Case Reports in Neurological Medicine The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . 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. Occipital Neuralgia after Hair Transplantation and Its Treatment Mon, 26 Jan 2015 07:01:40 +0000 While undergoing full thickness tissue harvest from the posterior scalp, a 72-year-old man experienced immediate severe pain in the right occiput and was unable to complete the procedure. The pain was constant “sharp” and “shocking” with numbness in the distribution of the lesser occipital nerve, exacerbated by physical activity, and local anesthetic blocks provided temporary complete relief. After numerous treatments over several years, including oral analgesics, botulinum toxin injections, and acupuncture, proved ineffective, pulsed radiofrequency neuromodulation provided greater than 80% relief for 5 months. Jason Siefferman and Yury Khelemsky Copyright © 2015 Jason Siefferman and Yury Khelemsky. All rights reserved. Papillary Tumor of the Pineal Region: MR Signal Intensity Correlated to Histopathology Thu, 22 Jan 2015 10:05:04 +0000 Tumors of the pineal region are rare and can be challenging to differentiate by imaging. Papillary tumor of the pineal region (PTPR) was recently recognized as a neoplasm in the World Health Organization (WHO) 2007 classification, arising from specialized ependymocytes in the subcommissural organ, which is located in the pineal region. It is a rare histological type of pineal tumor with only a few cases reported. Here, we describe a case of histologically confirmed PTPR in a 17-year-old man who presented with a headache. A literature review was performed to clarify the clinical, radiological, and pathological features of PTPR. Pineal neoplasms do not have pathognomonic imaging findings; however, we discuss T1 hyperintensity, which is a key for imaging diagnosis according to recent reports. In particular, if the hyperintensity in T1 is not due to fat, calcification, melanin, or hemorrhage in a mass of the posterior commissure or pineal region, the diagnosis of a PTPR may be suggested, as observed in this case. Marcos Rosa Junior, Antonio Jose da Rocha, Adriano Zanon da Silva, and Sergio Rosemberg Copyright © 2015 Marcos Rosa Junior et al. All rights reserved. Massive Cerebral Gas Embolism under Discectomy due to Hydrogen Peroxide Irrigation Tue, 20 Jan 2015 10:50:41 +0000 Massive cerebral and spinal gas embolism occurs rarely as a complication of discectomy. We report a 54-year-old female who had undergone a discectomy (L3/4 and L4/5) under epidural anesthesia in a local hospital developed multiple massive gas embolisms. At closure, surgeons irrigated the incision wound with hydrogen peroxide. Soon after the irrigation, the patient suddenly developed tachycardia, hypotension, and rapid oxygen desaturation. Subsequently, patient progressed into unconsciousness and right hemianopsia quadriplegia. Computed tomography (CT) scan showed multiple hypointensity spots around the brain due to cerebral gas embolism, which indicated the pneumoencephalos. The likely mechanism was the absorption of hydrogen peroxide into blood. When the amount of oxygen evolved exceeded its maximal blood solubility, venous embolization occurred. Though the patient was treated with supportive treatments and hyperbaric oxygen, she did not get full recovery and was left with severe long-term cerebral injury. Junjie Zhang, Chengliang Zhang, and Jianqin Yan Copyright © 2015 Junjie Zhang et al. All rights reserved. Subarachnoid Hemorrhage Secondary to Forceful Sneeze Sun, 18 Jan 2015 10:22:20 +0000 Subarachnoid hemorrhage (SAH) is a relatively less common but important neurological condition comprising 5% of all the cerebrovascular accidents. In most populations the reported incidence is 6-7 per 100,000 person-years and one-third of survivors become dependent. It is a serious but potentially treatable cause of neurological morbidity. Multiple authors have identified the most unusual novel associations and triggers of subarachnoid bleeds over the past decade. We herein report a rare case of subarachnoid hemorrhage leading to focal neurological deficit in a middle aged man secondary to forceful sneeze. Ali Zohair Nomani, Haris Majid Rajput, Mansoor Iqbal, Zakir Jan, Muhammad Irshad, Mazhar Badshah, and Rao Sohail Yasin Khan Copyright © 2015 Ali Zohair Nomani et al. All rights reserved. Consideration of Two Cases of Ascending Aortic Dissection That Began with Stroke-Like Symptoms Sun, 18 Jan 2015 06:26:21 +0000 We recently experienced two patients with stroke-like symptoms and ascending aortic dissection (AAD) in our outpatient department. Both patients were transferred to our hospital presenting with neurological deficit such as hemiparesis and conjugate deviation. They did not complain from any chest or abdominal pain. Their MRI did not show fresh infarction or main branch occlusion. A chest CT image showed AAD. The former patient was immediately transferred to a tertiary hospital and the latter received conservative management in the cardiovascular department. Discussion. As neither patient was experiencing any pain, we initially diagnosed them with ischemic stroke and began treatment. Fortunately, bleeding complications did not occur. In such cases, problems are caused when intravenous tissue plasminogen activator (t-PA) injection is administered with the aim of reopening the occluded intracranial arteries. In fact, patients with AAD undergoing t-PA injection have been reported to die from bleeding complications without any recognition of the dissection. These findings suggest that confirmation using carotid ultrasound, carotid MR angiography, and a D-dimer test is crucial and should be adopted in emergency departments. Chiaki Takahashi and Takashi Sasaki Copyright © 2015 Chiaki Takahashi and Takashi Sasaki. All rights reserved. Intradural Solitary Fibrous Tumor of the Lumbar Spine: A Distinctive Case Report Wed, 14 Jan 2015 13:25:41 +0000 Background. Solitary fibrous tumors are ubiquitous mesenchymal neoplasms of putative fibroblastic origin. They were originally described in the pleura but subsequently have been reported in many extraserosal sites. Solitary fibrous tumors may also occur in the meninges, central nervous system parenchyma, and spinal cord. Case. A 67-year-old male patient with progressive lower extremity weakness, urinary urgency, and sexual dysfunction has been admitted to our hospital. On his lumbar MRI, we detected an intradural lesion posterior to the L3 vertebral corpus. We resected the lesion by L3 total laminectomy. Immunohistological findings revealed strong and diffuse immunopositivity with vimentin, CD34, and bcl-2. Ki-67 proliferation index was 5–8%. We did not detect any recurrence 12 months after his operation. Conclusion. SFT is mostly seen in young and middle-aged patients and should be considered among differential diagnosis in cases suffering from pain, hypoesthesia, and urinary dysfunction. Gross total resection should be primary treatment. Tumors that have high Ki-67 labeling should be followed up for potential recurrences. Recep Basaran, Mustafa Kaksi, Mustafa Onoz, Ece Balkuv, and Aydin Sav Copyright © 2015 Recep Basaran et al. All rights reserved. Acupuncture Alleviated the Nonmotor Symptoms of Parkinson’s Disease including Pain, Depression, and Autonomic Symptoms Wed, 31 Dec 2014 12:30:15 +0000 A woman started to feel intractable pain on her lower legs when she was 76. At the age of 78, she was diagnosed as having Parkinson’s disease (PD). The leg pain was suspected to be a symptom of PD after eliminating other causes. The patient also suffered from nonmotor symptoms, depression, anxiety, hot flashes, and paroxysmal sweating. Though the patient had received pharmacotherapy including levodopa for 5 years, she still suffered from the nonmotor symptoms and was referred to our department. We treated her with acupuncture based on the Chinese traditional medicine and electroacupuncture five times per week. After the 2-week treatment, the assessment for the symptoms was as follows; visual analogue scale (VAS) score of the leg pain was 16 mm (70 mm, before), Hamilton’s rating scales for depression (HAM-D) score was 9 (18, before), timed 3 m Up and Go took 20 steps in 30 sec (24 steps in 38 sec, before), and the Movement Disorder Society-sponsored revision of the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) Part 1 score was 13 (21, before). Autonomic symptoms, hot flashes and paroxysmal sweating, were also alleviated. Acupuncture may be a good treatment modality for nonmotor symptoms in PD. Chifumi Iseki, Taiga Furuta, Masao Suzuki, Shingo Koyama, Keiji Suzuki, Tomoko Suzuki, Akiyo Kaneko, and Tadamichi Mitsuma Copyright © 2014 Chifumi Iseki et al. All rights reserved. Thyroid-Induced Worsening of Parkinsonian Tremor Resistant to Drugs and Subthalamic Nucleus Deep Brain Stimulation Wed, 31 Dec 2014 11:18:24 +0000 Introduction. Symptoms of both hypothyroidism and thyrotoxicosis can be easily overlooked in patients with Parkinson’s disease (PD). We report on a patient whose parkinsonian tremor worsened and proved refractory not only to common treatment, but also to deep brain stimulation (DBS). Case Presentation. A 61-year-old woman with advanced PD underwent bilateral subthalamic DBS, with an excellent outcome. Twenty-one months after the surgery, however, patient’s resting/postural tremor markedly worsened. There was a slight improvement for 1 month after repeated adjustments of DBS parameters, but then the tremor worsened again. Since even a minimal increase of the dose of dopaminergic drugs caused extremely severe dyskinesias, an anticholinergic drug biperiden and benzodiazepine clonazepam were introduced, what helped for another month. With the onset of severe diarrhoea, a laboratory workup was performed. Thyrotoxicosis was detected. During treatment with the antithyroid agent carbimazole, the parkinsonian tremor clearly improved within two weeks. Conclusion. A hyperthyroid state can markedly exaggerate all forms of tremor, as well as other types of movement disorders. This condition can be overlooked or masked by other symptoms. Therefore, if the tremor in a patient with PD gradually worsens and proves resistant to the usual treatment, examine the thyroid gland. Michal Minár and Peter Valkovič Copyright © 2014 Michal Minár and Peter Valkovič. All rights reserved. Varicella Zoster Virus Meningitis in a Young Immunocompetent Adult without Rash: A Misleading Clinical Presentation Mon, 29 Dec 2014 06:53:32 +0000 Meningitis caused by varicella zoster virus (VZV) is rare in healthy population. Predominantly immunocompromised patients are affected by reactivation of this virus with primary clinical features of rash and neurological symptoms. Here we report a young otherwise healthy man diagnosed with a VZV meningitis without rash. He complained of acute headache, nausea, and vomiting. The clinical examination did not show any neurological deficits or rash. Cerebrospinal fluid (CSF) analysis revealed a high leukocyte cell count of 1720 cells/µL and an elevated total protein of 1460 mg/L misleadingly indicating a bacterial infection. Further CSF analyses, including polymerase chain reaction (PCR) and detection of intrathecal synthesis of antibodies, showed a VZV infection. Clinical and CSF follow-up examinations proved the successful antiviral treatment. In conclusion, even young immunocompetent patients without rash might present with VZV meningitis. CSF examination is a key procedure in the diagnosis of CNS infections but in rare cases the standard values cell count and total protein might misleadingly indicate a bacterial infection. Thus, virological analyses should be considered even when a bacterial infection is suspected. Thomas Pasedag, Karin Weissenborn, Ulrich Wurster, Tina Ganzenmueller, Martin Stangel, and Thomas Skripuletz Copyright © 2014 Thomas Pasedag et al. All rights reserved. Epidermoid Causing Ischemic Stroke in the Brainstem Mon, 15 Dec 2014 00:10:33 +0000 Intracranial tumors may rarely cause stroke. We report an epidermoid cyst causing stroke in a pediatric patient. We have also reviewed the literature and pathogenesis of stroke caused by intracranial tumors. Raghvendra Ramdasi, Amit Mahore, Abhijeet Kulkarni, Vithal Rangarajan, Manoj Patil, and Juhi Kawale Copyright © 2014 Raghvendra Ramdasi et al. All rights reserved. Clinicoradiological Correlation of Macropsia due to Acute Stroke: A Case Report and Review of the Literature Tue, 09 Dec 2014 06:57:54 +0000 Dysmetropsia (macropsia, micropsia, teleopsia, or pelopsia) most commonly results from retinal pathologies, epileptic seizure, neoplastic lesions, viral infection, or psychoactive drugs. Vascular lesions are an uncommon cause of dysmetropsia. Vascular hemimicropsia, although rare, has been more frequently described in the literature, whereas hemimacropsia from acute ischemic injury is exceedingly rare. We describe a patient presenting in the emergency room (ER) with visual perception disturbances characterized by a distorted perception of the size of objects, compatible with left hemimacropsia. Magnetic resonance imaging (MRI) of the brain showed an acute occipitotemporal ischemic injury corresponding to the posterior cerebral artery (PCA) territory. The location of the lesion is consistent with previous case reports that suggest that hemimacropsia is associated with the occipitotemporal projection, which plays a decisive role in the visual identification of objects by interconnecting the striate, prestriate, and inferior temporal areas. The difference of our case as compared to previous case reports is that the lesion in our patient spared Brodmann area 17 (calcarine cortex) and therefore did not present symptoms of quadrantanopsia. Instead, the patient presented isolated hemimacropsia, therefore suggesting that the anatomical lesion causing hemimacropsia is located in the ventral portion of the occipitotemporal projection, more specifically Brodmann areas 18 (parastriate) and 19 (peristriate). Mayra Johana Montalvo and Muhib Alam Khan Copyright © 2014 Mayra Johana Montalvo and Muhib Alam Khan. All rights reserved. Motor Neuron Syndrome as a New Phenotypic Manifestation of Mutation 9185T>C in Gene MTATP6 Mon, 08 Dec 2014 12:22:32 +0000 Background. The mutation 9185T>C in ATP6 gene, associated with Leigh syndrome, was reported in only few families. Motor neuron disease (MND), both clinically and electrophysiologically, was not previously described in association with this mutation. Case Report. 33-year-old male, with family history of mitochondrial disease, presented with cognitive impairment, exercise intolerance, and progressive muscle weakness. Examination revealed global hypotonia, and proximal tetraparesis, without atrophy or fasciculation, pyramidal signs, or sensory symptoms. The laboratory findings revealed an increase of lactate and lactate/pyruvate ratio; electromyogram showed chronic neurogenic compromise; muscle biopsy was suggestive of spinal muscular atrophy and mitochondriopathy; genetic study of SMN1 was negative but detected a homoplasmic mutation 9185T>C in ATP6 gene. His younger sister, with the same mutation, had cognitive impairment, ataxia, and muscle weakness. EMG showed axonal peripheral neuropathy. Conclusion. This case is unique because of the benignity and the coexistence of clinical, neurophysiological, and pathological findings suggestive of MND that, although described in mitochondrial disease, have not yet been reported in association with 9185T>C mutation. The present case contributes to the expansion of the phenotypic expressions of this particular mutation. Marisa Brum, Cristina Semedo, Rui Guerreiro, and José Pinto Marques Copyright © 2014 Marisa Brum et al. All rights reserved. Intraventricular Hemorrhage after Epidural Blood Patching: An Unusual Complication Thu, 04 Dec 2014 11:50:25 +0000 The authors present two cases of intraventricular hemorrhage (IVH) believed to be a result of epidural blood patching. The first was a 71-year-old woman who had new onset of nontraumatic IVH on computed tomography (CT) scan after undergoing an epidural blood patch (EBP). This amount of intraventricular blood was deemed an incidental finding since it was of very small volume to account for her overall symptoms. The second patient, a 29-year-old woman, was found to have nontraumatic IVH three days after undergoing an EBP. This was seen on CT scan of the head for workup of pressure-like headaches, nausea, vomiting, and absence seizures. Conservative management was followed in both instances. Serial CT scan of the head in our first patient displayed complete resolution of her IVH. The second patient did not have follow-up CT scans because her overall clinical picture had improved significantly. This highlights a potential sequel of EBP that may be observed on CT scan of the head. In the event that IVH is detected, signs and symptoms of hydrocephalus should be closely monitored with the consideration for a future workup if warranted by the clinical picture. Mohammad Sorour, Khaled M. Krisht, and William T. Couldwell Copyright © 2014 Mohammad Sorour et al. All rights reserved. Cerebral Venous Thrombosis in Two Patients with Spontaneous Intracranial Hypotension Thu, 27 Nov 2014 08:24:26 +0000 Although few patients with spontaneous intracranial hypotension develop cerebral venous thrombosis, the association between these two entities seems too common to be simply a coincidental finding. We describe two cases of spontaneous intracranial hypotension associated with cerebral venous thrombosis. In one case, extensive cerebral venous thrombosis involved the superior sagittal sinus and multiple cortical cerebral veins. In the other case, only a right frontoparietal cortical vein was involved. Several mechanisms could contribute to the development of cerebral venous thrombosis in spontaneous intracranial hypotension. When spontaneous intracranial hypotension and cerebral venous thrombosis occur together, it raises difficult practical questions about the treatment of these two conditions. In most reported cases, spontaneous intracranial hypotension was treated conservatively and cerebral venous thrombosis was treated with anticoagulation. However, we advocate aggressive treatment of the underlying cerebrospinal fluid leak. M. C. Garcia-Carreira, D. Cánovas Vergé, J. Branera, M. Zauner, J. Estela Herrero, E. Tió, and G. Ribera Perpinyà Copyright © 2014 M. C. Garcia-Carreira et al. All rights reserved. Successful Thrombolysis despite Having an Incidental Unruptured Cerebral Aneurysm Mon, 24 Nov 2014 08:40:27 +0000 Purpose. To report a case of successful thrombolysis performed in a patient with an incidental unruptured intracranial aneurysm and review the literature. Case Report. Patient admitted for ischemic stroke due to left posterior cerebral artery occlusion, with an incidental right middle cerebral artery aneurysm, who underwent treatment with tissue plasminogen activator (rtPA) resulting in clinical improvement without complications. Conclusion. The presence of unruptured intracranial aneurysms is considered as a contraindication to thrombolysis, due to a potentially higher hemorrhagic risk of aneurysm rupture. Patients, otherwise, eligible for thrombolysis are usually excluded from receiving this emergent treatment, despite its potential benefits. A reevaluation of the strict exclusion criteria for thrombolysis in acute stroke patients should be considered. Diana Briosa e Gala, André Almeida, Nadine Monteiro, Ana Paiva Nunes, Patrícia Ferreira, Nuno Mendonça, Alexandre Amaral-Silva, and João Alcântara Copyright © 2014 Diana Briosa e Gala et al. All rights reserved. PML-IRIS during Fingolimod Diagnosed after Natalizumab Discontinuation Sun, 23 Nov 2014 12:41:54 +0000 Background. Natalizumab treatment is frequently discontinued and replaced by alternative medication in multiple sclerosis (MS) patients having a high risk of progressive multifocal leukoencephalopathy (PML). Case Presentation. We report a PML case that was missed on magnetic resonance imaging (MRI) at the time Natalizumab treatment was discontinued. The patient subsequently developed a PML-immune reconstitution inflammatory syndrome after the initiation of Fingolimod treatment, suggesting that immune reconstitution may occur even during Fingolimod induced lymphopenia. Conclusion. This report highlights the need for strict drug surveillance using MRI of Natalizumab-associated MS patients at the time of drug discontinuation and beyond. This is important with respect to pharmacovigilance purposes not only for Natalizumab, but also for alternative drugs used after Natalizumab discontinuation. J. Killestein, A. Vennegoor, A. E. L. van Golde, R. L. J. H. Bourez, M. L. B. Wijlens, and M. P. Wattjes Copyright © 2014 J. Killestein et al. All rights reserved. Menkes Disease Presenting with Epilepsia Partialis Continua Sun, 23 Nov 2014 07:59:53 +0000 Aim. We aim to describe a female patient with Menkes disease who presented with epilepsia partialis continua. Case Presentation. Seventeen-months-old Saudi infant was presented with repetitive seizures and was diagnosed to have epilepsia partialis continua. Discussion. Menkes disease (OMIM: 309400) is considered a rare, X-linked recessive neurodegenerative disorder resulting from a mutation in the gene coding for the copper transporting ATPase (ATP7A). Affected individuals usually present with kinky hair, skeletal changes, prolonged jaundice, hypothermia, developmental regression, decreased tone, spasticity, weakness, and therapy resistant seizures. Conclusion. Raising awareness of abnormal presentation of this rare disease may help in the control of seizures through subcutaneous copper supplementation. Tamer Rizk, Adel Mahmoud, Tahani Jamali, and Salah Al-Mubarak Copyright © 2014 Tamer Rizk et al. All rights reserved. Primary Neuroendocrine Tumor in Brain Thu, 20 Nov 2014 11:53:29 +0000 The incidence of brain metastases for neuroendocrine tumor (NET) is reportedly 1.5~5%, and the origin is usually pulmonary. A 77-year-old man presented to our hospital with headache and disturbance of specific skilled motor activities. Computed tomography (CT) showed a massive neoplastic lesion originating in the left temporal and parietal lobes that caused a mass edematous effect. Grossly, total resection of the tumor was achieved. Histological examination revealed much nuclear atypia and mitotic figures. Staining for CD56, chromogranin A, and synaptophysin was positive, indicating NET. The MIB-1 index was 37%. Histopathologically, the tumor was diagnosed as NET. After surgery, gastroscopy and colonoscopy were performed, but the origin was not seen. After discharge, CT and FDG-PET (fluoro-2-deoxy-d-glucose positron emission tomography) were performed every 3 months. Two years later we have not determined the origin of the tumor. It is possible that the brain is the primary site of this NET. To our knowledge, this is the first reported case of this phenomenon. Ryota Tamura, Yoshiaki Kuroshima, and Yoshiki Nakamura Copyright © 2014 Ryota Tamura et al. All rights reserved. Unpredicted Sudden Death due to Recurrent Infratentorial Hemangiopericytoma Presenting as Massive Intratumoral Hemorrhage: A Case Report and Review of the Literature Sun, 16 Nov 2014 07:35:44 +0000 Unpredicted sudden death arising from hemangiopericytoma with massive intracranial hemorrhage is quite rare. We encountered a patient with recurrent infratentorial hemangiopericytoma presenting as life-threatening massive intracerebral hemorrhage. A 43-year-old man who had undergone craniotomy for total resection of an infratentorial hemangiopericytoma 17 months earlier presented with morning headache and generalized convulsions. Computed tomography revealed a massive hematoma in the right infratentorial region causing tonsillar herniation and emergency surgery was performed to evacuate the hematoma. Histological findings revealed hemangiopericytoma with hemorrhage. Neurological status remained unimproved and brain death was confirmed postoperatively. Hemangiopericytoma presenting as massive hemorrhage is quite rare. Since the risk of life-threatening massive hemorrhage should be considered, careful postoperative long-term follow-up is very important to identify tumor recurrences, particularly in the posterior cranial fossa, even if the tumor is completely removed. Toshihide Tanaka, Naoki Kato, Yuzuru Hasegawa, and Yuichi Murayama Copyright © 2014 Toshihide Tanaka et al. All rights reserved. Myeloid Sarcoma: A Rare Case of an Orbital Mass Mimicking Orbital Pseudotumor Requiring Neurosurgical Intervention Sun, 16 Nov 2014 00:00:00 +0000 Objective. A rare case of myeloid sarcoma (MS), previously referred to as granulocytic sarcoma or chloroma, is presented. Representing a unique form of acute myeloid leukemia (AML), MS may rarely occur in adults. Even rarer, MS may occur as the initial presentation of AML. Methods. We report a singular and illustrative case of an orbital pseudotumor mimicking mass in a 65-year-old male as the initial presentation of AML. Results. Neurosurgical intervention was required to establish the definitive diagnosis via right modified orbitofrontozygomatic craniotomy as well as to decompress the optic canal, superior orbital fissure, and orbit. Conclusion. Postoperatively, he reported decreased pain and improvement of his vision. Further examination revealed decreased proptosis and improved extraocular mobility. Pathological findings demonstrated MS. We review the literature and discuss the neurosurgical relevance of MS as the initial presentation of AML. Christopher Payne, William C. Olivero, Bonnie Wang, Seong-Jin Moon, Arash Farahvar, Eric Chen, and Huan Wang Copyright © 2014 Christopher Payne et al. All rights reserved. Major Cognitive Changes and Micrographia following Globus Pallidus Infarct Wed, 12 Nov 2014 12:04:07 +0000 Importance. Globus pallidus (GP) lesions are well known to cause motor deficits but are less commonly—and perhaps not conclusively—associated with cognitive problems. Observations. We present a 45-year-old male with no significant neurological or psychological problems who after suffering a GP infarct was subsequently found to have substantial cognitive problems and micrographia. Formal neuropsychological testing was not possible due to lack of patient follow-up. Conclusions and Relevance. Despite the conflicting literature on the association of GP lesions and cognitive deficits, our patient demonstrated significant neuropsychological changes following his stroke. In addition, evidence of micrographia likely adds to the literature on the localization of this finding. Our case thus suggests that neuropsychological testing may be beneficial after GP strokes. Sarah Nelson, Hassanain Toma, Haley LaMonica, and Tinatin Chabrashvili Copyright © 2014 Sarah Nelson et al. All rights reserved. Osmotic Demyelination Syndrome as the Initial Manifestation of a Hyperosmolar Hyperglycemic State Sun, 09 Nov 2014 06:47:49 +0000 Osmotic demyelination syndrome (ODS) is a life-threatening demyelinating syndrome. The association of ODS with hyperosmolar hyperglycemic state (HHS) has been seldom reported. The aim of this study was to present and discuss previous cases and the pathophysiological mechanisms involved in ODS secondary to HHS. A 47-year-old man arrived to the emergency room due to generalized tonic-clonic seizures and altered mental status. The patient was lethargic and had a Glasgow coma scale of 11/15, muscle strength was 4/5 in both lower extremities, and deep tendon reflexes were diminished. Glucose was 838 mg/dL; serum sodium and venous blood gas analyses were normal. Urinary and plasma ketones were negative. Brain magnetic resonance revealed increased signal intensity on T2-weighted FLAIR images with restricted diffusion on the medulla and central pons. Supportive therapy was started and during the next 3 weeks the patient progressively regained consciousness and muscle strength and was able to feed himself. At 6-month follow-up, the patient was asymptomatic and MRI showed no residual damage. In conclusion, the association of ODS with HHS is extremely rare. The exact mechanism by which HHS produces ODS still needs to be elucidated, but we favor a rapid hypertonic insult as the most plausible mechanism. Karla Victoria Rodríguez-Velver, Analy J. Soto-Garcia, María Azucena Zapata-Rivera, Juan Montes-Villarreal, Jesús Zacarías Villarreal-Pérez, and René Rodríguez-Gutiérrez Copyright © 2014 Karla Victoria Rodríguez-Velver et al. All rights reserved. Stroke after Initiating IV Penicillin for Neurosyphilis: A Possible Jarisch-Herxheimer Reaction Wed, 05 Nov 2014 09:30:52 +0000 Introduction. Syphilis incidence has increased in the US in the last decade. Jarisch-Herxheimer reaction (JHR) is a well-documented adverse effect of penicillin treatment in syphilis. Stroke has not been reported as part of its phenomenology. Case Report. A 57-year-old man presented with worsening memory. His minimental status examination score was 14/30. Serum RPR test was positive and VDRL test in the CSF was reactive. Within six hours of first dose of IV crystalline penicillin G, he was found to have hemineglect and difficulty moving the left leg. MRI of the brain showed multiple acute ischemic strokes. Immediate MRA ruled out vascular occlusion. Penicillin treatment was stopped. Four hours later, he was found to be febrile and had two episodes of generalized tonic-clonic seizures. Conclusions. We report a case of confirmed neurosyphilis with no known modifiable stroke risk factors, who developed acute ischemic stroke and other constitutional symptoms consistent with JHR after IV penicillin. This is the first reported case in literature where an acute ischemic stroke can be attributed to Jarisch-Herxheimer reaction. Given an increase in incidence of syphilis in recent years, our case underlies the importance of keeping in mind potential catastrophic drug adverse reactions in neurosyphilis patients. Vineet Punia, Appaji Rayi, and Adithya Sivaraju Copyright © 2014 Vineet Punia et al. All rights reserved. Anton’s Syndrome due to Bilateral Ischemic Occipital Lobe Strokes Mon, 03 Nov 2014 07:16:50 +0000 We present a case of a patient with Anton’s syndrome (i.e., visual anosognosia with confabulations), who developed bilateral occipital lobe infarct. Bilateral occipital brain damage results in blindness, and patients start to confabulate to fill in the missing sensory input. In addition, the patient occasionally becomes agitated and talks to himself, which indicates that, besides Anton’s syndrome, he might have had Charles Bonnet syndrome, characterized by both visual loss and hallucinations. Anton syndrome, is not so frequent condition and is most commonly caused by ischemic stroke. In this particular case, the patient had successive bilateral occipital ischemia as a result of massive stenoses of head and neck arteries. Sanela Zukić, Osman Sinanović, Lejla Zonić, Renata Hodžić, Svjetlana Mujagić, and Edina Smajlović Copyright © 2014 Sanela Zukić et al. All rights reserved. Unique Presentation of Akinetic Mutism and Coexisting Thyroid Storm Relating to Stroke Tue, 28 Oct 2014 13:35:19 +0000 Akinetic mutism is described in various clinical presentations but typically is defined as a state wherein the patient appears awake but does not move or speak. It can be divided into two different subtypes; the most common subtypes depend on the lesion location, mesencephalic-diencephalic region, also called apathetic akinetic mutism (somnolent mutism), and those involving the anterior cingulate gyrus and adjacent frontal lobes called hyperpathic akinetic mutism. The pathway of akinetic mutism is believed to originate from circuits that link the frontal and subcortical structures. This case reports a 48-year-old African American female with bilateral anterior cerebral artery stroke and akinetic mutism with coexisting thyroid storm. This patient with bilateral anterior cerebral artery infarcts presented with characteristics that are typical for akinetic mutism such as having intact eye movements but an inability to respond to auditory or visual commands. With the incidence of bilateral anterior cerebral artery (ACA) ischemic stroke being rare and the incidence of akinetic mutism secondary to ischemic stroke even rarer, we suspect that this patient potentially had a unilateral occlusion of anomalous anterior cerebral vasculature. Mohankumar Kurukumbi, Thao Dang, Najeeb Crossley, Alice Esame, and Annapurni Jayam-Trouth Copyright © 2014 Mohankumar Kurukumbi et al. All rights reserved. New Onset Refractory Status Epilepticus in a Young Man with H1N1 Infection Tue, 14 Oct 2014 11:39:07 +0000 Objective. To report a case of refractory status epilepticus (SE) as an unusual early manifestation of H1N1 influenza infection. Introduction. H1N1 neurological complications have been reported and consist mainly of seizures or encephalopathy occurring in children. However, we only found a single report of an adult developing complex partial SE with H1N1 infection. Case Report. A 21-year-old previously healthy man was brought to the emergency room (ER) after a witnessed generalized tonic clonic seizure (GTCS). He was fully alert and afebrile upon ER arrival, but a second GTCS prompted treatment with Lorazepam and Fosphenytoin. The initial EEG showed diffuse slowing, but a repeat one requested as the patient failed to regain consciousness revealed recurrent focal seizures of independent bihemispheric origin, fulfilling the criteria for nonconvulsive SE. Chest X-ray, followed by chest CT scan, showed a left upper lobe consolidation. H1N1 infection was confirmed with PCR on bronchoalveolar lavage material. Despite aggressive treatment with Midazolam, Propofol, and multiple high dose antiepileptic drugs, the electrographic seizures recurred at every attempt to reduce the intravenous sedative drugs. The patient died two weeks after his initial presentation. Conclusion. H1N1 should be added to the list of rare causes of refractory SE, regardless of the patient’s age. Faisal Ibrahim and Naim Haddad Copyright © 2014 Faisal Ibrahim and Naim Haddad. All rights reserved. Clinical and Genetic Characteristics of Mexican Patients with Juvenile Presentation of Niemann-Pick Type C Disease Thu, 02 Oct 2014 10:43:47 +0000 Niemann-Pick type C disease (NPC) is a rare lysosomal disease with a protean presentation, ranging from a fatal neonatal course with visceromegaly to an adult presentation with only neurological or psychiatric symptomatology. In this report we describe the genetic and clinical characteristics of 3 Mexican patients from different families with juvenile presentation of NPC. Clinical examination, imaging of central nervous and gastrointestinal system, and EEG were performed. Genetic studies include sequencing and deletion/duplication analysis of NPC1 and NPC2 genes. All patients presented with cognitive impairment, ataxia, and supranuclear vertical gaze palsy; one case had gelastic cataplexy. Also they developed epilepsy and cortical atrophy and two patients had thinning of corpus callosum. The 3 patients were compound heterozygotes for NPC1 sequence variants, including 5 missense and 1 nonsense mutations: p.P1007A and p.F1087L in Case 1; p.Q921P and p.G992R in Case 2; and p.R348* and p.V1165M in case 3. Mexican patients with juvenile NPC presented with a variable clinical phenotype and compound heterozygosity. This suggests a relative high frequency of mutation carriers as it is reported for European population. Consequently, clinicians should consider NPC as a diagnosis possibility in any adolescent or young adult patient with juvenile dementia and/or ataxia, even in absence of gelastic cataplexy and supranuclear vertical gaze palsy. Raul E. Piña-Aguilar, Aurea Vera-Loaiza, Oscar F. Chacón-Camacho, Juan Carlos Zenteno, Lilia Nuñez-Orozco, and Yuritzi Santillán-Hernández Copyright © 2014 Raul E. Piña-Aguilar et al. All rights reserved. From Mild Ataxia to Huntington Disease Phenocopy: The Multiple Faces of Spinocerebellar Ataxia 17 Thu, 02 Oct 2014 10:03:12 +0000 Introduction. Spinocerebellar ataxia 17 (SCA 17) is a rare autosomal dominant cerebellar ataxia (ADCA) caused by a CAG/CAA expansion in the TBP gene, reported from a limited number of countries. It is a very heterogeneous ADCA characterized by ataxia, cognitive decline, psychiatric symptoms, and involuntary movements, with some patients presenting with Huntington disease (HD) phenocopies. The SCA 17 expansion is stable during parent-child transmission and intrafamilial phenotypic homogeneity has been reported. However, significant phenotypic variability within families has also been observed. Report of the Family. We presently report a Greek family with a pathological expansion of 54 repeats at the SCA 17 locus that displayed remarkable phenotypic variability. Among 3 affected members, one presented with HD phenocopy; one with progressive ataxia, dementia, chorea, dystonia, and seizures, and one with mild slowly progressive ataxia with minor cognitive and affective symptoms. Conclusions. This is the first family with SCA 17 identified in Greece and highlights the multiple faces of this rare disorder, even within the same family. Georgios Koutsis, Marios Panas, George P. Paraskevas, Anastasia M. Bougea, Athina Kladi, Georgia Karadima, and Elisabeth Kapaki Copyright © 2014 Georgios Koutsis et al. All rights reserved. Delayed Cerebral Radiation Necrosis after Neutron Beam Radiation of a Parotid Adenocarcinoma: A Case Report and Review of the Literature Tue, 30 Sep 2014 08:09:48 +0000 Cerebral radiation necrosis (CRN) is a well described possible complication of radiation for treatment of intracranial pathology. However, CRN as sequelae of radiation to extracranial sites is rare. Neutron beam radiation is a highly potent form of radiotherapy that may be used to treat malignant tumors of the salivary glands. This report describes a patient who underwent neutron beam radiation for a parotid adenocarcinoma and who developed biopsy-confirmed temporal lobe radiation necrosis thirty months later. This represents the longest time interval described to date, from initial neutron radiation for extracranial pathology to development of CRN. Two other detailed case studies exist in the literature and are described in this report. These reports as well as our patient’s case are reviewed, and additional recommendations are made to minimize the development of CRN after extracranial neutron beam radiation. Physicians should include the possible diagnosis of CRN in any patient with new neurologic signs or symptoms and a history of head and neck radiation that included planned fields extending to the base of the skull. Counseling of patients prior to neutron beam radiation should include potential neurologic complications associated with CRN and risks of treatment for CRN including neurosurgical intervention. Christopher S. Hong, Hamza N. Gokozan, José J. Otero, Michael Guiou, and J. Bradley Elder Copyright © 2014 Christopher S. Hong et al. All rights reserved.