Case Reports in Neurological Medicine The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . 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. Hypoglycemia-Induced Hemiparesis in a Diabetic Woman after Childbirth Thu, 23 Apr 2015 13:07:34 +0000 A 24-year-old female with type 1 diabetes mellitus presented with hemiparesis induced by hypoglycemia. She was hospitalized because she has noticed a weakness of her right hand and leg three days after childbirth. On physical examination she had an expressive dysphasia and right side hemiparesis with facial drop. Hypoglycemia is rarely associated with hemiparesis and it is often overlooked, especially when it happens in patients at higher risk of other diseases frequently associated with hemiparesis. Although sporadical cases of hypoglycemia-induced hemiparesis were reported, the clear pathophysiology behind this is not well determined. However, any individual case is important in order to increase the awareness of hypoglycemia as an important etiology of this condition. Vera Kukaj, Fisnik Jashari, Dren Boshnjaku, Enis Istrefi, and Pranvera Ibrahimi Copyright © 2015 Vera Kukaj et al. All rights reserved. Cavernous Hemangioma of the Skull and Meningioma: Association or Coincidence? Thu, 16 Apr 2015 12:19:23 +0000 Intraosseous cavernous hemangiomas of the skull are rare. Meningiomas are quite frequently encountered in a neurosurgical practice. The association between these two entities is nevertheless very uncommon. The authors present a case of a 72-year-old woman suffering from headache. The MRI showed a parietal meningioma with adjacent thick bone. The meningioma and the bone were removed. The histological examination confirmed the diagnosis of meningioma and revealed a cavernoma of the skull. The relationship between the lesions suggests more than a coincidental association. Several hypotheses are proposed to explain common causal connections. M. Kilani, M. Darmoul, F. Hammedi, A. Ben Nsir, and M. N. Hattab Copyright © 2015 M. Kilani et al. All rights reserved. Neuralgia of the Glossopharyngeal Nerve in a Patient with Posttonsillectomy Scarring: Recovery after Local Infiltration of Procaine—Case Report and Pathophysiologic Discussion Thu, 16 Apr 2015 06:24:35 +0000 We describe a patient with a three-year history of severe progressive left-sided glossopharyngeal neuralgia (GPN) that failed to adequately respond to various drug therapies. The application of lidocaine spray to the posterior pharyngeal wall provided no more than short-term relief. Apart from a large hypertrophic tonsillectomy scar on the left side all clinical and radiologic findings were normal. In terms of therapeutic local anaesthesia, the hypertrophic tonsillectomy scar tissue was completely infiltrated with the local anaesthetic (LA) procaine 1%. The patient has been almost completely pain-free ever since, and the lidocaine spray is no longer needed. Six weeks after the first treatment a repeat infiltration of the tonsillectomy scar led to the complete resolution of all symptoms. The patient has become totally symptom-free without the need to take any medication now for two and a half years. This is the first report of a successful therapeutic infiltration of a tonsillectomy scar using an LA in a patient with GPN that has been refractory to medical treatment for several years. A possible explanation may be that the positive feedback loop maintaining neurogenic inflammation is disrupted and “sympathetically maintained pain” resolved by LA infiltration. L. Fischer, S. M. Ludin, K. Puente de la Vega, and M. Sturzenegger Copyright © 2015 L. Fischer et al. All rights reserved. Severe Hyperammonemia in Late-Onset Ornithine Transcarbamylase Deficiency Triggered by Steroid Administration Thu, 09 Apr 2015 09:29:26 +0000 Ornithine transcarbamylase deficiency (OTCD) is a rare X-linked disorder of urea synthesis leading to hyperammonemia. Several late-onset cases have been reported. Undiagnosed and untreated patients are at the risk of death or suffering from irreversible sequelae. We describe a 56-year-old patient who presented with acute encephalopathy after steroid treatment. Hyperammonemia due to OTCD was diagnosed and a mutation was found. This allowed us to diagnose two other family members with unexplained encephalopathy who are now asymptomatic on a low-protein diet. OTCD should be considered in any patient with hyperammonemic encephalopathy and immediate treatment should be given to avoid a fatal outcome. We emphasize the need to examine other family members if the diagnosis is confirmed, in order to prevent further life-threatening episodes of encephalopathy or neonatal coma of newborn. Jordi Gascon-Bayarri, Jaume Campdelacreu, Jordi Estela, and Ramon Reñé Copyright © 2015 Jordi Gascon-Bayarri et al. All rights reserved. Extraspinal Type I Dural Arteriovenous Fistula with a Lumbosacral Lipomyelomeningocele: A Case Report and Review of the Literature Wed, 08 Apr 2015 14:15:41 +0000 Seven cases of adult spinal vascular malformations presenting in conjunction with spinal dysraphism have been reported in the literature. Two of these involved male patients with a combined dural arteriovenous fistula (DAVF) and lipomyelomeningocele. The authors present the third case of a patient with an extraspinal DAVF and associated lipomyelomeningocele in a lumbosacral location. A 58-year-old woman with rapid decline in bilateral motor function 10 years after a prior L4-5 laminectomy and cord detethering for diagnosed tethered cord underwent magnetic resonance imaging showing evidence of persistent cord tethering and a lipomyelomeningocele. Diagnostic spinal angiogram showed a DAVF with arterial feeders from bilateral sacral and the right internal iliac arteries. The patient underwent Onyx embolization of both feeding right and left lateral sacral arteries. At 6-month follow-up, MRI revealed decreased flow voids and new collateralized supply to the DAVF. The patient underwent successful lipomyelomeningocele exploration, resection, AV fistula ligation, and cord detethering. This report discusses management of this patient as well as the importance of endovascular embolization followed by microsurgery for the treatment of cases with combined vascular and dysraphic anomalies. Khaled M. Krisht, Michael Karsy, Wilson Z. Ray, and Andrew T. Dailey Copyright © 2015 Khaled M. Krisht et al. All rights reserved. Unusual Neurologic Manifestations of a Patient with Cyanotic Congenital Heart Disease after Phlebotomy Thu, 02 Apr 2015 18:25:37 +0000 Secondary erythrocytosis in cyanotic congenital heart disease (CCHD) is a compensatory response to chronic hypoxia which should be managed with caution. CCHD patients, who have compensated erythrocytosis but do not manifest significant neurologic symptoms, may experience secondary life-threatening complications such as stroke in case of inappropriate phlebotomy. This study reports a young man with CCHD who developed frequently repeated transient neurologic deficits with various presentations after one session of phlebotomy. The symptoms resolved a few days after the hematocrit (Hct) level returned to the prephlebotomy level. Hooman Salimipour, Somayeh Mehdizadeh, Reza Nemati, Mohamad Reza Pourbehi, Gholam Reza Pourbehi, and Majid Assadi Copyright © 2015 Hooman Salimipour et al. All rights reserved. Resolution of Internal Carotid Dissection with Middle Cerebral Artery Occlusion in Pregnancy Mon, 30 Mar 2015 07:03:37 +0000 Introduction. Cervical artery dissection (CAD) is a common cause of stroke in younger patients. While the incidence of stroke in pregnancy is increasing, CAD remains a rare cause of ischemic stroke in the pregnant population, with only 30 cases described in the literature, most in the postpartum period. Methods. The case of a pregnant patient at 18 weeks of gestation presenting with CAD and ischemic stroke following intercourse is discussed. Discussion. CAD results from an intimal tear in the carotid artery, allowing accumulation of blood in the vessel wall. Stroke results from embolization of thrombogenic material in the wall. Etiology includes minor trauma, connective tissue disorders, or anatomic variations of the carotid artery. Most patients present with headache and/or neck pain, while ischemic symptoms are seen in at least 50% of patients. In the pregnant population, imaging with MRI or MRA of the head and neck aids in diagnosis. Once the diagnosis is made, patients are treated with either anticoagulation or antiplatelet medications. The optimal treatment in both pregnant and nonpregnant patients has not been well-studied. Conclusion. CAD is an important diagnosis to consider in a pregnant patient with persistent headache, especially if neurological symptoms are present. Imaging should be quickly obtained so treatment can be initiated. Nicole Ulrich, Amanda Johnson, Dominique Jodry, Chi Dola, Sheryl Martin-Schild, and Ramy El Khoury Copyright © 2015 Nicole Ulrich et al. All rights reserved. Idiopathic Generalized Epilepsy and Hypokalemic Periodic Paralysis in a Family of South Indian Descent Sun, 29 Mar 2015 05:47:27 +0000 Inherited channelopathies are a heterogeneous group of disorders resulting from dysfunction of ion channels in cellular membranes. They may manifest as diseases affecting skeletal muscle contraction, the conduction system of the heart, nervous system function, and vision syndromes. We describe a family of South Indian descent with hypokalemic periodic paralysis in which four members also have idiopathic generalized epilepsy. Hypokalemic periodic paralysis is a genetically heterogeneous channelopathy that has been linked to mutations in genes encoding three ion channels CACNIAS, SCN4A, and KCNJ2 predominantly. Although data on specific gene in idiopathic generalized epilepsy is relatively scarce, mutations of voltage gated sodium channel subunit genes (CACNB4) and nonsense mutations in voltage gated calcium channels (CACNA1A) have been linked to idiopathic generalized epilepsy in two families. We speculate that gene mutations altering the ability of the beta subunit to interact with the alpha subunit of the CaV1.1 channel and mutations in the pore-forming potassium channel subunit may be possible explanations for the combined manifestation of both diseases. Functional analysis of voltage gated calcium channel and other ion channels mutations may provide additional support and insight for the causal role of these mutations. The understanding of mutations in ion-channel genes will lead to improved diagnosis and treatment of such inherited channelopathies. Muthiah Subramanian, N. Senthil, and S. Sujatha Copyright © 2015 Muthiah Subramanian et al. All rights reserved. Minor Trauma Causing Stroke in a Young Athlete Thu, 26 Mar 2015 12:38:54 +0000 A 17-year-old Caucasian male presented with sudden dizziness, ataxia, vertigo, and clumsiness lasting for a couple of hours. He had a subtle trauma during a wrestling match 2 days prior to the presentation. A CT Angiogram (CTA) and MRI showed left vertebral artery dissection (VAD). The patient was treated with anticoagulation with heparin drip in the ICU. The patient was discharged home on the third day on Lovenox-warfarin bridging. This case underscores the importance of considering VAD as a differential diagnosis in patients with sports-related symptoms especially in activities entailing hyperextension or hyperrotation of neck. Due to a varied latent period, often minor underlying trauma, and subtle presentation, a low index of suspicion is warranted in timely diagnosis and treatment of VAD. Considering recent evidence in treatment modality, either antiplatelet therapy or anticoagulation may be used for treatment of VAD. Vineet Gupta, Naveen Dhawan, and Jaya Bahl Copyright © 2015 Vineet Gupta et al. All rights reserved. Relapsing Remitting Multiple Sclerosis in X-Linked Charcot-Marie-Tooth Disease with Central Nervous System Involvement Wed, 25 Mar 2015 11:34:55 +0000 We report a patient with relapsing remitting multiple sclerosis (MS) and X-linked Charcot-Marie-Tooth disease (CMTX), carrying a GJB1 mutation affecting connexin-32 (c.191G>A, p. Cys64Tyr) which was recently reported by our group. This is the third case report of a patient with CMTX developing MS, but it is unique in the fact that other family members carrying the same mutation were found to have asymptomatic central nervous system (CNS) involvement (diffuse white matter hyperintensity on brain MRI and extensor plantars). Although this may be a chance association, the increasing number of cases with CMTX and MS, especially with mutations involving the CNS, may imply some causative effect and provide insights into MS pathogenesis. Georgios Koutsis, Georgia Karadima, Paraskewi Floroskoufi, Maria Raftopoulou, and Marios Panas Copyright © 2015 Georgios Koutsis et al. All rights reserved. GH-Producing Pituitary Adenoma and Concomitant Rathke’s Cleft Cyst: A Case Report and Short Review Wed, 25 Mar 2015 08:32:44 +0000 Concomitant pituitary adenoma (PA) and Rathke’s cleft cyst (RCC) are rare. In some cases, such PA is known to produce pituitary hormones. A 53-year-old man was admitted to our hospital with a diagnosis of lacunar infarction in the left basal ganglia. Magnetic resonance imaging (MRI) incidentally showed a suprasellar mass with radiographic features of RCC. When he consulted with a neurosurgical outpatient clinic, acromegaly was suspected based on his appearance. A diagnosis of growth hormone- (GH-) producing PA was confirmed from hormonal examinations and additional MRI. Retrospectively, initial MR images also showed intrasellar mass that is compatible with the diagnosis of PA other than suprasellar RCC. The patient underwent endonasal-endoscopic removal of the PA. Since we judged that the RCC of the patient was asymptomatic, only the PA was completely removed. The postoperative course of the patient was uneventful and GH levels gradually normalized. Only 40 cases of PA with concomitant RCC have been reported to date, including 13 cases of GH-producing PA. In those 13 cases, RCC tended to be located in the sella turcica, and suprasellar RCC like this case appears rare. In a few cases, concomitant RCCs were fenestrated, but GH levels normalized postoperatively as in the cases without RCC fenestration. If radiographic imaging shows typical RCC, and PA is not obvious at first glance, the possibility of concomitant PA still needs to be considered. In terms of treatment, removal of the RCC is not needed to achieve hormone normalization. Ryota Tamura, Satoshi Takahashi, Katsura Emoto, Hideaki Nagashima, Masahiro Toda, and Kazunari Yoshida Copyright © 2015 Ryota Tamura et al. All rights reserved. JC Virus PCR Detection Is Not Infallible: A Fulminant Case of Progressive Multifocal Leukoencephalopathy with False-Negative Cerebrospinal Fluid Studies despite Progressive Clinical Course and Radiological Findings Thu, 12 Mar 2015 09:31:15 +0000 We describe a case with a false-negative PCR-based analysis for JC virus in cerebrospinal fluid (CSF) in a patient with clinical and radiological findings suggestive of progressive multifocal leukoencephalopathy (PML) who was on chronic immunosuppressive therapy for rheumatoid arthritis. Our patient developed rapidly progressive global decline with clinical and radiographic findings suggestive of PML, but JC virus PCR in CSF was negative. The patient passed away 3 months from the onset of her neurological symptoms. Autopsy confirmed the diagnosis of PML with presence of JC-polyoma virus by immunohistochemical staining. This case highlights the potential of false-negative JC virus PCR in CSF when radiographic and clinical features are suggestive of “possible PML.” We review the plausible causes of potential false-negative CSF results and suggest that when the clinical presentation is suspicious for PML repeat CSF analysis utilizing ultrasensitive PCR assay and subsequent brain biopsy should be considered if CSF remains negative. Additionally, appropriate exclusion of other neurologic conditions is essential. Mohamed-Ali Babi, William Pendlebury, Steven Braff, and Waqar Waheed Copyright © 2015 Mohamed-Ali Babi et al. All rights reserved. Acute Aortic Occlusion Presenting as Flaccid Paraplegia Wed, 11 Mar 2015 13:47:28 +0000 A 67-year-old male known to be hypertensive and diabetic had a sudden onset of severe low back pain and flaccid paraplegia with no sensory level or bladder affection and the distal pulsations were felt. Acute compressive myelopathy was excluded by MRI of the dorsal and lumbar spines. The nerve conduction study and CSF analysis was suggestive of acute demyelinating polyneuropathy. The patient developed ischemic changes of the lower limb and CT angiography revealed severe stenosis of the abdominal aorta and both common iliac arteries. We emphasize the importance of including acute aortic occlusion in the differential diagnosis of acute flaccid paraplegia especially in the presence of severe back pain even if the distal pulsations were felt. Ayman Kilany, Jasem Y. Al-Hashel, and Azza Rady Copyright © 2015 Ayman Kilany et al. All rights reserved. A CADASIL-Like Case with a Novel Noncysteine Mutation of the NOTCH3 Gene and Granular Deposits in the Renal Arterioles Thu, 05 Mar 2015 12:03:00 +0000 We herein report the finding of a 62-year-old male, who developed dysarthria and dysphagia, with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy- (CADASIL-) like cerebral lesions. He also suffered from slowly progressive renal failure with the findings of granular deposits similar to electron-dense granular osmiophilic material in the renal arterioles. We found a novel heterozygous missense mutation of the NOTCH3 gene, c.4039G>C in exon 24, resulting in a p.Gly1347Arg substitution in its extracellular domain. The noncysteine substitution may underlie the pathogenesis of white matter lesions in the brain and of the chronic renal failure in the present case. Kuniyuki Nakamura, Tetsuro Ago, Akihiro Tsuchimoto, Nozomi Noda, Asako Nakamura, Toshiharu Ninomiya, Takeshi Uchiumi, Kazuhiko Tsuruya, Masahiro Kamouchi, Hiroaki Ooboshi, and Takanari Kitazono Copyright © 2015 Kuniyuki Nakamura et al. All rights reserved. Acute Dysphasia and Reversible Cognitive Decline in a Patient with Probable Cerebral Amyloid Angiopathy-Related Inflammation Mon, 02 Mar 2015 13:23:48 +0000 Cerebral amyloid angiopathy related inflammation (CAAri) is becoming increasingly recognised as a subset of cerebral amyloid angiopathy (CAA). CAAri generally presents with subacute cognitive decline, headaches, seizures, behavioral changes, and focal neurological deficits. We describe a patient who developed acute dysphasia and reversible cognitive decline due to probable CAAri. CT brain showed bilateral vasogenic edema in the cerebral hemispheres, predominantly involving the parietal and temporal lobes, left greater than right without enhancement. Magnetic resonance brain imaging showed extensive multifocal areas of subcortical white matter T2 hyperintensity in the frontal and temporal regions with associated mass effect, negligible enhancement, and multiple foci of microhemorrhage on susceptibility weighted imaging sequences consistent with a diagnosis of probable CAAri. She responded dramatically to a course of intravenous methylprednisolone followed by further immunosuppression with pulse intravenous cyclophosphamide. Her dysphasia resolved within 5 days of intravenous methylprednisolone therapy. Her MMSE improved from 11/30 at day 5 of admission to 28/30 at 6-month follow-up. The notable features of our case were the unusual CT findings, which were inconsistent with stroke and diagnostic utility of susceptibility-weighted magnetic resonance imaging in confirming the diagnosis which allowed for prompt institution of immunosuppression. Louise Rigney, Dale Sebire, and Dennis Cordato Copyright © 2015 Louise Rigney et al. All rights reserved. A Case of Apoplexy of Rathke’s Cleft Cyst Followed by Cerebral Infarction Wed, 25 Feb 2015 09:40:23 +0000 Rathke’s cleft cyst (RCC) apoplexy is a rare clinical entity. We report a case of apoplexy of an RCC followed by cerebral infarction. A 67-year-old woman was found lying on the street unconscious. She had fallen from her motorbike. On referral to our hospital she gradually regained consciousness and presented with no neurological deficits. CT showed a round and slightly hyperdense area in the suprasellar region. However, the attending physician did not find this abnormal finding on CT and the patient was discharged the same day. Thirteen days after the first emergency visit she developed left hemiparesis and dysarthria. CT showed a round hypodense area in the suprasellar region. The change of the density in the suprasellar region on CT suggested the pituitary apoplexy. CT also showed a low density area in the territory of the right middle cerebral artery, which indicated the cerebral infarction. MR angiography revealed poor visibility and stenotic changes of right middle cerebral arteries. Transsphenoidal surgery was performed. Histopathological findings confirmed a hemorrhagic RCC. Postoperative MR angiography showed that the visibility and stenosis of right middle cerebral arteries were recovered. This is the rare case of apoplexy of an RCC followed by cerebral infarction. Yu-ichiro Ohnishi, Yasunori Fujimoto, Koichi Iwatsuki, and Toshiki Yoshimine Copyright © 2015 Yu-ichiro Ohnishi et al. All rights reserved. Posterior Reversible Encephalopathy Syndrome as Presenting Form of Very Early Systemic Sclerosis Mon, 23 Feb 2015 09:35:07 +0000 Introduction. Posterior Reversible Encephalopathy Syndrome (PRES) is an increasingly recognized clinical and radiological entity with a wide spectrum of symptoms. Its mechanism depends on failure of the blood-brain barrier due to high systemic blood pressure (BP) and loss of integrity of vascular endothelium related with different triggers. Methods. We aim to report a case of PRES induced by arterial hypertension and very early systemic sclerosis (SSc) not previously known. Results. A 64-year-old female was admitted due to 1-week pulsating headache more prominent on frontal scalp, accompanied by phonophobia, photophobia, and facial flushing. Neurological exam revealed brisk deep tendon reflex. Brain magnetic resonance imaging (MRI) showed subcortical lesions mainly located in posterior regions. BP was monitored and episodic arterial hypertension was detected. In laboratory tests positive anti-topoisomerase I antibodies were detected. BP was controlled with angiotensin-converting-enzyme inhibitors and headache improved. In a new MRI a month later improvement of white matter lesions was observed. Capillaroscopy showed “active pattern,” considered typical of SSc. Conclusion. In SSc anti-endothelial cell antibodies impair vascular endothelium and liberation of vasoconstrictors leads to BP increasing and disruption of blood-brain barrier autoregulation mechanisms. PRES can be the first manifestation of very early SSc and this entity should be considered even in absence of skin lesions or Raynaud phenomenon. María Isabel Pedraza, Julia Barbado, Marina Ruiz, and Ángel Luis Guerrero Copyright © 2015 María Isabel Pedraza et al. All rights reserved. Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration Mon, 23 Feb 2015 08:13:05 +0000 Pantothenate kinase-associated neurodegeneration (PKAN) is usually associated with dystonia, which is typically severe and progressive over time. Pallidal stimulation (GPi DBS) has been carried out in selected cases of PKAN with drug-resistant dystonia with variable results. We report a 30-month follow-up study of a 30-year-old woman with PKAN-related dystonia treated with GPi DBS. Postoperatively, the benefit quickly became evident, as the patient exhibited a marked improvement in her dystonia, including her writing difficulty. This result has been maintained up to the present. GPi DBS should be considered in dystonic PKAN patients provided fixed contractures and/or pyramidal symptoms are not present. Pedro J. Garcia-Ruiz, Joaquin Ayerbe, Lydia Vela Desojo, Cici E. Feliz, and Javier del Val Fernandez Copyright © 2015 Pedro J. Garcia-Ruiz et al. All rights reserved. Meningioma of Foramen Magnum Causing Drop Attacks Sun, 22 Feb 2015 14:30:42 +0000 A 52-year-old female presented with frequent episodes of falls without loss of consciousness. These episodes lasted for brief period followed by full neurological recovery. Magnetic resonance imaging (MRI) of the brain showed foramen magnum meningioma encasing left vertebral artery. The patient had dramatic improvement after excision of the tumor. Amit Mahore, Raghvendra Ramdasi, Sandip Mavani, Vithal Rangarajan, Manoj Patil, Prashant Sathe, Juhi Kawale, and Vishakha Tikeykar Copyright © 2015 Amit Mahore et al. All rights reserved. Crossed-Brain Representation of Verbal and Nonverbal Functions Sat, 31 Jan 2015 14:27:27 +0000 A 74-year-old, left-handed man presented with a rapidly evolving loss of strength in his right leg associated with difficulty in walking. MR images disclosed an extensive left hemisphere tumor. A neuropsychological examination revealed that language was broadly normal but that the patient presented with severe nonlinguistic abnormalities, including hemineglect (both somatic and spatial), constructional defects, and general spatial disturbances; symptoms were usually associated with right hemisphere pathologies. No ideomotor apraxia was found. The implications of crossed-brain representations of verbal and nonverbal functions are analyzed. Esmeralda Matute, Alfredo Ardila, Monica Rosselli, Jahaziel Molina Del Rio, Ramiro López Elizalde, Manuel López, and Angel Ontiveros Copyright © 2015 Esmeralda Matute et al. All rights reserved. Late Occurrence of PML in a Patient Treated for Lymphoma with Immunomodulatory Chemotherapies, Bendamustine, Rituximab, and Ibritumomab Tiuxetan Thu, 29 Jan 2015 09:27:50 +0000 PML caused by John Cunningham (JC) virus is a rare but an increasingly recognized entity. With the advent of newer immunomodulatory therapies with monoclonal antibodies, there is an increasing incidence of PML. Initially concern was restricted to patients treated for multiple sclerosis with natalizumab but more case reports are being reported during treatment for other conditions like Crohn’s disease and lymphoma with agents such as rituximab. We report the case of a 66-year-old woman who developed PML a year after completion of therapy with rituximab, ibritumomab, and bendamustine. Michael A. Lane, Vijay Renga, Andrew R. Pachner, and Jeffrey A. Cohen Copyright © 2015 Michael A. Lane et al. All rights reserved. Funicular Myelosis in a Butcher: It Was the Cream Cans Wed, 28 Jan 2015 15:44:33 +0000 Background. Funicular myelosis is a known consequence of exposure to nitrous oxide. Nevertheless, there are only a few clinical trials assessing its long-term effects and there is no literature about the role of nutritional vitamin B12 supplementation in the context of nitrous oxide abuse. Case Descriptions. We diagnosed funicular myelosis in a young butcher, who consumed high amounts of meat regularly. Since the diagnostic process did not reveal any metabolic causes, reinterrogation of the patient uncovered recreational abuse of nitrous oxide out of whipped cream can gas cartridges. After stopping abuse and supplementation of vitamin B12, the patient recovered almost completely. Conclusions. In our case, even high nutritional vitamin B12 uptake could not compensate the noxious effects of nitrous oxide. Since there are emerging reports of increasing misuse, this should be considered in the diagnostic and therapeutic care of patients with nitrous oxide abuse. Furthermore, our case emphasizes that patients with vitamin B12 deficiency should be assessed for nitrous oxide abuse. Fabian Wolpert, Krisztina Baráth, Janis Brakowski, Roland Renzel, Michael Linnebank, and Andreas R. Gantenbein Copyright © 2015 Fabian Wolpert et al. All rights reserved. Cerebral Metastasis from Breast Cancer in a Male Patient with HIV Wed, 28 Jan 2015 11:27:14 +0000 Context. Breast cancer (BC) in men is a rare condition, corresponding to 1% of all neoplasms in this gender. Some studies show that up to 93% of BC cases in men are advanced disease. If its occurrence constitutes an uncommon fact, the appearance of a metastasis to the central nervous system (CNS) is extremely rare. The objective of the present study is to present the case of a male patient, bearer of HIV infection, who presented with BC and later metastasis to the CNS. We also include a brief review of the literature. Case Report. We describe a case of a male patient, 59 years old, with HIV infection and a history of BC treated 4 years earlier, which progressed into headache and vertigo. Neuroimaging exams showed lesions suggestive of cerebral metastasis and a stereotaxic biopsy confirmed BC metastasis. Conclusion. Breast cancer in men with metastasis to the CNS is a rare condition and similar reports were not found in the available databases. It should be pointed out that even though rare, it should be considered among the differential diagnoses for SNC metastases in men, although HIV infection favors the appearance of some types of cancer. Guilherme Lellis Badke, Guilherme Brasileiro de Aguiar, João Miguel de Almeida Silva, Aline Lariessy Campos Paiva, Eduardo Urbano da Silva, and José Carlos Esteves Veiga Copyright © 2015 Guilherme Lellis Badke et al. All rights reserved. Syringomyelia with Chiari I Malformation Presenting as Hip Charcot Arthropathy: A Case Report and Literature Review Tue, 27 Jan 2015 06:52:29 +0000 Neuroarthropathy (neuropathic osteoarthropathy), also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot) neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis). We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI) of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine. Roya Memarpour, Basheer Tashtoush, Lydia Issac, and Fernando Gonzalez-Ibarra Copyright © 2015 Roya Memarpour et al. All rights reserved. Intraosseous Lipomatous Meningioma Mon, 26 Jan 2015 14:38:24 +0000 A 49-year-old man with intermittent headaches and right sided parietal lump was found to have an intraosseous right parietal lesion on computed tomography (CT) and magnetic resonance imaging (MRI). A stereotactic craniectomy and excision of the lesion were performed with histopathology confirming features consistent with primary lipomatous meningioma with intraosseous extension. Lipomatous meningiomas are very uncommon subtype of meningiomas, with ongoing discussions as to their true pathogenesis. To our knowledge this case represents the first reported case of a lipomatous meningioma with predominant intraosseous extension. Lauren Kim, Christopher Huang, Adrienne L. Morey, and Mark J. Winder Copyright © 2015 Lauren Kim et al. All rights reserved.