Case Reports in Neurological Medicine http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Miller-Fisher Syndrome: Are Anti-GAD Antibodies Implicated in Its Pathophysiology? Sat, 30 Apr 2016 13:37:51 +0000 http://www.hindawi.com/journals/crinm/2016/3431849/ Miller-Fisher syndrome (MFS) is considered as a variant of the Guillain-Barre syndrome (GBS) and its characteristic clinical features are ophthalmoplegia, ataxia, and areflexia. Typically, it is associated with anti-GQ1b antibodies; however, a significant percentage (>10%) of these patients are seronegative. Here, we report a 67-year-old female patient who presented with the typical clinical features of MFS. Workup revealed antibodies against glutamic acid decarboxylase (GAD) in relatively high titers while GQ1b antibodies were negative. Neurological improvement was observed after intravenous gamma globulin and follow-up examinations showed a continuous clinical amelioration with simultaneous decline of anti-GAD levels which finally returned to normal values. This case indicates that anti-GAD antibodies may be associated with a broader clinical spectrum and future studies in GQ1b-seronegative patients could determine ultimately their clinical and pathogenetic significance in this syndrome. Ioannis E. Dagklis, Sotirios Papagiannopoulos, Varvara Theodoridou, Dimitrios Kazis, Ourania Argyropoulou, and Sevasti Bostantjopoulou Copyright © 2016 Ioannis E. Dagklis et al. All rights reserved. Neurological Complications of Middle East Respiratory Syndrome Coronavirus: A Report of Two Cases and Review of the Literature Thu, 28 Apr 2016 06:20:12 +0000 http://www.hindawi.com/journals/crinm/2016/3502683/ Middle East Respiratory Syndrome Coronavirus (MERS-CoV) was first discovered in September 2012 in Saudi Arabia. Since then, it caused more than 1600 laboratory-confirmed cases and more than 580 deaths among them. The clinical course of the disease ranges from asymptomatic infection to severe lower respiratory tract illness with multiorgan involvement and death. The disease can cause pulmonary, renal, hematological, and gastrointestinal complications. In this paper, we report neurological complications of MERS-CoV in two adult patients, and we hypothesize the pathophysiology. The first patient had an intracerebral hemorrhage as a result of thrombocytopenia, disseminated intravascular coagulation, and platelet dysfunction. The second case was a case of critical illness polyneuropathy complicating a long ICU stay. In these cases, the neurological complications were secondary to systemic complications and long ICU stay. Autopsy studies are needed to further understand the pathological mechanism. Hussein Algahtani, Ahmad Subahi, and Bader Shirah Copyright © 2016 Hussein Algahtani et al. All rights reserved. A Rare Case of Spontaneous Pneumocephalus Associated with Nontraumatic Cerebrospinal Fluid Leak Wed, 27 Apr 2016 14:25:00 +0000 http://www.hindawi.com/journals/crinm/2016/1828461/ Introduction. Spontaneous nontraumatic pneumocephalus (PNC) and cerebrospinal fluid (CSF) leaks are both very uncommon conditions. We report a rare case of spontaneous pneumocephalus associated with CSF leak secondary to right sphenoid sinus bony defect without history of trauma. Case Description. 51-year-old Hispanic female with past medical history of hypertension and idiopathic intracranial hypertension (Pseudotumor Cerebri) presented to the emergency room complaining of headache and clear discharge from the right nostril. Physical examination was significant for right frontal sinus tenderness and clear discharge from right nostril. Computed Tomography (CT) scan of the brain showed moderate amount of extra-axial air within the right cerebral hemisphere indicative of pneumocephalus. CT scan of facial bones showed bony defect along the right sphenoid sinus with abnormal CSF collection. The patient was started on intravenous antibiotics for meningitis prophylaxis and subsequently underwent transsphenoidal repair of cerebrospinal fluid leak with abdominal fat graft. CSF rhinorrhea stopped completely after the surgery with near complete resolution of pneumocephalus before discharge. Conclusions. Early identification of pneumocephalus and surgical intervention can help decrease the morbidity and avoid possible complications. Idiopathic intracranial hypertension, although rare, can lead to CSF leak and pneumocepahlus. Murad Baba, Omer Tarar, and Amer Syed Copyright © 2016 Murad Baba et al. All rights reserved. An Unusual Case of Asystole Occurring during Deep Brain Stimulation Surgery Wed, 27 Apr 2016 06:37:47 +0000 http://www.hindawi.com/journals/crinm/2016/8930296/ Background. Symptomatic bradycardia and hypotension in neurosurgery can produce severe consequences if not managed appropriately. The literature is scarce regarding its occurrence during deep brain stimulation (DBS) surgery. Case Presentation. A 67-year-old female presented for left DBS lead placement for essential tremors. During lead implantation, heart rate and blood pressure dropped rapidly; the patient became unresponsive and asystolic. Chest compressions were initiated and epinephrine was given. Within 30 seconds, the patient became hemodynamically stable and conscious. A head CT demonstrated no acute findings. After deliberation, a decision was made to complete the procedure. Assuming the etiology of the episode was the Bezold-Jarisch reflex (BJR), appropriate accommodations were made. The procedure was completed uneventfully. Conclusion. The episode was consistent with a manifestation of the BJR. The patient had a history of neurocardiogenic syncope and a relatively low-volume state, factors prone to the BJR. Overall, lead implantation can still occur safely if preventive measures are employed. Ha Son Nguyen, Harvey Woehlck, and Peter Pahapill Copyright © 2016 Ha Son Nguyen et al. All rights reserved. Endoport-Assisted Microsurgical Treatment of a Ruptured Periventricular Aneurysm Tue, 19 Apr 2016 10:56:02 +0000 http://www.hindawi.com/journals/crinm/2016/8654262/ Background and Importance. Ruptured periventricular aneurysms in patients with moyamoya disease represent challenging pathologies. The most common methods of treatment include endovascular embolization and microsurgical clipping. However, rare cases arise in which the location and anatomy of the aneurysm make these treatment modalities particularly challenging. Clinical Presentation. We report a case of a 34-year-old female with moyamoya disease who presented with intraventricular hemorrhage. CT angiography and digital subtraction angiography revealed an aneurysm located in the wall of the atrium of the right lateral ventricle. Distal endovascular access was not possible, and embolization risked the sacrifice of arteries supplying critical brain parenchyma. Using the BrainPath endoport system, the aneurysm was able to be accessed. Since the fusiform architecture of the aneurysm prevented clip placement, the aneurysm was ligated with electrocautery. Conclusion. We demonstrate the feasibility of endoport-assisted approach for minimally invasive access and treatment of uncommon, distally located aneurysms. Ching-Jen Chen, James Caruso, Robert M. Starke, Dale Ding, Thomas Buell, R. Webster Crowley, and Kenneth C. Liu Copyright © 2016 Ching-Jen Chen et al. All rights reserved. Profound Autonomic Instability Complicated by Multiple Episodes of Cardiac Asystole and Refractory Bradycardia in a Patient with Anti-NMDA Encephalitis Sun, 17 Apr 2016 10:22:40 +0000 http://www.hindawi.com/journals/crinm/2016/7967526/ Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) is autoimmune encephalitis primarily affecting young adults and children. First described about a decade ago, it frequently manifests as a syndrome that includes progressive behavioral changes, psychosis, central hypoventilation, seizures, and autonomic instability. Although cardiac arrhythmias often accompany anti-NMDARE, the need for long-term electrophysiological support is rare. We describe the case of NMDARE whose ICU course was complicated by progressively worsening episodes of tachyarrhythmia-bradyarrhythmia and episodes of asystole from which she was successfully resuscitated. Her life-threatening episodes of autonomic instability were successfully controlled only after the placement of a permanent pacemaker during her ICU stay. She made a clinical recovery and was discharged to a skilled nursing facility after a protracted hospital course. Stephanie R. Mehr, Roy C. Neeley, Melissa Wiley, and Avinash B. Kumar Copyright © 2016 Stephanie R. Mehr et al. All rights reserved. S3 Dorsal Root Ganglion/Nerve Root Stimulation for Refractory Postsurgical Perineal Pain: Technical Aspects of Anchorless Sacral Transforaminal Lead Placement Wed, 30 Mar 2016 13:24:41 +0000 http://www.hindawi.com/journals/crinm/2016/8926578/ Chronic perineal pain limits patients in physical and sexual activities, leading to social and psychological distress. In most cases, this pain develops after surgery in the urogenital area or as a consequence of trauma. Neuromodulation is one of the options in chronic postsurgical perineal pain treatment. We present a case of refractory perineal pain after right sided surgical resection of a Bartholin’s cyst which was treated with third sacral nerve root/dorsal root ganglion stimulation using the transforaminal approach. We describe a new anchorless lead placement technique using a unique curved lead delivery sheath. We postulate that this new posterior foraminal technique of lead placement is simple, safe, and reversible and may lower the occurrence of lead related complications. X. Zuidema, J. Breel, and F. Wille Copyright © 2016 X. Zuidema et al. All rights reserved. Unusual Spread of Renal Cell Carcinoma to the Clivus with Cranial Nerve Deficit Tue, 15 Mar 2016 09:53:58 +0000 http://www.hindawi.com/journals/crinm/2016/9184501/ Renal cell carcinoma (RCC) has unusual presentation affecting elderly males with a smoking history. The incidence of RCC varies while the incidence of spread of RCC to the clivus is rare. The typicality of RCC presentation includes hematuria, flank pain, and a palpable flank mass; however, RCC can also present with clival metastasis. The unique path of the abducens nerve in the clivus makes it susceptible to damage in metastasis. We report a case of a 54-year-old African American female that was evaluated for back pain, weakness, numbness, and tingling of bilateral lower extremities and subsequently disconjugate gaze and diplopia. Brain MRI confirmed metastasis to the clivus. She was started on radiotherapy and was planned for chemotherapy and transfer to a nursing home. When a patient presents with sudden unusual cranial nerve pathology, the possibility of metastatic RCC should be sought. Jerome Okudo and Nwabundo Anusim Copyright © 2016 Jerome Okudo and Nwabundo Anusim. All rights reserved. Spinal Cord Infarction in a Patient with Hereditary Spherocytosis: A Case Report and Discussion Mon, 14 Mar 2016 13:19:37 +0000 http://www.hindawi.com/journals/crinm/2016/7024120/ The etiology of spinal cord infarcts (SCIs), besides being related to aortic perioperative events, in large subset of SCIs, remains cryptogenic. We present a first case of SCI in a patient with hereditary spherocytosis and discuss the potential pathophysiologic considerations for vascular compromise. A 43-year-old woman with a history of hereditary spherocytosis, post splenectomy status, presented with chest, back, and shoulder pain with subsequent myelopathic picture; SCI extending from C4-T2 was confirmed by MRI. Despite aggressive treatment her stroke progressed leading to her demise. Her autopsy confirmed the SCI and revealed some incidental findings, but the cause of SCI remained unidentified. Exclusion of the known etiologies of SCI by extensive negative workup including autopsy evaluation suggested that SCI in our case was related to her history of hereditary spherocytosis. Both venous and arterial adverse vascular events, at a higher rate, have been associated in patients with hereditary spherocytosis who had their spleens removed compared to nonsplenectomized patients. Postsplenectomy increases in the platelet, red blood cell count, leukocyte count, and cholesterol concentrations are postulated to contribute to increased thrombotic risk. Additional prothrombotic factors include continuous platelet activation and adhesion as well as abnormalities of the red blood cell membrane. Waqar Waheed, Anjali L. Varigonda, Chris E. Holmes, Christopher Trevino, Neil M. Borden, and W. Pendlebury Copyright © 2016 Waqar Waheed et al. All rights reserved. A Rare Case Report on Suboccipital Region Benign Giant Osteoma Wed, 09 Mar 2016 13:37:51 +0000 http://www.hindawi.com/journals/crinm/2016/2096701/ Herein we report a rare case of a giant suboccipital osteoma in a 55-year-old woman presenting primarily due to cosmetic issue. We discuss the management algorithm taken in the patient, highlighting excision of a potentially curable bony tumor only after ruling out its extension to the ear cavity, mastoid ear cells, transverse sinus, and the intracranial compartment. Sunil Munakomi and Binod Bhattarai Copyright © 2016 Sunil Munakomi and Binod Bhattarai. All rights reserved. Septic Encephalopathy Characterized by Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion and Early Nonconvulsive Status Epilepticus Wed, 09 Mar 2016 13:23:05 +0000 http://www.hindawi.com/journals/crinm/2016/7528238/ Infection, whether viral or bacterial, can result in various forms of brain dysfunction (encephalopathy). Septic encephalopathy (SE) is caused by an excessive immune reaction to infection, with clinical features including disturbed consciousness and seizures. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is usually accompanied by viral infection in children and is characterized by biphasic seizures and impaired consciousness. The initial neurologic symptom of AESD is typically a febrile seizure that frequently lasts longer than 30 minutes. However, the possible forms this seizure takes are unclear. For example, it is unknown if nonconvulsive status epilepticus (NCSE) could be an early seizure symptomatic of AESD. In addition, thus far no cases of combined SE and AESD have been reported. Here, we describe the first reported case of SE with AESD that notably demonstrated NCSE as an early seizure. Hiroshi Yamaguchi, Tsukasa Tanaka, Azusa Maruyama, and Hiroaki Nagase Copyright © 2016 Hiroshi Yamaguchi et al. All rights reserved. Cerebral Venous Thrombosis with Migraine-Like Headache and the Trigeminovascular System Wed, 17 Feb 2016 14:38:17 +0000 http://www.hindawi.com/journals/crinm/2016/2059749/ Cerebral venous thrombosis- (CVT-) associated headache is considered a secondary headache, commonly presenting as intracranial hypertension headache in association with seizures and/or neurological signs. However, it can occasionally mimic migraine. We report a patient presenting with a migraine-like, CVT-related headache refractory to several medications but intravenous dihydroergotamine (DHE). The response to DHE, which is considered to be an antimigraine medication, in addition to the neurovascular nature of migraine, points out to a probable similarity between CVT-headache and migraine. Based on experimental studies, we discuss this similarity and hypothesize a trigeminovascular role in the genesis of CVT-associated headache. Fábio A. Nascimento, Marília Grando Sória, Vanessa Rizelio, and Pedro A. Kowacs Copyright © 2016 Fábio A. Nascimento et al. All rights reserved. The Kleine-Levin Syndrome: A Rare Disease with Often Delayed Diagnosis—A Report of Two Cases in the Department of Neurology of the University Hospital of Cocody (Côte d’Ivoire) Wed, 17 Feb 2016 13:57:36 +0000 http://www.hindawi.com/journals/crinm/2016/8929413/ The Kleine-Levin syndrome is a rare pathology characterized by recurrent episodes of hypersomnia associated with behavioral and cognitive disorders with, among others, hyperphagia and hypersexuality. The disease mainly affects young males. A few studies mention cases that occurred in Africa, especially in Côte d’Ivoire. In this paper, we report the very first two cases observed in the Neurology Department of the University Hospital of Cocody. The diagnosis was clinical, based on the recurrence of hypersomnia, cognitive and behavioral disorders during the periods of hypersomnia, and the return of patients to normal state between episodes. This diagnosis was delayed due to failure to understand the pathology, thereby leading patients to wandering. In fact, the two patients were consulted, respectively, 3 years and 6 years after the hypersomnia began. The objective was to report the very first cases observed in the Neurology Department of the University Hospital of Cocody, Côte d’Ivoire. Berthe Assi, Constance Yapo-Ehounoud, Mohamed Ben Allaoui Baby, Evelyne Aka-Diarra, Muriel Amon-Tanoh, and Christian Tanoh Copyright © 2016 Berthe Assi et al. All rights reserved. Babinski-Nageotte Syndrome Diagnosed in Postpartum Period Tue, 16 Feb 2016 16:22:51 +0000 http://www.hindawi.com/journals/crinm/2016/5206430/ Babinski-Nageotte Syndrome (BNS) is one of the brainstem syndromes characterized by muscle weakness in the opposite half of the body with classic Wallenberg findings. According to our literature survey, only a few cases have been reported and none of them was in the postpartum period. We report a case of a typical BNS in a postpartum woman with an ischemic lesion in the medulla oblongata shown on magnetic resonance imaging. Serdar Oruç, Hayri Demirbaş, Abdullah Güzel, Mehtap Beker Acay, and Mehmet Yaman Copyright © 2016 Serdar Oruç et al. All rights reserved. A Case of Ataxia with Isolated Vitamin E Deficiency Initially Diagnosed as Friedreich’s Ataxia Tue, 16 Feb 2016 10:03:16 +0000 http://www.hindawi.com/journals/crinm/2016/8342653/ Ataxia with isolated vitamin E deficiency (AVED) is a rare autosomal recessive condition that is caused by a mutation in the alpha tocopherol transfer protein gene. It is almost indistinguishable clinically from Friedreich’s ataxia but with appropriate treatment its devastating neurological features can be prevented. Patients can present with a progressive cerebellar ataxia, pyramidal spasticity, and evidence of a neuropathy with absent deep tendon reflexes. It is important to screen for this condition on initial evaluation of a young patient presenting with progressive ataxia and it should be considered in patients with a long standing ataxia without any diagnosis in view of the potential therapeutics and genetic counselling. In this case report we present a patient who was initially diagnosed with Friedreich’s ataxia but was later found to have AVED. Michael Bonello and Partha Ray Copyright © 2016 Michael Bonello and Partha Ray. All rights reserved. Neuroendoscopic Removal of Acute Subdural Hematoma with Contusion: Advantages for Elderly Patients Mon, 15 Feb 2016 12:21:57 +0000 http://www.hindawi.com/journals/crinm/2016/2056190/ Background. Large craniotomy for acute subdural hematoma is sometimes too invasive. We report good outcomes for two cases of neuroendoscopic evacuation of hematoma and contusion by 1 burr hole surgery. Case Presentation. Both patients arrived by ambulance at our hospital with disturbed consciousness after falling. Case was an 81-year-old man who took antiplatelet drugs for brain infarction. Case was a 73-year-old alcoholic woman. CT scanning showed acute subdural hematoma and frontal contusion in both cases. In the acute stage, glycerol was administered to reduce edema; CTs after 48 and 72 hours showed an increase of subdural hematoma and massive contusion of the frontal lobe. Disturbed consciousness steadily deteriorated. The subdural hematoma and contusion were removed as soon as possible by neuroendoscopy under local anesthesia, because neither patient was a good candidate for large craniotomy considering age and past history. 40%~70% of the hematoma was removed, and the consciousness level improved. Conclusion. Neuroendoscopic removal of acute subdural hematoma and contusion has advantages and disadvantages. For patients with underlying medical issues or other risk factors, it is likely to be effective. Ryota Tamura, Yoshiaki Kuroshima, and Yoshiki Nakamura Copyright © 2016 Ryota Tamura et al. All rights reserved. Unusual Presentation of Unilateral Isolated Probable Lyme Optic Neuritis Wed, 03 Feb 2016 08:37:51 +0000 http://www.hindawi.com/journals/crinm/2016/7471842/ Optic neuritis (ON) is one of the most common manifestations of central nervous system involvement caused by various etiologies. Lyme ON is an exceedingly rare ocular manifestation of Lyme disease (LD) and only a few cases have been published in the literature. Lyme ON is very rare but should be included in the differential diagnosis in unexplained cases, particularly in Lyme endemic areas. Careful and detailed examination and investigation are warranted to make the diagnosis. We report this case to increase awareness of clinicians to include Lyme disease in differential diagnosis of ON for unexplained cases of ON. Herein we present a unique case with a unilateral ON caused by LD along with pre- and posttreatment findings and literature review. Ahmet Z. Burakgazi and Carl S. Henderson Copyright © 2016 Ahmet Z. Burakgazi and Carl S. Henderson. All rights reserved. A New Concept for Carotid Artery Stenting: Coating the Atherosclerotic Plaque by Covered Stent before Bare Stent Implantation Tue, 02 Feb 2016 09:39:11 +0000 http://www.hindawi.com/journals/crinm/2016/3125629/ In carotid artery stenting (CAS) procedures, distal embolism, periprocedural stent thrombosis, and 30-day stroke due to the plaque fragmentation and protrusion caused by stent implantation and balloon dilation are frequent complications. In this technical case report, a case is presented of extracranial carotid artery stenosis treated with a covered stent and subsequent implantation of a bare stent. In addition, the possibility is discussed that this new technique prevents the distal microembolic complications, periprocedural stent thrombosis, and 30-day stroke of extracranial CAS. Erol Akgul Copyright © 2016 Erol Akgul. All rights reserved. Isolated Intracranial Rosai-Dorfman Disease Tue, 02 Feb 2016 07:28:53 +0000 http://www.hindawi.com/journals/crinm/2016/1972594/ Background. Rosai-Dorfman disease (RDD) is a benign histiocytic proliferative disorder of unknown etiology. This rare condition commonly causes massive cervical lymphadenopathy. Intracranial RDD without any nodal involvement is extremely rare. Case Report. A young Bangladeshi male complained of bilateral complete blindness with left sided deafness for about three years. There was no lymphadenopathy. MRI and CT scan of brain suggested an inflammatory/neoplastic (?meningioma) lesion located at left parasellar region which extended frontally to encircle both optic nerves and also to left prepontine area. Histopathologically the lesion was diagnosed as RDD. The patient was treated with steroid and significant clinical improvement observed. Conclusion. The prognosis of intracranial RDD is not poor. It can be treated with surgery with or without corticosteroids, chemotherapy, and so forth. But as the condition is extremely rare and often misdiagnosed, the clinician, radiologist, and histopathologist should have a suspicion in their mind about the possibility of RDD. Md. Taufiq, Abul Khair, Ferdousy Begum, Shabnam Akhter, Md. Shamim Farooq, and Mohammed Kamal Copyright © 2016 Md. Taufiq et al. All rights reserved. Isolated Cranial Nerve-III Palsy Secondary to Perimesencephalic Subarachnoid Hemorrhage Mon, 01 Feb 2016 09:53:45 +0000 http://www.hindawi.com/journals/crinm/2016/6319548/ We describe isolated cranial nerve-III palsy as a rare clinical finding in a patient with perimesencephalic subarachnoid hemorrhage. In this unusual case, the patient presented with complete cranial nerve-III palsy including ptosis and pupillary involvement. Initial studies revealed subarachnoid hemorrhage in the perimesencephalic, prepontine, and interpeduncular cisterns. Angiographic studies were negative for an intracranial aneurysm. The patient’s neurological deficits improved with no residual deficits on follow-up several months after initial presentation. Our case report supports the notion that patients with perimesencephalic subarachnoid hemorrhage have an excellent prognosis. Our report further adds a case of isolated cranial nerve-III palsy as a rare initial presentation of this type of bleeding, adding to the limited body of the literature. Justin R. Abbatemarco and Hussam A. Yacoub Copyright © 2016 Justin R. Abbatemarco and Hussam A. Yacoub. All rights reserved. Chronic Encapsulated Expanding Thalamic Hematoma Associated with Obstructive Hydrocephalus following Radiosurgery for a Cerebral Arteriovenous Malformation: A Case Report and Literature Review Sun, 24 Jan 2016 09:56:02 +0000 http://www.hindawi.com/journals/crinm/2016/5130820/ Chronic encapsulated intracerebral hematoma is a unique type of intracerebral hematoma accompanied by a capsule that is abundant in fragile microvasculature occasionally causing delayed regrowth. A 37-year-old man who had undergone radiosurgery for an arteriovenous malformation (AVM) causing intracerebral hematoma in the left parietal lobe presented with headache, vomiting, and progressive truncal ataxia due to a cystic lesion that had been noted in the left thalamus, leading to progressive obstructive hydrocephalus. He underwent left frontal craniotomy via a transsylvian fissure approach, and the serous hematoma was aspirated. The hematoma capsule was easy to drain and was partially removed. Pathological findings demonstrated angiomatous fibroblastic granulation tissue with extensive macrophage invasion. The concentration of vascular endothelial growth factor (VEGF) was high in the hematoma (12012 pg/mL). The etiology and pathogenesis of encapsulated hematoma are unclear, but the gross appearance and pathological findings are similar to those of chronic subdural hematoma. Based on the high concentration of VEGF in the hematoma, expansion of the encapsulated hematoma might have been caused by the promotion of vascular permeability of newly formed microvasculature in the capsule. Jun Takei, Toshihide Tanaka, Yohei Yamamoto, Akihiko Teshigawara, Satoru Tochigi, Yuzuru Hasegawa, and Yuichi Murayama Copyright © 2016 Jun Takei et al. All rights reserved. Optic Nerve Sheath Meningioma Masquerading as Optic Neuritis Tue, 19 Jan 2016 14:07:07 +0000 http://www.hindawi.com/journals/crinm/2016/5419432/ Optic neuritis is a common presentation of demyelinating disorders such as multiple sclerosis. It typically presents with acute painful monocular vision loss, whereas chronic optic neuropathy can be caused by compressive lesions along the anterior visual pathway, genetic, toxic, or nutritional causes. We report an unusual presentation mimicking optic neuritis, which was subsequently diagnosed as optic nerve sheath meningioma (ONSM). Misinterpretation of white matter lesions on MRI of brain and the failure to image the optic nerves at the time of acute loss of vision led to the misdiagnosis of optic neuritis in this case. A comprehensive accurate history and ordering the appropriate imaging modality remain paramount in diagnosing progressive visual deterioration. R. Alroughani and R. Behbehani Copyright © 2016 R. Alroughani and R. Behbehani. All rights reserved. Clival Ectopic Pituitary Adenoma Mimicking a Chordoma: Case Report and Review of the Literature Wed, 13 Jan 2016 13:16:23 +0000 http://www.hindawi.com/journals/crinm/2016/8371697/ Background. Purely ectopic pituitary adenomas are exceedingly rare. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Case Presentation. A 41-year-old male presented with an incidental clival lesion presumed to be a chordoma. On MRI it involved the entire clivus, extended laterally to the petroclival junction, and invaded the cavernous sinuses bilaterally, encasing both internal carotid arteries, without direct extension into the sella. Intraoperatively, it was clear that the tumor originated from the clivus and that the sellar dura was completely intact. Frozen-section pathology was consistent with a pituitary adenoma. Immunostaining was positive for synaptophysin and prolactin with a low Ki-67 index, suggestive of a prolactinoma. Additional immunohistochemical stains seen in chordomas (EMA, S100, and Brachyury) and other metastatic tumors were negative. A postoperative endocrine workup revealed an elevated serum prolactin of 881.3 ng/mL (normal < 20). Conclusions. In conclusion, it is crucial to maintain an extensive differential diagnosis when evaluating a patient with a clival lesion. Ectopic clival pituitary adenomas, although rare, may warrant an endocrinological workup preoperatively as the majority may respond to medical treatment. Constantine L. Karras, Isaac Josh Abecassis, Zachary A. Abecassis, Joseph G. Adel, Esther N. Bit-Ivan, Rakesh K. Chandra, and Bernard R. Bendok Copyright © 2016 Constantine L. Karras et al. All rights reserved. Cognitive Impairments Preceding and Outlasting Autoimmune Limbic Encephalitis Sun, 10 Jan 2016 14:06:42 +0000 http://www.hindawi.com/journals/crinm/2016/7247235/ Mild cognitive impairment (MCI) can be the initial manifestation of autoimmune limbic encephalitis (ALE), a disorder that at times presents a diagnostic challenge. In addition to memory impairment, clinical features that might suggest this disorder include personality changes, agitation, insomnia, alterations of consciousness, and seizures. Once recognized, ALE typically responds to treatment with immune therapies, but long-term cognitive deficits may remain. We report two cases of patients with MCI who were ultimately diagnosed with ALE with antibodies against the voltage gated potassium channel complex. Months after apparent resolution of their encephalitides, both underwent neuropsychological testing, which demonstrated persistent cognitive deficits, primarily in the domains of memory and executive function, for cases 1 and 2, respectively. A brief review of the literature is included. Robert Gross, Jennifer Davis, Julie Roth, and Henry Querfurth Copyright © 2016 Robert Gross et al. All rights reserved. Angiographic Evidence of a Purely Pial Bihemispheric Intracranial Hemangiopericytoma Tue, 05 Jan 2016 14:12:58 +0000 http://www.hindawi.com/journals/crinm/2016/5245078/ Background. Classification of hemangiopericytoma (HPC) has evolved to a mesenchymal, nonmeningothelial grade two or three neoplasm according to the World Health Organization; however its blood supply has always been defined by dual origin, pial and dural contribution. Case Description. We present the case of a patient with an intracranial HPC with only pial vascular supply. Angiography confirmed the lack of dural supply to this bihemispheric intracranial mass. Subsequent histologic examination confirmed the diagnosis of hemangiopericytoma. Angiographic evidence here is atypical of the natural history of hemangiopericytomas with dual vascular supply and was critical in the decision-making towards surgical resection without tumor embolization. Conclusion. Data presented suggests the lack of dural vascular supply alone does not rule out the diagnosis of hemangiopericytoma. Nathaniel Stetson, Sudhakar Vadivelu, Jiang Y. Li, Avi Setton, and David J. Chalif Copyright © 2016 Nathaniel Stetson et al. All rights reserved. Two Uncommon Causes of Guillain-Barré Syndrome: Hepatitis E and Japanese Encephalitis Tue, 22 Dec 2015 14:10:12 +0000 http://www.hindawi.com/journals/crinm/2015/759495/ We are presenting two cases of Guillain-Barré syndrome where it is preceded by hepatitis E virus (HEV) and Japanese encephalitis virus (JEV) infection, respectively. Our first case is a forty-three-year-old nondiabetic, nonhypertensive female who was initially diagnosed with acute HEV induced viral hepatitis and subsequently developed acute onset ascending quadriparesis with lower motor neuron type of bilateral facial nerve palsies and respiratory failure. Second patient was a 14-year-old young male who presented with meningoencephalitis with acute onset symmetric flaccid paraparesis. After thorough investigations it was revealed as a case of Japanese encephalitis. Our idea of reporting these two cases is to make ourselves aware about this potential complication of these two common infections. Dhrubajyoti Bandyopadhyay, Vijayan Ganesan, Cankatika Choudhury, Suvrendu Sankar Kar, Parthasarathi Karmakar, Vivek Choudhary, Prasun Banerjee, Debarati Bhar, Adrija Hajra, Manas Layek, and Sabyasachi Mukhopadhyay Copyright © 2015 Dhrubajyoti Bandyopadhyay et al. All rights reserved. Stroke as an Unusual First Presentation of Lyme Disease Wed, 16 Dec 2015 13:13:56 +0000 http://www.hindawi.com/journals/crinm/2015/389081/ Introduction. Lyme neuroborreliosis is a nervous system infection caused by spirochete Borrelia burgdorferi with diverse neurological complications. Stroke due to cerebral vasculitis is a rare consequence of neuroborreliosis and has been described in just a few case reports. Case Presentation. Here, we report the case of a 43-year-old patient who presented with discrete left-sided hemiparesis and amnestic cognitive impairment. Brain magnetic resonance imaging showed a thalamic infarct, and serological and cerebrospinal fluid (CSF) tests confirmed the diagnosis of active neuroborreliosis. The antibiotic treatment with intravenous ceftriaxone for three weeks led to an improvement of the symptoms and remarkable regression of radiological findings, but not to full recovery of the amnestic cognitive disorder. Conclusion. Lyme neuroborreliosis should be suspected in patients with cerebrovascular events without obvious risk factors, especially those living in endemic areas such as northern Europe or those who have been exposed to ticks and those with clinical or radiological findings suggesting Lyme neuroborreliosis, in order to establish the diagnosis and start a proper antibiotic therapy. Mohamad Almoussa, Angelika Goertzen, Barbara Fauser, and Christoph W. Zimmermann Copyright © 2015 Mohamad Almoussa et al. All rights reserved. Subacute Subdural Hematoma in a Patient with Bilateral DBS Electrodes Tue, 08 Dec 2015 13:23:02 +0000 http://www.hindawi.com/journals/crinm/2015/390727/ Subdural hematomas (SDH) in patients with implanted deep brain stimulating (DBS) electrodes are rare. Only a handful of cases have been reported in the literature. No clear management guidelines exist regarding the management of the hematoma and the existing electrodes. We describe a 68-year-old female with bilateral DBS electrodes, who presented with acute, severe hemiparesis due to a large subacute SDH with associated electrode displacement. Urgent hematoma evacuation reversed the hemiparesis; the electrodes were left undisturbed. Brain reexpansion occurred promptly. The patient was able to benefit from stable DBS therapies within 3 weeks of hematoma evacuation, maintained at 1.5-year follow-up. The case highlights that despite relative electrode migration due to a subdural hematoma, the electrodes may not require revision during initial hematoma evacuation or in a delayed fashion. Timely hematoma evacuation, coupled with brain reexpansion, may be adequate for the electrode to travel back to its original position and effect reasonable DBS therapies. Ha Son Nguyen and Peter A. Pahapill Copyright © 2015 Ha Son Nguyen and Peter A. Pahapill. All rights reserved. Paraspinal and Extensive Epidural Abscess: The Great Masqueraders of Abdominal Pain Sun, 06 Dec 2015 11:58:45 +0000 http://www.hindawi.com/journals/crinm/2015/103624/ Paraspinal and epidural abscesses are rare conditions often diagnosed later in the disease process that can have significant morbidity and mortality. Predisposing risk factors include diabetes, human immunodeficiency virus, intravenous drug abuse, and previous history of spinal surgery or injection. They can threaten the spinal cord by compressive effect, leading to sensory motor deficits and ultimately paralysis and death. Diagnosis may be a challenge due to the delayed presentation of nonspecific back pain or radicular pain such as chest pain or abdominal pain. We present a rare case on a patient with periumbilical pain, constipation, and urinary retention who was ultimately diagnosed with a paraspinal abscess extending into the epidural space from T1 to S2. He underwent decompressive laminectomy with incision and drainage of the abscesses. The patient made an excellent recovery postoperatively, and repeat magnetic resonance imaging at six weeks showed resolution of the abscess. Andrew Chu, Thu Thu Aung, and Uday Shankar Copyright © 2015 Andrew Chu et al. All rights reserved. The Myotonic Plot Thickens: Electrical Myotonia in Antimuscle-Specific Kinase Myasthenia Gravis Thu, 03 Dec 2015 11:58:00 +0000 http://www.hindawi.com/journals/crinm/2015/242691/ Electrical myotonia is known to occur in a number of inherited and acquired disorders including myotonic dystrophies, channelopathies, and metabolic, toxic, and inflammatory myopathies. Yet, electrical myotonia in myasthenia gravis associated with antibodies against muscle-specific tyrosine kinase (MuSK) has not been previously reported. We describe two such patients, both of whom had a typical presentation of proximal muscle weakness with respiratory failure in the context of a significant electrodecrement in repetitive nerve stimulation. In both cases, concentric needle examination revealed electrical myotonia combined with myopathic motor unit morphology and early recruitment. These findings suggest that MuSK myasthenia should be included within the differential diagnosis of disorders with electrical myotonia. Marcus Magnussen, Ioannis Karakis, and Taylor B. Harrison Copyright © 2015 Marcus Magnussen et al. All rights reserved.