Case Reports in Neurological Medicine The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Unusual Presentation of Unilateral Isolated Probable Lyme Optic Neuritis Wed, 03 Feb 2016 08:37:51 +0000 Optic neuritis (ON) is one of the most common manifestations of central nervous system involvement caused by various etiologies. Lyme ON is an exceedingly rare ocular manifestation of Lyme disease (LD) and only a few cases have been published in the literature. Lyme ON is very rare but should be included in the differential diagnosis in unexplained cases, particularly in Lyme endemic areas. Careful and detailed examination and investigation are warranted to make the diagnosis. We report this case to increase awareness of clinicians to include Lyme disease in differential diagnosis of ON for unexplained cases of ON. Herein we present a unique case with a unilateral ON caused by LD along with pre- and posttreatment findings and literature review. Ahmet Z. Burakgazi and Carl S. Henderson Copyright © 2016 Ahmet Z. Burakgazi and Carl S. Henderson. All rights reserved. A New Concept for Carotid Artery Stenting: Coating the Atherosclerotic Plaque by Covered Stent before Bare Stent Implantation Tue, 02 Feb 2016 09:39:11 +0000 In carotid artery stenting (CAS) procedures, distal embolism, periprocedural stent thrombosis, and 30-day stroke due to the plaque fragmentation and protrusion caused by stent implantation and balloon dilation are frequent complications. In this technical case report, a case is presented of extracranial carotid artery stenosis treated with a covered stent and subsequent implantation of a bare stent. In addition, the possibility is discussed that this new technique prevents the distal microembolic complications, periprocedural stent thrombosis, and 30-day stroke of extracranial CAS. Erol Akgul Copyright © 2016 Erol Akgul. All rights reserved. Isolated Intracranial Rosai-Dorfman Disease Tue, 02 Feb 2016 07:28:53 +0000 Background. Rosai-Dorfman disease (RDD) is a benign histiocytic proliferative disorder of unknown etiology. This rare condition commonly causes massive cervical lymphadenopathy. Intracranial RDD without any nodal involvement is extremely rare. Case Report. A young Bangladeshi male complained of bilateral complete blindness with left sided deafness for about three years. There was no lymphadenopathy. MRI and CT scan of brain suggested an inflammatory/neoplastic (?meningioma) lesion located at left parasellar region which extended frontally to encircle both optic nerves and also to left prepontine area. Histopathologically the lesion was diagnosed as RDD. The patient was treated with steroid and significant clinical improvement observed. Conclusion. The prognosis of intracranial RDD is not poor. It can be treated with surgery with or without corticosteroids, chemotherapy, and so forth. But as the condition is extremely rare and often misdiagnosed, the clinician, radiologist, and histopathologist should have a suspicion in their mind about the possibility of RDD. Md. Taufiq, Abul Khair, Ferdousy Begum, Shabnam Akhter, Md. Shamim Farooq, and Mohammed Kamal Copyright © 2016 Md. Taufiq et al. All rights reserved. Isolated Cranial Nerve-III Palsy Secondary to Perimesencephalic Subarachnoid Hemorrhage Mon, 01 Feb 2016 09:53:45 +0000 We describe isolated cranial nerve-III palsy as a rare clinical finding in a patient with perimesencephalic subarachnoid hemorrhage. In this unusual case, the patient presented with complete cranial nerve-III palsy including ptosis and pupillary involvement. Initial studies revealed subarachnoid hemorrhage in the perimesencephalic, prepontine, and interpeduncular cisterns. Angiographic studies were negative for an intracranial aneurysm. The patient’s neurological deficits improved with no residual deficits on follow-up several months after initial presentation. Our case report supports the notion that patients with perimesencephalic subarachnoid hemorrhage have an excellent prognosis. Our report further adds a case of isolated cranial nerve-III palsy as a rare initial presentation of this type of bleeding, adding to the limited body of the literature. Justin R. Abbatemarco and Hussam A. Yacoub Copyright © 2016 Justin R. Abbatemarco and Hussam A. Yacoub. All rights reserved. Chronic Encapsulated Expanding Thalamic Hematoma Associated with Obstructive Hydrocephalus following Radiosurgery for a Cerebral Arteriovenous Malformation: A Case Report and Literature Review Sun, 24 Jan 2016 09:56:02 +0000 Chronic encapsulated intracerebral hematoma is a unique type of intracerebral hematoma accompanied by a capsule that is abundant in fragile microvasculature occasionally causing delayed regrowth. A 37-year-old man who had undergone radiosurgery for an arteriovenous malformation (AVM) causing intracerebral hematoma in the left parietal lobe presented with headache, vomiting, and progressive truncal ataxia due to a cystic lesion that had been noted in the left thalamus, leading to progressive obstructive hydrocephalus. He underwent left frontal craniotomy via a transsylvian fissure approach, and the serous hematoma was aspirated. The hematoma capsule was easy to drain and was partially removed. Pathological findings demonstrated angiomatous fibroblastic granulation tissue with extensive macrophage invasion. The concentration of vascular endothelial growth factor (VEGF) was high in the hematoma (12012 pg/mL). The etiology and pathogenesis of encapsulated hematoma are unclear, but the gross appearance and pathological findings are similar to those of chronic subdural hematoma. Based on the high concentration of VEGF in the hematoma, expansion of the encapsulated hematoma might have been caused by the promotion of vascular permeability of newly formed microvasculature in the capsule. Jun Takei, Toshihide Tanaka, Yohei Yamamoto, Akihiko Teshigawara, Satoru Tochigi, Yuzuru Hasegawa, and Yuichi Murayama Copyright © 2016 Jun Takei et al. All rights reserved. Optic Nerve Sheath Meningioma Masquerading as Optic Neuritis Tue, 19 Jan 2016 14:07:07 +0000 Optic neuritis is a common presentation of demyelinating disorders such as multiple sclerosis. It typically presents with acute painful monocular vision loss, whereas chronic optic neuropathy can be caused by compressive lesions along the anterior visual pathway, genetic, toxic, or nutritional causes. We report an unusual presentation mimicking optic neuritis, which was subsequently diagnosed as optic nerve sheath meningioma (ONSM). Misinterpretation of white matter lesions on MRI of brain and the failure to image the optic nerves at the time of acute loss of vision led to the misdiagnosis of optic neuritis in this case. A comprehensive accurate history and ordering the appropriate imaging modality remain paramount in diagnosing progressive visual deterioration. R. Alroughani and R. Behbehani Copyright © 2016 R. Alroughani and R. Behbehani. All rights reserved. Clival Ectopic Pituitary Adenoma Mimicking a Chordoma: Case Report and Review of the Literature Wed, 13 Jan 2016 13:16:23 +0000 Background. Purely ectopic pituitary adenomas are exceedingly rare. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Case Presentation. A 41-year-old male presented with an incidental clival lesion presumed to be a chordoma. On MRI it involved the entire clivus, extended laterally to the petroclival junction, and invaded the cavernous sinuses bilaterally, encasing both internal carotid arteries, without direct extension into the sella. Intraoperatively, it was clear that the tumor originated from the clivus and that the sellar dura was completely intact. Frozen-section pathology was consistent with a pituitary adenoma. Immunostaining was positive for synaptophysin and prolactin with a low Ki-67 index, suggestive of a prolactinoma. Additional immunohistochemical stains seen in chordomas (EMA, S100, and Brachyury) and other metastatic tumors were negative. A postoperative endocrine workup revealed an elevated serum prolactin of 881.3 ng/mL (normal < 20). Conclusions. In conclusion, it is crucial to maintain an extensive differential diagnosis when evaluating a patient with a clival lesion. Ectopic clival pituitary adenomas, although rare, may warrant an endocrinological workup preoperatively as the majority may respond to medical treatment. Constantine L. Karras, Isaac Josh Abecassis, Zachary A. Abecassis, Joseph G. Adel, Esther N. Bit-Ivan, Rakesh K. Chandra, and Bernard R. Bendok Copyright © 2016 Constantine L. Karras et al. All rights reserved. Cognitive Impairments Preceding and Outlasting Autoimmune Limbic Encephalitis Sun, 10 Jan 2016 14:06:42 +0000 Mild cognitive impairment (MCI) can be the initial manifestation of autoimmune limbic encephalitis (ALE), a disorder that at times presents a diagnostic challenge. In addition to memory impairment, clinical features that might suggest this disorder include personality changes, agitation, insomnia, alterations of consciousness, and seizures. Once recognized, ALE typically responds to treatment with immune therapies, but long-term cognitive deficits may remain. We report two cases of patients with MCI who were ultimately diagnosed with ALE with antibodies against the voltage gated potassium channel complex. Months after apparent resolution of their encephalitides, both underwent neuropsychological testing, which demonstrated persistent cognitive deficits, primarily in the domains of memory and executive function, for cases 1 and 2, respectively. A brief review of the literature is included. Robert Gross, Jennifer Davis, Julie Roth, and Henry Querfurth Copyright © 2016 Robert Gross et al. All rights reserved. Angiographic Evidence of a Purely Pial Bihemispheric Intracranial Hemangiopericytoma Tue, 05 Jan 2016 14:12:58 +0000 Background. Classification of hemangiopericytoma (HPC) has evolved to a mesenchymal, nonmeningothelial grade two or three neoplasm according to the World Health Organization; however its blood supply has always been defined by dual origin, pial and dural contribution. Case Description. We present the case of a patient with an intracranial HPC with only pial vascular supply. Angiography confirmed the lack of dural supply to this bihemispheric intracranial mass. Subsequent histologic examination confirmed the diagnosis of hemangiopericytoma. Angiographic evidence here is atypical of the natural history of hemangiopericytomas with dual vascular supply and was critical in the decision-making towards surgical resection without tumor embolization. Conclusion. Data presented suggests the lack of dural vascular supply alone does not rule out the diagnosis of hemangiopericytoma. Nathaniel Stetson, Sudhakar Vadivelu, Jiang Y. Li, Avi Setton, and David J. Chalif Copyright © 2016 Nathaniel Stetson et al. All rights reserved. Two Uncommon Causes of Guillain-Barré Syndrome: Hepatitis E and Japanese Encephalitis Tue, 22 Dec 2015 14:10:12 +0000 We are presenting two cases of Guillain-Barré syndrome where it is preceded by hepatitis E virus (HEV) and Japanese encephalitis virus (JEV) infection, respectively. Our first case is a forty-three-year-old nondiabetic, nonhypertensive female who was initially diagnosed with acute HEV induced viral hepatitis and subsequently developed acute onset ascending quadriparesis with lower motor neuron type of bilateral facial nerve palsies and respiratory failure. Second patient was a 14-year-old young male who presented with meningoencephalitis with acute onset symmetric flaccid paraparesis. After thorough investigations it was revealed as a case of Japanese encephalitis. Our idea of reporting these two cases is to make ourselves aware about this potential complication of these two common infections. Dhrubajyoti Bandyopadhyay, Vijayan Ganesan, Cankatika Choudhury, Suvrendu Sankar Kar, Parthasarathi Karmakar, Vivek Choudhary, Prasun Banerjee, Debarati Bhar, Adrija Hajra, Manas Layek, and Sabyasachi Mukhopadhyay Copyright © 2015 Dhrubajyoti Bandyopadhyay et al. All rights reserved. Stroke as an Unusual First Presentation of Lyme Disease Wed, 16 Dec 2015 13:13:56 +0000 Introduction. Lyme neuroborreliosis is a nervous system infection caused by spirochete Borrelia burgdorferi with diverse neurological complications. Stroke due to cerebral vasculitis is a rare consequence of neuroborreliosis and has been described in just a few case reports. Case Presentation. Here, we report the case of a 43-year-old patient who presented with discrete left-sided hemiparesis and amnestic cognitive impairment. Brain magnetic resonance imaging showed a thalamic infarct, and serological and cerebrospinal fluid (CSF) tests confirmed the diagnosis of active neuroborreliosis. The antibiotic treatment with intravenous ceftriaxone for three weeks led to an improvement of the symptoms and remarkable regression of radiological findings, but not to full recovery of the amnestic cognitive disorder. Conclusion. Lyme neuroborreliosis should be suspected in patients with cerebrovascular events without obvious risk factors, especially those living in endemic areas such as northern Europe or those who have been exposed to ticks and those with clinical or radiological findings suggesting Lyme neuroborreliosis, in order to establish the diagnosis and start a proper antibiotic therapy. Mohamad Almoussa, Angelika Goertzen, Barbara Fauser, and Christoph W. Zimmermann Copyright © 2015 Mohamad Almoussa et al. All rights reserved. Subacute Subdural Hematoma in a Patient with Bilateral DBS Electrodes Tue, 08 Dec 2015 13:23:02 +0000 Subdural hematomas (SDH) in patients with implanted deep brain stimulating (DBS) electrodes are rare. Only a handful of cases have been reported in the literature. No clear management guidelines exist regarding the management of the hematoma and the existing electrodes. We describe a 68-year-old female with bilateral DBS electrodes, who presented with acute, severe hemiparesis due to a large subacute SDH with associated electrode displacement. Urgent hematoma evacuation reversed the hemiparesis; the electrodes were left undisturbed. Brain reexpansion occurred promptly. The patient was able to benefit from stable DBS therapies within 3 weeks of hematoma evacuation, maintained at 1.5-year follow-up. The case highlights that despite relative electrode migration due to a subdural hematoma, the electrodes may not require revision during initial hematoma evacuation or in a delayed fashion. Timely hematoma evacuation, coupled with brain reexpansion, may be adequate for the electrode to travel back to its original position and effect reasonable DBS therapies. Ha Son Nguyen and Peter A. Pahapill Copyright © 2015 Ha Son Nguyen and Peter A. Pahapill. All rights reserved. Paraspinal and Extensive Epidural Abscess: The Great Masqueraders of Abdominal Pain Sun, 06 Dec 2015 11:58:45 +0000 Paraspinal and epidural abscesses are rare conditions often diagnosed later in the disease process that can have significant morbidity and mortality. Predisposing risk factors include diabetes, human immunodeficiency virus, intravenous drug abuse, and previous history of spinal surgery or injection. They can threaten the spinal cord by compressive effect, leading to sensory motor deficits and ultimately paralysis and death. Diagnosis may be a challenge due to the delayed presentation of nonspecific back pain or radicular pain such as chest pain or abdominal pain. We present a rare case on a patient with periumbilical pain, constipation, and urinary retention who was ultimately diagnosed with a paraspinal abscess extending into the epidural space from T1 to S2. He underwent decompressive laminectomy with incision and drainage of the abscesses. The patient made an excellent recovery postoperatively, and repeat magnetic resonance imaging at six weeks showed resolution of the abscess. Andrew Chu, Thu Thu Aung, and Uday Shankar Copyright © 2015 Andrew Chu et al. All rights reserved. The Myotonic Plot Thickens: Electrical Myotonia in Antimuscle-Specific Kinase Myasthenia Gravis Thu, 03 Dec 2015 11:58:00 +0000 Electrical myotonia is known to occur in a number of inherited and acquired disorders including myotonic dystrophies, channelopathies, and metabolic, toxic, and inflammatory myopathies. Yet, electrical myotonia in myasthenia gravis associated with antibodies against muscle-specific tyrosine kinase (MuSK) has not been previously reported. We describe two such patients, both of whom had a typical presentation of proximal muscle weakness with respiratory failure in the context of a significant electrodecrement in repetitive nerve stimulation. In both cases, concentric needle examination revealed electrical myotonia combined with myopathic motor unit morphology and early recruitment. These findings suggest that MuSK myasthenia should be included within the differential diagnosis of disorders with electrical myotonia. Marcus Magnussen, Ioannis Karakis, and Taylor B. Harrison Copyright © 2015 Marcus Magnussen et al. All rights reserved. Herpes Simplex Viral Encephalitis Masquerading as a Classic Left MCA Stroke Thu, 03 Dec 2015 08:16:02 +0000 Objective. Stroke is a clinical diagnosis, with a history and physical examination significant for acute onset focal neurological symptoms and signs, often occurring in patients with known vascular risk factors and is frequently confirmed radiographically. Case Report. A 79-year-old right-handed woman, with a past medical history of hypertension, hyperlipidemia, and prior transient ischemic attack (TIA), presented with acute onset global aphasia and right hemiparesis, in the absence of fever or prodrome. This was initially diagnosed as a proximal left middle cerebral artery (MCA) stroke. However, CT perfusion failed to show evidence of reduced blood volume, and CT angiogram did not show evidence of a proximal vessel occlusion. Furthermore, MRI brain did not demonstrate any areas of restricted diffusion. EEG demonstrated left temporal periodic lateralized epileptiform discharges (PLEDs). The patient was empirically loaded with a bolus valproic acid and started on acyclovir, both intravenously. CSF examination demonstrated a pleocytosis and PCR confirmed the diagnosis of herpes simplex viral encephalitis (HSVE). Conclusions. HSVE classically presents in a nonspecific fashion with fever, headache, and altered mental status. However, acute focal neurological signs, mimicking stroke, are possible. A high degree of suspicion is required to institute appropriate therapy and decrease morbidity and mortality associated with HSVE. Peter A. Abdelmalik, Timothy Ambrose, and Rodney Bell Copyright © 2015 Peter A. Abdelmalik et al. All rights reserved. Culture Negative Listeria monocytogenes Meningitis Resulting in Hydrocephalus and Severe Neurological Sequelae in a Previously Healthy Immunocompetent Man with Penicillin Allergy Tue, 01 Dec 2015 06:12:49 +0000 A previously healthy 74-year-old Caucasian man with penicillin allergy was admitted with evolving headache, confusion, fever, and neck stiffness. Treatment for bacterial meningitis with dexamethasone and monotherapy ceftriaxone was started. The cerebrospinal fluid showed negative microscopy for bacteria, no bacterial growth, and negative polymerase chain reaction for bacterial DNA. The patient developed hydrocephalus on a second CT scan of the brain on the 5th day of admission. An external ventricular catheter was inserted and Listeria monocytogenes grew in the cerebrospinal fluid from the catheter. The patient had severe neurological sequelae. This case report emphasises the importance of covering empirically for Listeria monocytogenes in all patients with penicillin allergy with suspected bacterial meningitis. The case also shows that it is possible to have significant infection and inflammation even with negative microscopy, negative cultures, and negative broad range polymerase chain reaction in cases of Listeria meningitis. Follow-up spinal taps can be necessary to detect the presence of Listeria monocytogenes. Shahin Gaini, Gunn Hege Karlsen, Anirban Nandy, Heidi Madsen, Debes Hammershaimb Christiansen, and Sanna á Borg Copyright © 2015 Shahin Gaini et al. All rights reserved. Wernicke’s Encephalopathy, Wet Beriberi, and Polyneuropathy in a Patient with Folate and Thiamine Deficiency Related to Gastric Phytobezoar Mon, 30 Nov 2015 06:44:49 +0000 Background. Wernicke’s encephalopathy (WE) is an acute neurological disorder resulting from thiamine deficiency. It is mainly related to alcohol abuse but it can be associated with other conditions such as gastrointestinal disorders. This vitamin deficiency can also present with cardiovascular symptoms, called “wet beriberi.” Association with folate deficit worsens the clinical picture. Subject. A 70-year-old man with gastric phytobezoar presented with gait instability, dyspnoea, chest pain associated with right heart failure and pericarditis, and folate deficiency. Furosemide was administered and cardiac symptoms improved but he soon developed vertiginous syndrome, nystagmus, diplopia, dysmetria, and sensitive and motor deficit in all four limbs with areflexia. Results. A cerebral magnetic resonance imaging (MRI) showed typical findings of WE. He was immediately treated with thiamine. Neurological symptoms improved in a few days and abnormal signals disappeared in a follow-up MRI two weeks later. Conclusion. Patients with malabsorption due to gastrointestinal disorders have an increased risk of thiamine deficiency, and folate deficiency can make this vitamin malabsorption worse. An established deficiency mainly shows neurological symptoms, WE, or rarely cardiovascular symptoms, wet beriberi. Early vitamin treatment in symptomatic patients improves prognosis. We recommend administration of prophylactic multivitamins supplements in patients at risk as routine clinical practice. Nuria Huertas-González, Virgilio Hernando-Requejo, Zaida Luciano-García, and Juan Luis Cervera-Rodilla Copyright © 2015 Nuria Huertas-González et al. All rights reserved. Transient Global Amnesia with Reversible White Matter Lesions: A Variant of Posterior Reversible Encephalopathy Syndrome? Mon, 30 Nov 2015 06:43:55 +0000 Transient global amnesia (TGA) is a self-limited disease characterized by isolated amnesia, which resolves within 24 h. In contrast, posterior reversible encephalopathy syndrome (PRES) is a potentially life-threatening disease that usually presents with seizures, altered mental status, headache, and visual disturbances. It is characterized by reversible vasogenic edema that predominantly involves the parieto-occipital subcortical white matter as shown by neuroimaging studies. To date, there have been no reported cases of PRES with a clinical course resembling TGA. Here we report the case of a 58-year-old woman who presented with isolated amnesia and headache. On admission, her blood pressure was 187/100 mmHg. She had complete anterograde amnesia and slight retrograde amnesia without other neurological findings. After the treatment of her hypertension, the amnesia resolved within 24 h. Although the initial magnetic resonance image (MRI) was almost normal, the fluid attenuation inversion recovery (FLAIR) images of the MRI on the next day revealed several small foci of high intensity areas in the fronto-parieto-occipital subcortical white matter, presumed to be vasogenic edema in PRES. The lesions disappeared one month later. This case suggests that PRES can mimic the clinical course of TGA. PRES should be considered in the differential diagnosis for TGA. Tomoki Nakamizo, Ippei Tsuzuki, and Takashi Koide Copyright © 2015 Tomoki Nakamizo et al. All rights reserved. Cerebral Venous Air Embolism due to a Hidden Skull Fracture Secondary to Head Trauma Thu, 26 Nov 2015 14:19:19 +0000 Cerebral venous air embolism is sometimes caused by head trauma. One of the paths of air entry is considered a skull fracture. We report a case of cerebral venous air embolism following head trauma. The patient was a 55-year-old man who fell and hit his head. A head computed tomography (CT) scan showed the air in the superior sagittal sinus; however, no skull fractures were detected. Follow-up CT revealed a fracture line in the right temporal bone. Cerebral venous air embolism following head trauma might have occult skull fractures even if CT could not show the skull fractures. Ai Hosaka, Tetsuto Yamaguchi, Fumiko Yamamoto, and Yasuro Shibagaki Copyright © 2015 Ai Hosaka et al. All rights reserved. Susac’s Syndrome: A Case with Unusual Cardiac Vestibular and Imaging Manifestations Wed, 25 Nov 2015 07:20:04 +0000 Susac’s syndrome (SS) is a disease of the microvasculature of the retina, brain, and inner ear. We describe a patient with unusual manifestations of SS with possible involvement of the brainstem, cardiac arrhythmia, and MRI findings lacking the characteristic lesions found in Susac’s syndrome. Yaron River, Avi Shupak, Beatrice Tiosano, Vika Danilov, and Itzhak Braverman Copyright © 2015 Yaron River et al. All rights reserved. Artery of Percheron Infarction as an Unusual Cause of Korsakoff’s Syndrome Tue, 24 Nov 2015 09:57:25 +0000 The Korsakoff syndrome is defined as “an abnormal mental state in which memory and learning are affected out of all proportion to other cognitive functions in an otherwise alert and responsive patient.” Confabulation refers to false or erroneous memories arising, not deliberately, in the context of a neurological amnesia and is often thought of as pathognomonic of the Korsakoff syndrome. Although the exact pathophysiology is unknown, various studies have identified brain lesions in the thalami, mammillary bodies, and frontal cortex. We report a case of a 68-year-old male presenting with acute altered mental status on July 16, 2015. The neuropsychological dysfunctions included prominent Korsakoff’s syndrome, which became apparent when the altered mental status resolved. Amnesia was accompanied by prominent confabulation, disorientation, and lack of insight into his own disability. Neuroradiological data indicated that the intralaminar and dorsomedial nuclei in bilateral thalami were infarcted by occlusion of the artery of Percheron. We believe that ours is one of few reported cases of Korsakoff syndrome in a patient with infarction involving the territory of the artery of Percheron. We conclude that bilateral thalamic lesions could cause Korsakoff’s syndrome and the intralaminar and dorsomedial nuclei might be important structures in the pathogenesis of confabulation. Yongxing Zhou, Derrick Fox, Abhishek Anand, Amal Elhaj, Arushi Kapoor, Faranak Najibi, Han Kim, Roger Weir, and Annapurni Jayam-Trouth Copyright © 2015 Yongxing Zhou et al. All rights reserved. Clinical Significance of Human Metapneumovirus in Refractory Status Epilepticus and Encephalitis: Case Report and Review of the Literature Wed, 18 Nov 2015 13:54:59 +0000 Encephalitis is a complex neurological disease that is associated with significant morbidity and mortality, and the etiology of the disease is often not identified. Human metapneumovirus (hMPV) is a common cause of upper and lower respiratory tract infections in children. Few reports are available showing possible involvement of hMPV in development of neurologic complications. Here, we describe an infant, the youngest case in literature, with refractory status epilepticus and severe encephalitis in whom hMPV was detected in respiratory samples and review diagnostic workup of patient with encephalitis. Aysel Vehapoglu, Ozden Turel, Turkan Uygur Sahin, Nurettin Onur Kutlu, and Akın Iscan Copyright © 2015 Aysel Vehapoglu et al. All rights reserved. Longitudinal Follow-Up of Mirror Movements after Stroke: A Case Study Sun, 15 Nov 2015 08:40:07 +0000 Mirror movement (MM), or visible involuntary movements of a relaxed hand during voluntary fine finger movements of an activated opposite hand, can be observed in the hand that is on the unaffected side of patients with stroke. In the present study, we longitudinally examined the relationship between voluntary movement of the affected hand and MM in the unaffected hand in a single case. We report a 73-year-old woman with a right pontine infarct and left moderate hemiparesis. MM was observed as an extension movement of the unaffected right index finger during extension movement of the affected left index finger. The affected right index movement was found to increase, while MM of the unaffected left index finger was observed to decrease with time. These results indicate that the assessment of MM might be useful for studying the process of motor recovery in patients with stroke. Hiroyuki Ohtsuka, Daisuke Matsuzawa, Daisuke Ishii, and Eiji Shimizu Copyright © 2015 Hiroyuki Ohtsuka et al. All rights reserved. Changes over Time in Intracranial Air in Patients with Cerebral Air Embolism: Radiological Study in Two Cases Thu, 12 Nov 2015 11:54:57 +0000 Cerebral air embolism can be easily identified on computed tomography (CT) scans. However, changes in the distribution and amount of intracranial air are not well known. We report two patients with cerebral air embolism and present imaging findings on the serial changes in the intracranial air. We thought that the embolic source was venous in one patient because CT showed air inflow in cortical veins in the bilateral frontal areas, reflecting air buoyancy. In the other patient, CT showed air inflow into not only the cortical veins but also the bilateral cerebral hemispheres and we thought this to be a paradoxical cerebral air embolism. We found that intracranial air can be promptly absorbed and while cerebral infarcts due to air are clearly visualized on diffusion-weighted images (DWI), the air may rapidly disappear from images. In patients with suspected cerebral air embolism whose CT findings show no intracranial air, DWI should be performed because it may reveal cerebral infarction due to cerebral air embolism. Yoko Kaichi, Shingo Kakeda, Yukunori Korogi, Tomohisa Nezu, Shiro Aoki, Masayasu Matsumoto, Makoto Iida, and Kazuo Awai Copyright © 2015 Yoko Kaichi et al. All rights reserved. Fatal Vertebral Artery Injury in Penetrating Cervical Spine Trauma Thu, 12 Nov 2015 10:56:24 +0000 Study Design. This case illustrates complications to a vertebral artery injury (VAI) resulting from penetrating cervical spine trauma. Objectives. To discuss the management of both VAI and cervical spine trauma after penetrating gunshot wound to the neck. Summary of Background Data. Vertebral artery injury following cervical spine trauma is infrequent, and a unilateral VAI often occurs without neurologic sequela. Nevertheless, devastating complications of stroke and death do occur. Methods. A gunshot wound to the neck resulted in a C6 vertebral body fracture and C5–C7 transverse foramina fractures. Neck CT angiogram identified a left vertebral artery occlusion. A cerebral angiography confirmed occlusion of the left extracranial vertebral artery and patency of the remaining cerebrovascular system. Following anterior cervical corpectomy and stabilization, brainstem infarction occurred and resulted in death. Results. A fatal outcome resulted from vertebral artery thrombus propagation with occlusion of the basilar artery triggering basilar ischemia and subsequent brainstem and cerebellar infarction. Conclusions. Vertebral artery injury secondary to cervical spine trauma can lead to potentially devastating neurologic sequela. Early surgical stabilization, along with anticoagulation therapy, contributes towards managing the combination of injuries. Unfortunately, despite efforts, a poor outcome is sometimes inevitable when cervical spine trauma is coupled with a VAI. Chadi Tannoury and Anthony Degiacomo Copyright © 2015 Chadi Tannoury and Anthony Degiacomo. All rights reserved. Lymphoma Presenting as Acute-Onset Dysphagia Mon, 09 Nov 2015 07:48:30 +0000 A 61-year-old man with recent Bell’s palsy developed acute vocal cord paralysis causing severe dysphagia. CSF analysis showed elevated protein and a normal cell count; contrast-enhanced MRI of the brain was normal. He was treated with IVIG for a presumed bulbar-variant AIDP and gradually improved. Six months later, the patient developed rapidly progressive hearing loss and vestibular dysfunction. Repeat MRI revealed bilateral enhancement of the eighth cranial nerves and a hypercellular mass in the left temporal lobe. Biopsy of the mass confirmed the diagnosis of diffuse large B-cell lymphoma. Lymphomatous invasion of the cranial nerves should be considered in cases of relapsing cranial neuropathies. Daniel B. Simmons and Andrew W. Bursaw Copyright © 2015 Daniel B. Simmons and Andrew W. Bursaw. All rights reserved. Three Siblings with Prader-Willi Syndrome: Brief Review of Sleep and Prader-Willi Syndrome Tue, 03 Nov 2015 09:54:31 +0000 Prader-Willi syndrome (PWS) is a genetic disorder characterized by short stature, mental retardation, hypotonia, functionally deficient gonads, and uncontrolled appetite leading to extreme obesity at an early age. Patients with this condition require multidisciplinary medical care, which facilitates a significant improvement in quality of life. PWS is the first human disorder to be attributed to genomic imprinting. Prevalence varies in the literature, ranging from 1 in 8,000 in the Swedish population to 1 in 54,000 in the United Kingdom. Rarely, the genetic mechanism responsible for Prader-Willi syndrome can be inherited. We report a highly unique case of three siblings who share this condition. This report describes a case of two brothers and one half sister with PWS. All three siblings have sleep-related complaints. The sister died at the age of 24 years in her sleep, with the cause of death reported as obstructive sleep apnea. The outcome was positive in both of the brothers’ cases as a result of professional medical care and specific tailored recommendations implemented by their mother. A review of the relevant literature vis-à-vis sleep and PWS is provided. Arina Bingeliene, Colin M. Shapiro, and Sharon A. Chung Copyright © 2015 Arina Bingeliene et al. All rights reserved. GABAB Encephalitis: A Fifty-Two-Year-Old Man with Seizures, Dysautonomia, and Acute Heart Failure Mon, 02 Nov 2015 13:45:36 +0000 Autoantibodies to the -aminobutyric acid receptor, subtype B (), are a known cause of limbic encephalitis. The spectrum of clinical manifestations attributable to this antibody is not well defined at the present time. Here we present a case of encephalitis presenting with encephalopathy, status epilepticus, dysautonomia, and acute heart failure. To our knowledge, heart failure and dysautonomia have not yet been reported with this syndrome. Matthew C. Loftspring, Eric Landsness, Lindsey Wooliscroft, Robert Rudock, Sally Jo, and Kevin R. Patel Copyright © 2015 Matthew C. Loftspring et al. All rights reserved. Central Pontine Myelinolysis with Minimal Hyponatremia in the Setting of AIDS Thu, 29 Oct 2015 12:40:22 +0000 Central pontine myelinolysis (CPM) is classically attributed to overly rapid correction of profound hyponatremia. However, there are case reports of this disease in the setting of normal serum sodium or minimal hyponatremia. These cases have been hypothesized to be secondary to other metabolic disturbances such as hyperglycemia or hypophosphatemia. Eunatremic CPM has also been described in patients with advanced acquired immune deficiency syndrome (AIDS). The mortality risk in this special population is significantly higher than those with hyponatremia-associated CPM, but the mechanisms are unclear. We discuss a case of a man with AIDS who developed CPM with minimal hyponatremia and no other metabolic disturbances. Common variables within this population, such as hypoalbuminemia and lymphoma, are discussed as potential factors contributing to the pathophysiology. Reporting these atypical cases is crucial to our understanding of how to prevent future cases. Joseph M. Carrington, Galo Sanchez, and Jennifer L. Berkeley Copyright © 2015 Joseph M. Carrington et al. All rights reserved. Brain Herniation in Neurofibromatosis with Dysplasia of Occipital Bone and Posterior Skull Base Tue, 27 Oct 2015 08:39:55 +0000 A 22-year-old female, a known case of neurofibromatosis 1 (NF1), presented with a congenital swelling in the left occipital region. She had developed recent onset dysphagia and localized occipital headache. Neuroradiology revealed a left occipital meningoencephalocele and a left parapharyngeal meningocele. This was associated with ventriculomegaly. She was advised on cranioplasty along with duraplasty which she denied. She agreed to a lumbar-peritoneal shunt. She described a dramatic improvement in her symptoms following the lumbar-peritoneal shunt. Occipital dysplasias, though uncommon, have been reported in the literature. We review this case and its management and discuss relevant literature on occipital dysplasias in NF1. Vithal Rangarajan, Amit Mahore, Manoj Patil, Prashant Sathe, Amol Kaswa, Sandeep Gore, Pralhad Dharurkar, and Juhi Kawale Copyright © 2015 Vithal Rangarajan et al. All rights reserved.