissection of the left intracranial vertebral artery without brain infarction. The pain gradually disappeared in approximately 6 weeks, and the patient remained asymptomatic thereafter, except for a brief episode of vertigo. Follow-up MRI revealed progressive narrowing of the artery without brain infarction. This case indicates that IVAD can present with isolated facial pain that mimics TN. IVAD should be considered in the differential diagnosis of acute facial pain or TN. Tomoki Nakamizo, Takashi Koide, and Hiromichi Miyazaki Copyright © 2015 Tomoki Nakamizo et al. All rights reserved. Posterior Reversible Encephalopathy Syndrome Secondary to CSF Leak and Intracranial Hypotension: A Case Report and Literature Review Wed, 27 May 2015 13:08:42 +0000 Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical neuroradiological condition characterized by insidious onset of neurological symptoms associated with radiological findings indicating posterior leukoencephalopathy. PRES secondary to cerebrospinal fluid (CSF) leak leading to intracranial hypotension is not well recognized etiology of this condition. Herein, we report a case of PRES that occurred in the setting of CSF leak due to inadvertent dural puncture. Patient underwent suturing of the dural defect. Subsequently, his symptoms resolved and a repeated brain MRI showed resolution of brain lesions. The pathophysiology and mechanistic model for developing PRES in the setting of intracranial hypotension were discussed. We further highlighted the importance of tight blood pressure control in patients with CSF leak and suspected intracranial hypotension because they are more vulnerable to develop PRES with normal or slightly elevated bleed pressure values. Tariq Hammad, Alison DeDent, Rami Algahtani, Yaseen Alastal, Lawrence Elmer, Azedine Medhkour, Fadi Safi, and Ragheb Assaly Copyright © 2015 Tariq Hammad et al. All rights reserved. Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat Wed, 27 May 2015 11:20:40 +0000 Background. Stiff person syndrome (SPS) is a rare neurological disorder characterized by progressive rigidity of axial and limb muscles associated with painful spasms. SPS can be classified into classic SPS, paraneoplastic SPS, and SPS variants. Its underlying pathogenesis is probably autoimmune, as in most cases antibodies against glutamic acid decarboxylase (GAD) are observed. Similarly, paraneoplastic SPS is usually linked to anti-amphiphysin antibodies. Treatment is based on drugs enhancing gamma-aminobutyric acid (GABA) transmission and immunomodulatory agents. Case Series. Patient 1 is a 45-year-old male affected by the classic SPS, Patient 2 is a 73-year-old male affected by paraneoplastic SPS, and Patient 3 is a 68-year-old male affected by the stiff limb syndrome, a SPS variant where symptoms are confined to the limbs. Symptoms, diagnostic findings, and clinical course were extremely variable in the three patients, and treatment was often unsatisfactory and not well tolerated, thus reducing patient compliance. Clinical manifestations also included some unusual features such as recurrent vomiting and progressive dysarthria. Conclusions. SPS is a rare disorder that causes significant disability. Because of its extensive clinical variability, a multitask and personalized treatment is indicated. A clearer understanding of uncommon clinical features and better-tolerated therapeutic strategies are still needed. Susanne Buechner, Igor Florio, and Loredana Capone Copyright © 2015 Susanne Buechner et al. All rights reserved. Posterior Reversible Encephalopathy Syndrome due to High Dose Corticosteroids for an MS Relapse Tue, 26 May 2015 06:29:40 +0000 Increased blood pressure is a known adverse effect associated with corticosteroids but little is published regarding the risk with the high doses used in multiple sclerosis (MS). A 53-year-old female with known relapsing remitting MS presented with a new brainstem relapse. Standard of care treatment for an acute MS relapse, 1250 mg of oral prednisone for 5 days, was initiated. She developed an occipital headache and dizziness and felt generally unwell. These symptoms persisted after treatment was complete. On presentation to medical attention, her blood pressure was 199/110 mmHg, although she had no history of hypertension. MRI changes were consistent with posterior reversible encephalopathy syndrome (PRES), demonstrating abnormal T2 signal in both thalami, the posterior occipital and posterior parietal white matter with mild sulcal effacement. As her pressure normalized with medication, her symptoms resolved and the MRI changes improved. No secondary cause of hypertension was found. This is the first reported case of PRES secondary to high dose corticosteroid use for an MS relapse without a history of hypertension and with no other secondary cause of hypertension identified. This rare complication should be considered in MS patients presenting with a headache or other neurological symptoms during treatment for a relapse. Sarah A. Morrow, Robina Rana, Donald Lee, Terri Paul, and Jeffrey L. Mahon Copyright © 2015 Sarah A. Morrow et al. All rights reserved. The Quantitative Measurement of Reversible Acute Depression after Subthalamic Deep Brain Stimulation in a Patient with Parkinson Disease Mon, 18 May 2015 13:55:48 +0000 Background. Depression is the most commonly reported mood symptom affecting 2–8% of patients after deep brain stimulation (DBS). Usually, symptoms develop gradually; however there have been cases of reproducible events that the mood symptoms were elicited within seconds to minutes after stimulation and were immediately reversible upon cessation of the stimulus. In the current study, we applied a self-reported questionnaire to assess the patient’s mood state. Objective. To objectively measure the reversible acute depression induced by DBS in a patient with Parkinson disease (PD). Methods. A statistically validated Spanish version of the Beck Depression Inventory Short Form (BDI-SF) was used. The questionnaire was administered three times. Results. The patient became acutely depressed within ninety seconds of monopolar stimulation on the right side. His symptoms resolved immediately after changing the setting to bipolar stimulation. The BDI-SF scores during stimulation off, on, and off again were 15, 19, and 6, respectively. Conclusions. The BDI-SF scores increased during stimulation and decreased after cessation. This is consistent with a reversible depressive state. The poststimulation BDI-SF score decreased to less than half of the baseline score. This may suggest that the depression was more severe than the patient was able to express during the stimulation. Daniel B. Simmons and Khashayar Dashtipour Copyright © 2015 Daniel B. Simmons and Khashayar Dashtipour. All rights reserved. Lipoma in the Corpus Callosum Presenting with Epileptic Seizures Associated with Expanding Perifocal Edema: A Case Report and Literature Review Mon, 11 May 2015 11:49:13 +0000 This report describes a rare case of a patient with lipoma presenting with epileptic seizures associated with expanding perifocal edema. The patient was a 48-year-old man who presented with loss of consciousness and convulsions. Magnetic resonance imaging (MRI) revealed a calcified mass in the corpus callosum with perifocal edema causing mass effect. An interhemispheric approach was used to biopsy the mass lesion. Histological examination revealed typical adipose cells, along with hamartomatous components. These components contained neurofilament and S-100-positive structures showing marked calcification. Fibrous cells immunoreactive for -smooth muscle actin and epithelial membrane antigen proliferated with focal granulomatous inflammatory changes. MIB-1 index was approximately 5% in immature cells observed in granulomatous areas. We thus suspected a coexisting neoplastic component. The residual lesion persisted in a dormant state for 2 years following biopsy. Surgical resection of a lipoma is extremely difficult and potentially dangerous. However, in the present case, the lesion was accompanied by atypical, expanding, and perifocal edema. Surgical treatment was inevitable for the purpose of histological confirmation, considering differential diagnoses such as dermoid, epidermoid, and glioma. In the end, anticonvulsant therapy proved effective for controlling epileptic seizures. Michiyasu Fuga, Toshihide Tanaka, Yohei Yamamoto, Yuzuru Hasegawa, Yuichi Murayama, and Junko Takahashi-Fujigasaki Copyright © 2015 Michiyasu Fuga et al. All rights reserved. Cerebellum as Initial Site of Distant Metastasis from Papillary Carcinoma of Thyroid: Review of Three Cases Wed, 06 May 2015 08:45:19 +0000 Background. The cerebellum as initial site of distant metastasis from differentiated thyroid carcinoma (DTC) including papillary (PTC) and follicular thyroid carcinoma (FTC) is rare manifestation. Case Presentations. Herein, we present three cases of cerebellar metastasis (CBM) of PTC. Mean age of patients was 67 years (range: 64–72), and mean duration between initial diagnosis and CBM was 49.6 months (range: 37–61). Frequent location was left cerebellar hemisphere and was associated with hydrocephalus. All patients underwent suboccipital craniectomy, and in two patients postoperative intensity modulated radiation therapy (IMRT) was given to deliver 5000 cGy in 25 fractions to residual lesions. Patient without postoperative IMRT had cerebellar recurrence along with lung and bone metastasis after 38 months. However, two patients were found alive and free of disease at the time of last follow-up. Conclusion. CBM from PTC is a rare clinical entity and is often associated with hydrocephalus. Histopathological diagnosis is important to initiate effective treatment, which relies on multidisciplinary approach to prolong the disease-free and overall survival rates. Mutahir A. Tunio, Mushabbab Al Asiri, Khalid Hussain AL-Qahtani, and Wafa AlShakweer Copyright © 2015 Mutahir A. Tunio et al. All rights reserved. ESBL Escherichia coli Ventriculitis after Aneurysm Clipping: A Rare and Difficult Therapeutic Challenge Wed, 29 Apr 2015 11:40:15 +0000 Background. Extended spectrum beta-lactamase (ESBL) produced Escherichia coli (E. coli) ventriculitis is a rare infection of the central nervous system, with increasing rarity in the adult population. The therapeutic strategy to achieve cure may need to involve a combination of intraventricular and intravenous (IV) therapy. Objective. To describe a case of ESBL E. coli meningitis/ventriculitis in an adult and outline the antimicrobial therapy that leads to cure. Methods. We retrospectively reviewed the records of a patient admitted to the neurosurgical department for aneurysmal subarachnoid hemorrhage, who developed ESBL E. coli ventriculitis. Results. A 55-year-old female, admitted for a Fisher grade 3, World Federation of Neurological Surgeons grade 1, subarachnoid hemorrhage, developed ESBL E. coli ventriculitis requiring a combination of intraventricular gentamicin and high dose intravenous meropenem for clearance. Cerebrospinal fluid clearance occurred at 7 days after initiation of combined therapy. The patient remained shunt dependent. Conclusions. Meningitis and ventriculitis caused by ESBL E. coli species are rare and pose significant challenges to the treating physician. Early consideration for combined intraventricular and IV therapy should be made. F. A. Zeiler and J. Silvaggio Copyright © 2015 F. A. Zeiler and J. Silvaggio. All rights reserved. Ruptured Arteriovenous Malformation Presenting with Kernohan’s Notch Mon, 27 Apr 2015 14:00:41 +0000 AVMs are congenital lesions that predispose patients to intracranial hemorrhage and resultant neurological deficits. These deficits are often focal and due to the presence of local neurologic disruption from hemorrhage in the contralateral cerebral hemisphere. We present a rare case of a patient with ipsilateral neurological deficits due to Kernohan’s Notch phenomenon resulting from hemorrhage from an AVM. A 31-year-old woman with seizures underwent MR and angiographic imaging which confirmed an unruptured left parietal AVM. The patient declined treatment and presented with obtundation 4 years later. Imaging revealed an acute left parietal ICH and SDH with significant mass effect. The patient underwent emergent hemicraniectomy and hematoma evacuation. Postoperatively, she made significant improvement and was following commands contralaterally with ipsilateral hemiplegia. MR imaging revealed right Kernohan’s Notch. The patient had significant rehabilitation with neurological improvement. She eventually underwent elective embolization followed by subsequent surgical resection and bone replacement. Three years from the initial hemorrhage, the patient had only mild left-sided weakness and ambulates without assistance. A false localizing sign, Kernohan’s Notch phenomenon, should be considered in the setting of AVM hemorrhage with paradoxical motor impairment and can be identified through MRI. Christopher F. Dibble, Michael P. Wemhoff, Tarik Ibrahim, Deanna Sasaki-Adams, Sten Solander, and Anand V. Germanwala Copyright © 2015 Christopher F. Dibble et al. All rights reserved.