Case Reports in Neurological Medicine The latest articles from Hindawi © 2017 , Hindawi Limited . All rights reserved. Multiple Sclerosis Presenting with Facial Twitching (Myokymia and Hemifacial Spasms) Sun, 17 Sep 2017 10:32:07 +0000 Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system. The etiology is insufficiently understood. Autoimmune, genetic, viral, and environmental factors have been hypothesized. MS is twice as common in women as in men between the ages of 20 and 50 years. There is no known cure for MS. Current medical treatment helps to prevent new attacks and improve function after an attack. MS is diagnosed by physical examination, diagnostic imaging, and examination of cerebral spinal fluid. The most common physical signs and symptoms of MS include constitutional symptoms, muscle weakness, motor and autonomic spinal cord symptoms, paresthesias, and vision changes. Here we present a case of MS diagnosed in a 33-year-old male with facial myokymia of left eyelid, which progressed to left hemifacial spasm. This is an unusual presentation for multiple sclerosis. An awareness of this presentation not only may lead to an earlier diagnosis in some patients but can be a sign of relapse in patients with established multiple sclerosis. Risha Hertz, James Espinosa, Alan Lucerna, and Doug Stranges Copyright © 2017 Risha Hertz et al. All rights reserved. Thoracic Synovial Cyst at the Th2-3 Level Causing Myelopathy Thu, 07 Sep 2017 00:00:00 +0000 Intraspinal synovial cyst is a rare cause of myelopathy. These cysts present most often in the lumbar and cervical parts of the spine but are more infrequent in the thoracic spine. We present a case of a 73-year-old man with an intraspinal, extradural synovial cyst at the Th2-3 level causing paraesthesia and weakness in the legs. A laminectomy and excision of the cyst were performed and the patient recovered fully. In the thoracic spine, synovial cysts are almost exclusively found in the lower part. Laminectomy, with excision, is the treatment of choice, although steroid injections have been described. Martin M. Sundskarð and Shahin Gaini Copyright © 2017 Martin M. Sundskarð and Shahin Gaini. All rights reserved. An Atypical Porencephalic Cyst Manifesting as a Simple Partial Seizure: A Case Report and Literature Review Tue, 05 Sep 2017 00:00:00 +0000 Background. Porencephaly is an extremely rare neurological disease characterized by the presence of solitary or multiple degenerative cerebrospinal fluid (CSF) cavities within the brain parenchyma. Case Report. We describe a case involving a 23-year-old male who presented with involuntary movements of the left upper limb of 6 months’ duration. A diagnosis of porencephaly was confirmed by magnetic resonance imaging (MRI). Conclusion. The rarity of occurrence and atypical presentation of such a lesion present a challenge to clinicians. Little is known about the pathogenesis and appropriate management of porencephaly. Further studies of the implications of porencephaly for neurodevelopment and behavior are needed. Abdulaziz Ibrahim Al Thafar, Abdullatif Sami Al Rashed, Bayan Abdullah Al Matar, Abdulaziz Mohammad Al-Sharydah, Abdulrahman Hamad Al-Abdulwahhab, and Sari Saleh Al-Suhibani Copyright © 2017 Abdulaziz Ibrahim Al Thafar et al. All rights reserved. Cerebellar Involvement in an Immunocompetent Patient Presenting with Progressive Multifocal Leukoencephalopathy Thu, 24 Aug 2017 10:03:06 +0000 Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by the JC virus, a polyomavirus that can be reactivated under certain immunosuppressive conditions, such as AIDS, immunomodulatory therapy, and haematological malignancies. However, a few cases of immunocompetent patients have been reported in which no immunodeficiency was present. We describe the case of an 83-year-old immunocompetent man who presented with severe cerebellar symptoms with an MRI scan suggestive of severe demyelinating disease. We were not able to identify any occult immunosuppression or malignancy in our patient. Rafael Garcia-Carretero and Blanca San Jose Montano Copyright © 2017 Rafael Garcia-Carretero and Blanca San Jose Montano. All rights reserved. Accidental Intrathecal Administration of Digoxin in an Elderly Male with End-Stage Renal Disease Tue, 22 Aug 2017 07:43:35 +0000 The systemic effects of digoxin toxicity have been well-known. However, there has been no case citing the effects of intrathecal digoxin in light of end-stage renal disease in the elderly. Here, we report on the case of the successful management of accidental intrathecal digoxin administration in an elderly male with end-stage renal disease. Claudia Martin, Kitae Kevin Park, and Antonio Liu Copyright © 2017 Claudia Martin et al. All rights reserved. Could Hallucinogens Induce Permanent Pupillary Changes in (Ab)users? A Case Report from New Zealand Thu, 17 Aug 2017 00:00:00 +0000 An eighteen-year-old female patient of the Caucasian ethnicity from Australasia presented with a persistently dilated pupil causing her discomfort and occasional burning sensation when she is outdoors due to oversensitivity to sunlight. However, her pupillary reaction to light (pupillary light reflex) was intact. The patient is a known user of psychedelic substances (entheogens) including LSD, NBOMe, psilocybin, and DMT. The condition affects both eyes to the same extent. Thorough medical, neurological, and radiological examinations, including an EEG and an MRI of the head and neck region, were completely normal. All these tests failed to detect any pathophysiological or anatomical abnormalities. The patient is a known case of chronic endogenous depression in association with attention deficit hyperactivity disorder, for which she is taking citalopram and Ritalin, respectively. There was neither a family history nor a similar congenital condition in her family. Ahmed Al-Imam Copyright © 2017 Ahmed Al-Imam. All rights reserved. Why It Is Not Always Anxiety: A Tough Diagnosis of Stiff Person Syndrome Mon, 14 Aug 2017 00:00:00 +0000 Anxiety disorder is a commonly used diagnosis that may mask underlying conditions. Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by progressive rigidity and painful muscle spasms affecting axial and lower extremity musculature. These episodes can be triggered by sudden movement, noise, or emotional stress, which may present as a psychiatric condition. We report the case of a 30-year-old female who presented with recurrent panic attacks with multiple prior hospital admissions for anxiety, rigidity, and difficulty in walking. Previous electroencephalogram (EEG) and brain and cervical spine magnetic resonance imaging (MRI) were unremarkable. She was empirically treated with diazepam and beta-blockers for SPS, which was confirmed by positive glutamic acid decarboxylase (GAD) antibodies. The patient’s symptoms became refractory to benzodiazepines and required steroids with intravenous immunoglobulin (IVIG). Her rigidity subsequently responded to plasmapheresis. In SPS, antibodies in the cerebrospinal fluid (CSF) most commonly target the GAD antigen on gamma-aminobutyric acid (GABA) neurons. The goal of treatment is to ameliorate symptoms and improve quality of life. Our case of SPS was masked as generalized anxiety disorder for at least six years since onset of symptoms. The criteria for both diagnoses may overlap as seen in this patient. Carmen Elena Cervantes, Hsien Lee Lau, Tina Ataian Binazir, Keith O. O’Brien, and Jonathan S. Cross Copyright © 2017 Carmen Elena Cervantes et al. All rights reserved. Cooccurrence of Chorea-Acanthocytosis and Mesial Temporal Sclerosis: A Possible Role of Caudate Nucleus Sun, 13 Aug 2017 08:55:30 +0000 Chorea-acanthocytosis (ChAc) is an orphan disease, caused by mutations on chromosome 9. Epileptic seizures of mesial temporal origin can be a predominant symptom. We report on a 29-year-old woman with ChAc and bilateral MTS. Previously, few patients with coexisting ChAc and MTS were reported. The underlying pathophysiology is unknown, and further studies are needed. Mehri Salari, Alexander C. Lehn, Masoud Etemadifar, and Seyed Amir Hejazi Copyright © 2017 Mehri Salari et al. All rights reserved. Neuromyelitis Optica in a Nepalese Man Tue, 08 Aug 2017 06:37:40 +0000 Background. Neuromyelitis optica is a severely disabling inflammatory disorder of the central nervous system of autoimmune etiology that mainly affects the optic nerves and spinal cord. Here, we present a case report detailing a patient with tingling and weakness of right upper and lower limbs who was neuromyelitis optica immunoglobulin G-positive. Case Presentation. A 46-year-old Nepalese man presented to the hospital with a history of tingling and weakness of right upper and lower limbs that developed over a period of two months. Clinical evaluation showed diminished power across all major muscle groups in the right upper and lower limbs. Magnetic resonance imaging of his cervical spine showed T1 iso- to hypointense signal and T2 hyperintense signal in central cervical spinal cord from first to sixth cervical level, probably suggestive of myelitis or demyelination. The patient was immediately started on intravenous methylprednisolone. The diagnosis of neuromyelitis optica was later confirmed with strongly positive neuromyelitis optica immunoglobulin G. Conclusion. In resource limited setting, in the absence of tests for neuromyelitis optica immunoglobulin G, treatment was started and the patient’s condition started to get better. Hence, early initiation of aggressive immunosuppressive treatment is essential in such cases. Yogesh Subedi, Utsav Joshi, Sanjeeb Sudarshan Bhandari, Ashbina Pokharel, and Ashbita Pokharel Copyright © 2017 Yogesh Subedi et al. All rights reserved. A Case Report of Isolated Bilateral Cerebral Peduncular Infarction Mon, 31 Jul 2017 10:37:45 +0000 Isolated bilateral cerebral peduncular infarctions (BCPI) presenting as acute pseudobulbar palsy are rarely reported and, to the best of our knowledge, most of the previous reports of BCPI were related to locked-in syndrome and disturbance of consciousness. Herein, we described a case of a 55-year-old man who presented with acute pseudobulbar palsy and mild tetraparesis, but preserved eye movements, with no consciousness disturbance. DWI revealed an acute infarction involving the central portion of the cerebral peduncle with a characteristic “traditional Chinese eight character” sign. The relationship between the infarcted range in the cerebral peduncle and the clinical manifestation was discussed in our report. Chenguang Zhou, Yuanhong He, Xiaorui Tian, Zhiwen Chao, Yinghui Zhu, Du Cheng, and Kui Li Copyright © 2017 Chenguang Zhou et al. All rights reserved. Bilateral Ganglion Cysts of the Ligamentum Flavum in the Cervical Spine Causing a Progressive Cervical Radiculomyelopathy and Literature Review Mon, 31 Jul 2017 07:11:39 +0000 Here we report a unique case of bilateral ganglion cysts originating from the ligamentum flavum in the cervical spine. Degenerative cysts of the ligamentum flavum are rare lesions, and most had been reported in the lumbar spine. Its occurrence in the cervical spine is extremely rare: only eight have been reported. A 66-year-old male patient presented with progressive paraparesis, pain, and paresthesia in his bilateral T1 dermatomes that had lasted for three weeks. Magnetic resonance imaging of the cervical spine demonstrated a well-demarcated cystic lesion in the bilateral dorsolateral aspects of the C7/T1 segment and significant compression of the cervical cord. All case reports of ganglion cysts of the cervical ligamentum flavum including the present one showed characteristic symptoms and signs of myelopathy such as paraparesis or quadriparesis associated with varying degrees of paresthesia or pain in the upper extremities. Ganglion cysts of the cervical ligamentum flavum are considered a cause of cervical radiculomyelopathy due to cervical intraspinal cystic lesions. Bilateral occurrence and associated subluxation of the involved cervical segments again support the degenerative pathogenesis of ganglion cysts of the ligamentum flavum in the cervical spine. Juneki Kim, Jin-gyu Choi, and Byung-chul Son Copyright © 2017 Juneki Kim et al. All rights reserved. A Case of Congenital Brainstem Oligodendroglioma: Pathology Findings and Review of the Literature Wed, 26 Jul 2017 13:00:00 +0000 Congenital and perinatal primary brain neoplasms are extremely rare. Brainstem neoplasms in the perinatal and neonatal period are typically of high-grade nature and have poor prognoses with survival rates of less than 2 years from diagnosis. Herein, we report an unusual case of congenital anaplastic oligodendroglioma that arose in the pons and was detected as diffuse pontine glioma on in utero imaging studies during prenatal evaluation at 26 weeks’ gestation. A male infant was delivered at 36.4 weeks of gestation via Cesarean section who developed progressive dyspnea shortly after birth. Magnetic resonance imaging (MRI) studies of his head showed the expansile, poorly demarcated mass in the pons with minimal heterogeneous enhancement and severe communicating hydrocephalus. Despite aggressive management, including dexamethasone treatment, the infant expired on the third day of postnatal life. On postmortem examination cut sections through the brainstem and cerebellum disclosed the neoplasm that infiltrated the entire pons, extended into the midbrain, medulla, cerebellar peduncles, and caudal diencephalon. Histological sections demonstrated an anaplastic oligodendroglioma infiltrating the pons, 4th ventricle, midbrain, medulla, cerebellar white matter, posterior thalamus, and occipital white matter. The pathological features of the lesion distinguish it from previous reports in which spontaneous regression of pontine gliomas occurred and argue in favor of establishing a tissue diagnosis to plan for aggressive versus conservative management. Stefan Kostadinov and Suzanne de la Monte Copyright © 2017 Stefan Kostadinov and Suzanne de la Monte. All rights reserved. Primary Angiitis of the Center Nervous System: A Clinical Challenge Diagnosed Postmortem Wed, 05 Jul 2017 08:26:56 +0000 Primary angiitis of the central nervous system (PACNS) is a rare vasculitis involving medium and small blood vessels of the brain, spinal cord, and meninges, without systemic involvement. The diffuse and patchy nature of its pathology is reflected by a wide spectrum of nonspecific clinical symptoms. Diagnosis is challenging due to lack of defined clinical criteria or specific imaging findings. Specific workup should be done only after exclusion of other etiologies, including infectious, neoplastic, toxic, and other vascular etiologies including systemic vasculitis. Given the fact that it is a patchy disease with 25% of the biopsies being falsely negative, treating physician should have a high index of suspicion despite negative initial neurovascular imaging and biopsy results. Once diagnosed, early treatment with immunosuppressive therapy is essential to avoid permanent neurologic damage. Herein, we are reporting a case of 66-year-old female patient who presented with insidious onset right-sided frontal headache. Her hospital course progressively worsened and family decision based on her wishes was to refer her to hospice and comfort care. Despite an extensive workup with advanced imaging techniques, no diagnosis was established until postmortem autopsy and histopathology confirmed primary angiitis of the central nervous system. Bayan Al Share, Ali Zakaria, Evan Hiner, Ziyad Iskenderian, and Nader Warra Copyright © 2017 Bayan Al Share et al. All rights reserved. Multimodal Imaging in a Patient with Hemidystonia Responsive to GPi Deep Brain Stimulation Tue, 04 Jul 2017 08:51:46 +0000 Background. Dystonia is a syndrome with varied phenomenology but our understanding of its mechanisms is deficient. With neuroimaging techniques, such as fiber tractography (FT) and magnetoencephalography (MEG), pathway connectivity can be studied to that end. We present a hemidystonia patient treated with deep brain stimulation (DBS). Methods. After 10 years of left axial hemidystonia, a 45-year-old male underwent unilateral right globus pallidus internus (GPi) DBS. Whole brain MEG before and after anticholinergic medication was performed prior to surgery. 26-direction diffusion tensor imaging (DTI) was obtained in a 3 T MRI machine along with FT. The patient was assessed before and one year after surgery by using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Results. In the eyes-closed MEG study there was an increase in brain coherence in the gamma band after medication in the middle and inferior frontal region. FT demonstrated over 50% more intense ipsilateral connectivity in the right hemisphere compared to the left. After DBS, BFMDRS motor and disability scores both dropped by 71%. Conclusion. Multimodal neuroimaging techniques can offer insights into the pathophysiology of dystonia and can direct choices for developing therapeutics. Unilateral pallidal DBS can provide significant symptom control in axial hemidystonia poorly responsive to medication. Christos Sidiropoulos, Susan M. Bowyer, Andrew Zillgitt, Peter A. LeWitt, Hassan Bagher-Ebadian, Esmaeil Davoodi-Bojd, Jason M. Schwalb, Richard Rammo, Ellen Air, and Hamid Soltanian-Zadeh Copyright © 2017 Christos Sidiropoulos et al. All rights reserved. Multiple Spontaneous Intracranial-Extracranial Arterial Dissections in a Patient with Osteogenesis Imperfecta Sun, 02 Jul 2017 08:10:07 +0000 A 40-year-old male with osteogenesis imperfecta (OI) was admitted to the hospital with an acute right monoparesis. Diffusion-weighted MRI showed infarction in the territory of the left anterior cerebral artery (ACA) and in the left posterior cerebral artery (PCA). In his vascular imaging, occlusion of the left vertebral artery (VA) starting from V2 segment was consistent with dissection and pseudoaneurysm in the right ACA. We presented this case because of the presence of spontaneous and simultaneous occurrence of both intracranial and extracranial arterial dissections in OI. Mehmet Kolukısa, Elif Gökçal, Azize Esra Gürsoy, Çiğdem Deniz, Ayşe Aralaşmak, and Talip Asil Copyright © 2017 Mehmet Kolukısa et al. All rights reserved. Differential Effects of Awake Glioma Surgery in “Critical” Language Areas on Cognition: 4 Case Studies Thu, 22 Jun 2017 00:00:00 +0000 Awake surgery with electrocorticosubcortical stimulation is the golden standard treatment for gliomas in eloquent areas. Preoperatively, mostly mild cognitive disturbances are observed with postoperative deterioration. We describe pre- and postoperative profiles of 4 patients (P1–P4) with gliomas in “critical” language areas (“Broca,” “Wernicke,” and the arcuate fasciculus) undergoing awake surgery to get insight into the underlying mechanism of neuroplasticity. Neuropsychological examination was carried out preoperatively (at T1) and postoperatively (at T2, T3). At T1, cognition of P1 was intact and remained stable. P2 had impairments in all cognitive domains at T1 with further deterioration at T2 and T3. At T1, P3 had impairments in memory and executive functions followed by stable recovery. P4 was intact at T1, followed by a decline in a language test at T2 and recovery at T3. Intraoperatively, in all patients language positive sites were identified. Patients with gliomas in “critical” language areas do not necessarily present cognitive disturbances. Surgery can either improve or deteriorate (existing) cognitive impairments. Several factors may underlie the plastic potential of the brain, for example, corticosubcortical networks and tumor histopathology. Our findings illustrate the complexity of the underlying mechanism of neural plasticity and provide further support for a “hodotopical” viewpoint. Djaina Satoer, Elke De Witte, Marion Smits, Roelien Bastiaanse, Arnaud Vincent, Peter Mariën, and Evy Visch-Brink Copyright © 2017 Djaina Satoer et al. All rights reserved. Differential Diagnosis and Management of Incomplete Locked-In Syndrome after Traumatic Brain Injury Wed, 14 Jun 2017 08:43:57 +0000 Locked-in syndrome (LIS) is a rare diagnosis in which patients present with quadriplegia, lower cranial nerve paralysis, and mutism. It is clinically difficult to differentiate from other similarly presenting diagnoses with no standard approach for assessing such poorly responsive patients. The purpose of this case is to highlight the clinical differential diagnosis process and outcomes of a patient with LIS during acute inpatient rehabilitation. A 32-year-old female was admitted following traumatic brain injury. She presented with quadriplegia and mutism but was awake and aroused based on eye gaze communication. The rehabilitation team was able to diagnose incomplete LIS based on knowledge of neuroanatomy and clinical reasoning. Establishing this diagnosis allowed for an individualized treatment plan that focused on communication, coping, family training, and discharge planning. The patient was ultimately able to discharge home with a single caregiver, improving her quality of life. Continued evidence highlights the benefits of intensive comprehensive therapy for those with acquired brain injury such as LIS, but access is still limited for those with a seemingly poor prognosis. Access to a multidisciplinary, specialized team provides opportunity for continued assessment and individualized treatment as the patient attains more medical stability, improving long-term management. Lauren Surdyke, Jennifer Fernandez, Hannah Foster, and Pamela Spigel Copyright © 2017 Lauren Surdyke et al. All rights reserved. Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary Involvement Tue, 23 May 2017 07:06:01 +0000 Polyostotic sclerosing histiocytosis, also known as Erdheim-Chester disease (ECD), is a rare form of non-Langerhans histiocytosis. ECD has wide clinical spectrums which mainly affect skeletal, neurological, dermatological, retroperitoneal, cardiac, and pulmonary manifestations. Here we describe a case of ECD in a 45-year-old female who presented initially with bilateral knee pain and homonymous superior quadrantanopia progressed to ophthalmoplegia and complete visual loss of the left eye over a period of one year. Plain X-ray of both knees showed bilateral patchy sclerosis of the distal femur and upper parts of the tibiae. Initial brain magnetic resonance imaging (MRI) showed bilateral enhancing masses in the temporal lobes anterior to the temporal horns, thickening of the pituitary stalk, partially empty sella, and involvement of the left cavernous sinus one year later. Our case is a peculiar case of ECD initially presented with unilateral homonymous superior quadrantanopia due to involvement of the visual apparatus in the mesial temporal lobe which progressed to unilateral ophthalmoplegia and total visual loss secondary to involvement of the cavernous sinus. Thus, the diagnosis of ECD should be kept in mind in the presence of bilateral bone sclerotic lesions. Roaa Ridha Amer, Sara Mohammed Qubaiban, and Eman Abdulkarim Bakhsh Copyright © 2017 Roaa Ridha Amer et al. All rights reserved. Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids, with Cranial and Caudal Extension Sun, 14 May 2017 09:59:52 +0000 A 23-year-old lady presented with vertigo and imbalance in walking, blurring of vision, diplopia, and headache, in addition to numbness in the lower limbs over a period of six days. On examination patient had nystagmus, ataxia, positive Romberg test, and hyperreflexia. MRI examination of the brain and spinal cord showed evidence of faint bright signal intensity foci in T2/FLAIR involving bilateral cerebral hemispheres, subcortical deep white matter, bilateral thalami, posterior pons and left brachium pontis, and basal ganglia, with small nodular enhancement that aligned along curvilinear structures; those lesions also were apparent along the spinal cord at multiple levels. The clinical and radiological features suggested CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) syndrome. Symptoms improved dramatically with high dose oral corticosteroids. Our report addresses the radiological and clinical pattern of a case of CLIPPERS rhombencephalitis, with added superior and inferior extension to involve the brain and spinal cord, which is to emphasize the importance of raising the awareness of this disease and the combined role of radiologist and physicians for the diagnosis of this potentially treatable entity, responsive to glucocorticosteroid immunosuppression. Mahmood Mubasher, Aseel Sukik, Ahmed Hassan El Beltagi, and Ali Rahil Copyright © 2017 Mahmood Mubasher et al. All rights reserved. Uncommon Etiology for Seizure: Cerebral Hyperperfusion Syndrome Wed, 10 May 2017 00:00:00 +0000 Cerebral hyperperfusion syndrome (CHS) is a rare life-threatening complication of carotid endarterectomy (CEA) and carotid artery stenting (CAS) for carotid artery stenosis. The incidence varies between 0 and 3%, depending on the severity of the stenosis, perioperative hypertension, and contralateral carotid stenosis. This case report reports a 53-year-old female patient presenting with decreased alertness and multiple tonic-clonic seizures, in the background of bilateral CEA. She was found to have bilateral carotid stenosis. Her left CEA was performed three months prior and right CEA was four days prior to her current presentation with seizures. After bilateral CEA, the imaging showed extensive pathologic process involving primarily the subcortical white matter and overlying cortex, more on the right cerebral hemisphere. On follow-up six weeks later, she reported no recurrent seizures and imaging showed decrease in abnormal signal intensity of the grey and white matter. This was indicative of near complete resolution of hyperperfusion damage. CHS is a rare complication due to the loss of autoregulation of the cerebrovascular system and increased blood flow status after bilateral CEA. This case is reported because of a rare and unique presentation of seizures in the background of bilateral CEA. Mohankumar Kurukumbi, Ahn Truong, and Naghemeh Pirsaharkhiz Copyright © 2017 Mohankumar Kurukumbi et al. All rights reserved. Impaired Emotion Recognition after Left Hemispheric Stroke: A Case Report and Brief Review of the Literature Sun, 07 May 2017 06:40:20 +0000 Impaired recognition of emotion after stroke can have important implications for social competency, social participation, and consequently quality of life. We describe a case of left hemispheric ischemic stroke with impaired recognition of specifically faces expressing fear. Three months later, the patient’s spouse reports that the patient was irritable and slow in communication, which may be caused by the impaired emotion recognition. The case is discussed in relation to the literature concerning emotion recognition and its neural correlates. Our case supports the notion that emotion recognition, including fear recognition, is regulated by a network of interconnected brain regions located in both hemispheres. We conclude that impaired emotion recognition is not uncommon after stroke and can be caused by dysfunction of this emotion-network. Hugo P. Aben, Yael D. Reijmer, Johanna M. A. Visser-Meily, Jacoba M. Spikman, Geert Jan Biessels, Paul L. M. de Kort, and PROCRAS Study Group Copyright © 2017 Hugo P. Aben et al. All rights reserved. Chronic Neuropsychological Sequelae in a Patient with Nontumorous Anti-NMDA-Receptor Encephalitis Wed, 19 Apr 2017 00:00:00 +0000 Anti-N-methyl-D-aspartate receptor encephalitis is a neurological, autoimmune disorder tightly conceptualized only as recently as the mid-2000s. It presents itself in a combination of psychiatric, neurological, and autonomic features. We observe a unique case with probable earlier episode (prior to the mid-2000s conceptualization of the disease) and a later relapse, accompanying a comprehensive neuropsychological profile tracked after the relapse and subsequent improvement. Neurocognitive findings revealed residual frontal deficits with mood changes even in the state after plasmapheresis. This case is the first to describe posttreatment cognition in anti-NMDAR encephalitis after probable serial autoimmune episodes. Dong Y. Han, Lisa M. Koehl, Aarti Patel, Zhengqiu Zhou, Sarah Phillips, and Siddharth Kapoor Copyright © 2017 Dong Y. Han et al. All rights reserved. Open-Label Fosmetpantotenate, a Phosphopantothenate Replacement Therapy in a Single Patient with Atypical PKAN Sun, 16 Apr 2017 00:00:00 +0000 Objective. Pantothenate kinase-associated neurodegeneration (PKAN) is an autosomal recessive disorder with variable onset, rate of progression, and phenotypic expression. Later-onset, more slowly progressive PKAN often presents with neuropsychiatric as well as motor manifestations that include speech difficulties, progressive dystonia, rigidity, and parkinsonism. PKAN is caused by biallelic PANK2 mutations, a gene that encodes pantothenate kinase 2, a regulatory enzyme in coenzyme A biosynthesis. Current therapeutic strategies rely on symptomatic relief. We describe the treatment of the first, later-onset PKAN patient with oral fosmetpantotenate (previously known as RE-024), a novel replacement therapy developed to bypass the enzymatic defect. Methods. This was an open-label, uncontrolled, 12-month treatment with fosmetpantotenate of a single patient with a later-onset, moderately severe, and slowly progressive form of PKAN. Results. The patient showed improvement in all clinical parameters including the Unified Parkinson’s Disease Rating Scale (UPDRS), Barry-Albright Dystonia Scale, the EuroQol five-dimensional three-level (EQ-5D-3L) scale, timed 25-foot walk test, and electroglottographic speech analysis. Fosmetpantotenate was well-tolerated with only transient liver enzyme elevation which normalized after dose reduction and did not recur after subsequent dose increases. Conclusions. Fosmetpantotenate showed promising results in a single PKAN patient and should be further studied in controlled trials. Yiolanda-Panayiota Christou, George A. Tanteles, Elena Kkolou, Annita Ormiston, Kostas Konstantopoulos, Maria Beconi, Randall D. Marshall, Horacio Plotkin, and Kleopas A. Kleopa Copyright © 2017 Yiolanda-Panayiota Christou et al. All rights reserved. SSRI Facilitated Crack Dancing Tue, 11 Apr 2017 07:48:26 +0000 Choreoathetoid movement secondary to cocaine use is a well-documented phenomenon better known as “crack dancing.” It consists of uncontrolled writhing movements secondary to excess dopamine from cocaine use. We present a 32-year-old male who had been using cocaine for many years and was recently started on paroxetine, a selective serotonin reuptake inhibitor (SSRI) for worsening depression four weeks before presentation. He had been doing cocaine every 2 weeks for the last three years and had never “crack danced” before this episode. The authors have conducted a thorough literature review and cited studies that suggest “crack dancing” is associated with excess dopamine. There has never been a documented case report of an SSRI being linked with “crack dancing.” The authors propose that the excess dopaminergic effect of the SSRI lowered the dopamine threshold for “crack dancing.” There is a communication with the Raphe Nucleus and the Substantia Nigra, which explains how the SSRI increases dopamine levels. This is the first documented case of an SSRI facilitating the “crack dance.” Ravi Doobay, Lili Sun, Amish Shah, Pardeep Masuta, and Zachary Shepherd Copyright © 2017 Ravi Doobay et al. All rights reserved. A Horned Viper Bite Victim with PRES Mon, 10 Apr 2017 06:22:14 +0000 Neurological complications of snake bites have been well documented in the literature as neuromuscular paralysis and cerebrovascular complications; posterior reversible encephalopathy syndrome was rarely described. A 23-year-old lady presented near full term of her pregnancy with a horned snake Cerastes cerastes bite; after successful delivery she started complaining of altered mental status and visual disturbance with ulceration over the site of the snake bite. On admission, the patient had Glasgow Coma Score of 12, blood pressure 130/80 mmHg, temperature 38°C, sinus tachycardia at 120 beats per minute, severe dehydration, and reduction in visual acuity to “hand motion” in both eyes with poor light projection and sluggish pupillary reactions. CT brain was not conclusive; MRI revealed features of PRES. Treatment was mostly supportive within one week; the patient regained consciousness; visual disturbance, however, persisted. This patient as well as the few previously described cases highlights PRES as a possible complication of snake bites. Ahmed Mustafa Ibrahim, Tarek Talaat ElSefi, Maha Ghanem, Akram Muhammad Fayed, and Nesreen Adel Shaban Copyright © 2017 Ahmed Mustafa Ibrahim et al. All rights reserved. Watershed Infarct in Beta-Thalassemia Major Patient Mon, 27 Mar 2017 00:00:00 +0000 Background. The mechanism of stroke in beta-thalassemia was reported previously as cardioembolic and hypercoagulable state. However, there is no report of watershed infarct in beta-thalassemia anemia. Method. We present an adult β-thalassemia major patient with manifest asymptomatic chronic left carotid occlusion who suffered watershed infarct. Result. In the presence of asymptomatic chronic left internal carotid occlusion, we assumed that severe anemia (hemoglobin = 3) at admission leads to watershed infarct. Conclusion. Watershed infarct seems to be the cause of stroke in cases of β-thalassemia major with severe anemia. Blood transfusion can be applied in the setting of acute brain ischemia in such high risk patients. Behnaz Ansari, Mohammad Saadatnia, and Ali Asghar Okhovat Copyright © 2017 Behnaz Ansari et al. All rights reserved. Asymptomatic Severe Vagal and Sympathetic Cardiac Denervation in Holmes-Adie’s Syndrome Mon, 27 Mar 2017 00:00:00 +0000 A 40-year-old woman was found to have bilateral Adie’s pupils and generalized muscle stretch areflexia. She did not have orthostatic hypotension but, in an ECG strip in the office, she appeared to have an almost fixed heart rate. We thus studied the heart rate variability (HRV) and the systolic blood pressure variability (SBPV) in supine and standing position and also during rhythmic breathing. We found a decreased HRV in the time domain with very low standard deviation in supine and standing position and during rhythmic breathing. HRV in the frequency domain was low with a decrease in the absolute power of HF and LF and a decrease in the sympathovagal balance in supine and standing positions. SBPV in the time and frequency domains was found to be normal. This patient with Holmes-Adie syndrome had an asymptomatic severe loss of HRV and a preserved SBPV. The global decrease in the HRV in the time and frequency domains indicated that she had both vagal and sympathetic cardiac denervation, whereas the preserved SBPV suggested normal innervation of the blood vessels. B. Estañol, R. C. Callejas-Rojas, S. Cortés, R. Martínez-Memije, O. Infante-Vázquez, and G. Delgado-García Copyright © 2017 B. Estañol et al. All rights reserved. Levodopa Responsive Parkinsonism in Patients with Hemochromatosis: Case Presentation and Literature Review Thu, 23 Mar 2017 07:48:08 +0000 Hemochromatosis is an autosomal recessive disorder which leads to abnormal iron deposition in the parenchyma of multiple organs causing tissue damage. Accumulation of iron in the brain has been postulated to be associated with several neurodegenerative diseases including Parkinson’s disease. The excess iron promotes Parkin and α-synuclein aggregation in the neurons. Excess iron has also been noted in substantia nigra on MRI especially using susceptibility weighted imaging in patients with Parkinson’s disease. We present a case of a young male with alleles for both C282Y and H63D who presented with signs of Parkinsonism and demonstrated significant improvement with levodopa treatment. Tarun Girotra, Abhimanyu Mahajan, and Christos Sidiropoulos Copyright © 2017 Tarun Girotra et al. All rights reserved. Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis Tue, 14 Mar 2017 00:00:00 +0000 Central pontine myelinolysis (CPM) is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Common clinical manifestations of CPM include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees; however, coma, “locked-in” syndrome, or death can occur in most severe cases. Rarely, CPM presents with neuropsychiatric manifestations, such as personality changes, acute psychosis, paranoia, hallucinations, or catatonia, typically associated with additional injury to the brain, described as extrapontine myelinolysis (EPM). We present a patient with primarily neuropsychiatric manifestations of CPM, in the absence of focal neurologic deficits or radiographic extrapontine involvement. A 51-year-old female without significant medical history presented with dizziness, frequent falls, diarrhea, generalized weakness, and weight loss. Physical examination showed no focal neurological deficits. Laboratory data showed severe hyponatremia, which was corrected rather rapidly. Subsequently, the patient developed symptoms of an acute psychotic illness. Initial brain magnetic resonance imaging (MRI) was unremarkable, although a repeat MRI two weeks later revealed changes compatible with CPM. This case demonstrates that acute psychosis might represent the main manifestation of CPM, especially in early stages of the disease, which should be taken into consideration when assessing patients with acute abnormalities of sodium metabolism. Mangala Gopal, Melvin Parasram, Harsh Patel, Chike Ilorah, and Hrachya Nersesyan Copyright © 2017 Mangala Gopal et al. All rights reserved. Corrigendum to “Fatal Vertebral Artery Injury in Penetrating Cervical Spine Trauma” Sun, 12 Mar 2017 00:00:00 +0000 Chadi Tannoury and Anthony Degiacomo Copyright © 2017 Chadi Tannoury and Anthony Degiacomo. All rights reserved.