Case Reports in Neurological Medicine The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. Extensive Cortical Diffusion Restriction in a 50-Year-Old Female with Hyperammonemic Encephalopathy and Status Epilepticus Thu, 24 Apr 2014 16:43:06 +0000 Comorbid hyperammonemic encephalopathy (HE) and status epilepticus (SE) leading to extensive cortical diffusion restriction (CDR) on MRI have not been previously reported. We describe a patient with HE who subsequently developed provoked SE. Sequential MRIs demonstrated a progressive CDR that involved the entire bilateral supratentorial cortex, thalami, and basal ganglia, resulting in death from cerebral edema and brain herniation. Diffuse CDR is most frequently seen after hypotension or hypoxia, which our patient did not experience. Such findings have also been described in both HE and SE (Milligan et al. (2009), Chatzikonstantinou et al. (2011), U-King-Im et al. (2011), and Bindu et al. (2009)), but not to the extent seen in our patient. Additionally, our patient had distinct radiologic features of both disease processes, suggesting a cumulative effect. The diagnosis of HE and SE in the setting of extensive CDR should not be missed and could lead to improved outcomes for two progressive, malignant, and treatable illnesses that can be easily overlooked. Adam de Havenon, Kris French, and Safdar Ansari Copyright © 2014 Adam de Havenon et al. All rights reserved. Extensive VZV Encephalomyelitis without Rash in an Elderly Man Wed, 23 Apr 2014 11:33:07 +0000 Introduction. Varicella zoster virus (VZV) encephalomyelitis with cranial nerve involvement is rare. Characteristically it is preceded by a rash and primarily presents in the immunocompromised. The spectrum of VZV neurologic disease is extensive and it is not uncommon to present without rash. We report the case of an elderly otherwise immunocompetent patient who presented with diverse manifestations of VZV CNS infection all occurring without rash. Case Report. A 78-year-old man presented with 1 week of progressive paraparesis and sensory loss, malaise, and fevers. MRI of the neuraxis demonstrated numerous enhancing lesions: intramedullary, leptomeningeal, pachymeningeal, and cranial nerves. Cerebrospinal fluid (CSF) showed a white blood cell count of 420/μL with elevated protein (385 mg/dL). CSF VZV qualitative PCR was positive and CSF VZV immunofluorescence assay detected IgM antibody, confirming the diagnosis of VZV encephalomyelitis. Clinical and radiological improvement was observed after intravenous acyclovir treatment. Conclusion. This is a rare report of an immunocompetent patient with extensive VZV encephalomyelitis. We highlight the importance of considering this diagnosis even in the absence of the characteristic rash, and the potential risk of premature discontinuation of antiviral therapy once HSV has been excluded. Prompt recognition and treatment can dramatically reduce morbidity and mortality in patients. Karen Lynch, Prakhar Agarwal, Anu Paranandi, Susan Hadley, and Mithila Vullaganti Copyright © 2014 Karen Lynch et al. All rights reserved. Prolonged Toxic Encephalopathy following Accidental 4-Aminopyridine Overdose Thu, 17 Apr 2014 00:00:00 +0000 Background. 4-Aminopyridine (4-AP) is a drug that is used to improve motor fatigue in patients suffering from multiple sclerosis (MS). Medication error can occur, as commercial preparation may not be available in some countries. Case Presentation. A 58-year-old woman with progressive MS presented with status epilepticus. She was receiving 4-AP for more than 3 years. The symptoms started soon after the ingestion of a single pill that was supposed to contain 10 mg 4-AP, but further investigations revealed that each pill had been inadvertently prepared with an 100 mg 4-AP concentration. The patient was admitted to the intensive care unit (ICU) for appropriate management (orotracheal intubation, sedation, and antiepileptic drugs). The first electroencephalogram (EEG) showed abundant irregular spike-waves on the left central regions. Neurological condition gradually improved from day 7, while the EEG did not reveal any more electrical seizures but was still consistent with toxic encephalopathy. The patient stayed in the ICU until day 13. At discharge from the rehabilitation ward (2.5 months later), the patient had not yet recovered her previous cognitive and functional condition. Conclusion. A single 100 mg 4-AP accidental overdose may cause serious immediate complications, with a slow and incomplete neurological recovery. Maria Ballesta Méndez, Vincent van Pesch, Arnaud Capron, and Philippe Hantson Copyright © 2014 Maria Ballesta Méndez et al. All rights reserved. Open-Ring Enhancement in Pseudotumoral Multiple Sclerosis: Important Radiological Aspect Tue, 15 Apr 2014 12:30:52 +0000 Introduction. Observation of open-ring enhancement in magnetic resonance imaging (MRI) is considered a specificity marker for diagnosing pseudotumoral multiple sclerosis (MS). This finding is of great value in the differential diagnosis of tumefactive lesions. Case Report. We describe a 55-year-old white woman, with previous history of ovarian cancer and recent history of fatigue and bilateral retroorbital pain. Important bilateral visual impairment evolved over one month. Physical examination detected the presence of right homonymous hemianopia. Cranial MRI showed an expanding lesion with open-ring enhancement. Given the range of diagnostic possibilities, a stereotactic biopsy was performed, and histopathological examination was consistent with an active demyelinating disease. The patient was treated with 1 g of methylprednisolone and symptoms improved following a significant reduction in the lesion. Conclusions. We highlight the MRI results suggestive of pseudotumoral MS, especially open-ring enhancement, which is an important radiologic aspect to diagnosis and can assist in avoiding unnecessary biopsies. Frederico Carvalho de Medeiros, Lucas Alverne Freitas de Albuquerque, Jose Eymard Homem Pittella, Renata Brant de Souza, Antonio Pereira Gomes Neto, and Paulo Pereira Christo Copyright © 2014 Frederico Carvalho de Medeiros et al. All rights reserved. Usefulness of Preoperative Surgical Simulation with Three-Dimensional Fusion Images for Resection of Cerebral Cavernous Malformations Near Broca’s Area Mon, 14 Apr 2014 10:28:30 +0000 Treating subcortical brain lesions in or near eloquent areas is challenging not only because lesions must be resected while preserving brain tissue involved in essential functions, but also because lesions often cannot be easily identified from the surface of the brain. Here, we report 2 cases of cerebral cavernous malformations near Broca’s area. In both cases, lesions were surgically removed by utilizing three-dimensional fusion images created using preoperative magnetic resonance imaging and computed tomography data. Excisions were completed without any worsening of speech function, and the use of presurgical simulations was found to be useful in the design and execution of the actual operations. The technique described in this report serves as a useful tool in simulating surgical strategies by using brain gyri and sulci as surgical landmarks. Furthermore, in contrast to other intraoperative techniques, this method can aid in shortening the duration of surgery and can help limit damage to eloquent areas of the brain. Satoshi Takahashi, Yoshio Tanizaki, Kazunori Akaji, Tadashige Kano, Kenji Hiraga, and Ban Mihara Copyright © 2014 Satoshi Takahashi et al. All rights reserved. An Adult Patient with Ocular Myasthenia and Unusually Long Spontaneous Remission Sun, 13 Apr 2014 00:00:00 +0000 A male patient developed ocular myasthenia gravis (MG) at the age of 33. He was anti-acetylcholine receptor antibody (anti-AChR Ab) negative. He received cholinesterase blocker for 5 months and went into a complete clinical remission that lasted untreated for 17 years. He relapsed recently with ocular symptoms only. He is now anti-AChR Ab positive and SFEMG is abnormal in a facial muscle. The patient is controlled with steroids. He had one of the longest spontaneous remissions reported in the natural history of MG, particularly unusual for an adult with the disease. Jasem Al-Hashel, Hanaa M. Rashad, and Rossen T. Rousseff Copyright © 2014 Jasem Al-Hashel et al. All rights reserved. The Middle Fossa Approach for the Removal of a Trochlear Schwannoma Wed, 12 Mar 2014 13:34:06 +0000 Objectives. Schwannomas originating from the trochlear nerve are extremely rare; only 30 cases have been reported in the literature. Many operative approaches have been utilized for lesion resection, but the advantages of the anterior transpetrosal approach are numerous and include excellent exposure, minimal extradural retraction of the temporal lobe, and minimal cerebrospinal fluid leaks. We report the second case of a trochlear schwannoma resected via the anterior transpetrosal approach. Setting. A 64-year-old male presented with 3-month history of diplopia and headaches. On physical examination, he was found to have a right fourth nerve palsy. Brain magnetic resonance imaging revealed a mass within the right ambient cistern compressing the adjacent midbrain. A right-sided anterior transpetrosal approach was used—which confirmed that the trochlear nerve entered the mass—to achieve gross total resection. Pathological examination confirmed diagnosis of schwannoma. The patient was discharged on postoperative day 3. He experienced a persistent fourth nerve palsy postoperatively with an otherwise normal neurological examination. Follow-up imaging confirmed complete removal of the tumor. Conclusion. The anterior transpetrosal approach is an excellent approach for removal of trochlear schwannomas involving the cisternal course of the trochlear nerve. It affords complete visualization of this anatomical region while introducing minimal morbidity. Andrew B. Boucher and L. Madison Michael II Copyright © 2014 Andrew B. Boucher and L. Madison Michael II. All rights reserved. Persistent Cryptococcal Brain Infection despite Prolonged Immunorecovery in an HIV-Positive Patient Wed, 05 Mar 2014 00:00:00 +0000 Background. HIV-positive people starting combined antiretroviral therapy may develop immune reconstitution to latent or treated opportunistic infections. Immune reconstitution to cerebral Cryptococcus is poorly understood and can be fatal. Case Presentation. A 33-year-old Zimbabwean female presented with cryptococcal meningitis and newly diagnosed HIV with a CD4 count of 51 cells/μL (4%). She was treated with amphotericin and flucytosine. Combined antiretroviral therapy was started four weeks later and she showed early improvement. However, over the ensuing 18 months, her clinical course was marked by periodic worsening with symptoms resembling cryptococcal meningitis despite having achieved CD4 counts ≥400 cells/μL. Although initially treated for relapsing cryptococcal immune reconstitution syndrome, a brain biopsy taken 17 months after initial presentation showed budding Cryptococci. Conclusion. This unusually protracted case highlights the difficulties in differentiating relapsing cryptococcal meningitis from immune reconstitution and raises questions concerning the optimum timing of initiation of combined antiretroviral therapy in such patients. Tom Wingfield, Jo Baxter, Amit Herwadkar, Daniel du Plessis, Tom J. Blanchard, F. Javier Vilar, and Anoop Varma Copyright © 2014 Tom Wingfield et al. All rights reserved. Synovial Cyst Mimicking an Intraspinal Sacral Mass Tue, 04 Mar 2014 09:58:25 +0000 A 68-year-old female had a three-week history of severe low back pain radiating down the posterior left buttocks and left leg exacerbated by standing and walking. Lumbar spine MRI revealed cystic mass with similar intensity to cerebrospinal fluid located on dorsolateral left side of the sacral spinal canal inferior to the S1 pedicle. There was compression of left exiting S1 and traversing S2 nerve roots. Neurosurgery consult was requested to evaluate the cystic mass in the sacral spinal canal. After clinical evaluation, an unusually located synovial cyst was thought possible. Cyst contents were heterogeneous, suggestive of small hemorrhage and acute clinical history seemed reasonable. Left S1 and partial left S2 hemilaminectomy was performed and an epidural, partially hemorrhagic cyst was removed. There was no obvious connection to the ipsilateral L5-S1 facet joint. Pathology revealed synovial cyst, and the patient’s leg pain was improved postoperatively. This synovial cyst was unusual as it had no connection with the facet joint intraoperatively and its location in the sacral canal was uncommon. Jason Hoover and Stephen Pirris Copyright © 2014 Jason Hoover and Stephen Pirris. All rights reserved. Central Pontine and Extrapontine Myelinolysis: The Great Masquerader—An Autopsy Case Report Tue, 04 Mar 2014 08:38:36 +0000 Central pontine myelinolysis is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis usually caused by rapid correction of chronic hyponatremia. The clinical features vary depending on the extent of involvement. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. We herein report a case of myelinolysis involving pons, lateral geniculate bodies, subependymal region, and spinal cord. To the best of our knowledge, this case represents the second case of spinal cord involvement in osmotic demyelination syndrome and the first case of involvement of thoracic region of spinal cord. Sajish Jacob, Harsh Gupta, Dejan Nikolic, Betul Gundogdu, and Shirley Ong Copyright © 2014 Sajish Jacob et al. All rights reserved. Recurrent Transient Ischemic Attacks in a Patient with POEMS Syndrome Tue, 04 Mar 2014 08:37:28 +0000 A 47-year-old female with a prior history of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome was admitted with transient ischemic attacks complicated by dysarthria and right-sided hemiparesis. A blood survey indicated thrombocytosis and hyperfibrinogenemia while imaging of intracranial vasculature showed occlusion of the bilateral middle cerebral arteries. POEMS syndrome, of which arterial thromboses have been mentioned as a manifestation, is rarely accompanied by transient ischemic attacks. The pathophysiologic mechanism is yet unclear and needs further investigation. A. Akyol, B. Nazliel, H. Z. Batur Caglayan, Y. Oner, and G. Turkoz Sucak Copyright © 2014 A. Akyol et al. All rights reserved. Wernicke's Encephalopathy Mimicking Acute Onset Stroke Diagnosed by CT Perfusion Sun, 23 Feb 2014 15:32:38 +0000 Background. Metabolic syndromes such as Wernicke’s encephalopathy may present with a sudden neurological deficit, thus mimicking acute onset stroke. Due to current emphasis on rapid admission and treatment of acute stroke patients, there is a significant risk that these stroke mimics may end up being treated with thrombolysis. Rigorous clinical and radiological skills are necessary to correctly identify such metabolic stroke mimics, in order to avoid doing any harm to these patients due to the unnecessary use of thrombolysis. Patient. A 51-year-old Caucasian male was admitted to our hospital with suspicion of an acute stroke due to sudden onset dysarthria and unilateral facial nerve paresis. Clinical examination revealed confusion and dysconjugate gaze. Computed tomography (CT) including a CT perfusion (CTP) scan revealed bilateral thalamic hyperperfusion. The use of both clinical and radiological findings led to correctly diagnosing Wernicke’s encephalopathy. Conclusion. The application of CTP as a standard diagnostic tool in acute stroke patients can improve the detection of stroke mimics caused by metabolic syndromes as shown in our case report. Alok Bhan, Rajiv Advani, Kathinka D. Kurz, Elisabeth Farbu, and Martin W. Kurz Copyright © 2014 Alok Bhan et al. All rights reserved. Progressive Multifocal Leukoencephalopathy in a 62-Year-Old Immunocompetent Woman Sun, 23 Feb 2014 09:59:06 +0000 Progressive multifocal encephalopathy (PML) is a rare demyelinating disease that typically presents in immunodeficient patients. We report a case of a previously healthy 62-Year-Old woman who suffered from an unsteady gait, throbbing headaches, and progressive left-sided weakness and numbness. Stroke was initially suspected based on imaging and symptoms. A series of follow-up magnetic resonance images of the brain showed a right parietal lesion growing in size as the patient became unable to walk and experienced increasing lethargy and confusion. A biopsy of the lesion was positive for the John Cunningham virus (JCV). A diagnosis of PML was made and she was started on mefloquine. No improvement was seen on this treatment and her condition worsened. Although PML remains uncommon in immunocompetent individuals, it cannot be ruled out based on their immune status. Although the exact cause remains uncertain, underlying or transient states of immunosuppression may be responsible for reactivation of the JCV in these patients. Venkata C. Gourineni, Tristan Juvet, Yogesh Kumar, Doru Bordea, and Kanaga N. Sena Copyright © 2014 Venkata C. Gourineni et al. All rights reserved. Nonketotic Hyperglycemic Chorea Sun, 23 Feb 2014 00:00:00 +0000 This is a unique case of nonketotic hyperglycemic (NKH) chorea in a 34-year-old white male. The patient had a poorly controlled type 2 diabetes mellitus (DM) due to medication incompliance. He complained of polyuria, polydipsia, and weight loss of 20 pounds within a month before presentation. T2-weighted (T2W) MRI showed hyperintensity in the left basal ganglion. Glycated hemoglobin (HBA1c) was 13.6%. The patient was started on insulin and clonazepam and the chorea resolved after proper control of the glucose level. To our knowledge, this is the first reported case of NKH chorea in a young white male with high T2-weighted (T2W) magnetic resonance signal in the basal ganglia. Mahmoud Abdelghany and Samuel Massoud Copyright © 2014 Mahmoud Abdelghany and Samuel Massoud. All rights reserved. Thrombosed Large Distal Posterior Inferior Cerebellar Artery Aneurysm Mimicking an Infratentorial Ependymoma Tue, 11 Feb 2014 12:56:00 +0000 Large or giant intracranial aneurysms can simulate brain tumors clinically and radiologically by virtue of their progressive mass effect. Unlike aneurysms from alternative locations, those arising from the distal posterior inferior cerebellar artery (PICA) are uncommon. We report a patient who experienced progressive hemiparesis with magnetic resonance imaging findings suggestive of an infratentorial ependymoma. Intraoperatively, a thrombosed large aneurysm of the distal PICA was unexpectedly encountered. The aneurysm was clipped and the patient did not develop any permanent neurological deficit. This case illustrates the radiological nuances of large aneurysms and infratentorial ependymomas. Three-dimensional contrast-enhanced magnetic resonance angiography can be falsely negative and the importance of the “target” sign is emphasized. One should be cognizant of this possible diagnosis for patients with midline fourth ventricular lesions in order to reduce surgical risk. Peter Yat Ming Woo, Natalie Man Wai Ko, and Kwong Yau Chan Copyright © 2014 Peter Yat Ming Woo et al. All rights reserved. Recurrent Wernicke s Encephalopathy in a 16-Year-Old Girl with Atypical Clinical and Radiological Features Mon, 10 Feb 2014 07:41:17 +0000 Background. Wernicke’s Encephalopathy (WE) is a clinical diagnosis with serious neurological consequences. Its occurrence is underestimated in nonalcoholics and is uncommon in adolescents. We aim to draw the attention to a rare case, which had additional clinical and radiological features. Case. A 16-year-old girl presented with three-week history of vomiting secondary to intestinal obstruction. She developed diplopia soon after hospitalization. Neurological evaluation revealed restriction of bilateral lateral recti with horizontal nystagmus, and bilateral limb dysmetria. Brain MRI was normal. She had prompt improvement to thiamine. Four months later, she presented with headache, bilateral severe deafness, and tinnitus. Clinically, she had severe sensorineural hearing loss, bilateral lateral recti paresis, and gait ataxia. CT head showed bilateral caudate nucleus hypodensities. MRI brain revealed gadolinium enhancement of mamillary bodies and vermis. She had significant improvement after IV thiamine. Headache completely resolved while the ocular movements, hearing, and tinnitus improved partially in 72 hours. Conclusions. Recurrent WE in adolescence is uncommon. Headache, tinnitus, and deafness are rare clinical features. Although MRI study shows typical features of WE, the presence of bilateral caudate nuclei hypodensities on CT scan is uncommon. Prompt treatment with thiamine is warranted in suspected cases to prevent permanent neurological sequelae. S. Lamdhade, A. Almulla, and R. Alroughani Copyright © 2014 S. Lamdhade et al. All rights reserved. An Asian Patient with Myoclonus-Dystonia (DYT11) Responsive to Deep Brain Stimulation of the Globus Pallidus Internus Sun, 09 Feb 2014 11:54:08 +0000 We describe the case of a 42-year-old Japanese woman with childhood-onset myoclonus, dystonia, and psychiatric symptoms, including anxiety, phobia, and exaggerated startle response. The diagnosis was confirmed as myoclonus-dystonia (DYT11) by identifying a mutation in the gene encoding ε-sarcoglycan. Interestingly, while motor-related symptoms in DYT11 generally improve with alcohol ingestion, the patient’s symptoms were exacerbated by alcohol intake. Her severe and medically intractable symptoms were alleviated by bilateral deep brain stimulation of the globus pallidus internus, with myoclonus and dystonia scores showing 70% improvement after the surgery compared to presurgical scores. This is the first report of a genetically confirmed case of DYT11 in Japan. This paper together with other recent reports collectively demonstrates that DYT11 patients are distributed worldwide, including Asia. Thus, a diagnosis of DYT11 should be considered when clinicians encounter a patient with childhood-onset myoclonus and/or dystonia with psychiatric symptoms, regardless of ethnic background. Akinori Uruha, Katsuo Kimura, and Ryoichi Okiyama Copyright © 2014 Akinori Uruha et al. All rights reserved. Cooled Radiofrequency Ablation for Bilateral Greater Occipital Neuralgia Thu, 06 Feb 2014 09:11:27 +0000 This report describes a case of bilateral greater occipital neuralgia treated with cooled radiofrequency ablation. The case is considered in relation to a review of greater occipital neuralgia, continuous thermal and pulsed radiofrequency ablation, and current medical literature on cooled radiofrequency ablation. In this case, a 35-year-old female with a 2.5-year history of chronic suboccipital bilateral headaches, described as constant, burning, and pulsating pain that started at the suboccipital region and radiated into her vertex. She was diagnosed with bilateral greater occipital neuralgia. She underwent cooled radiofrequency ablation of bilateral greater occipital nerves with minimal side effects and 75% pain reduction. Cooled radiofrequency ablation of the greater occipital nerve in challenging cases is an alternative to pulsed and continuous RFA to alleviate pain with less side effects and potential for long-term efficacy. Tiffany Vu and Akhil Chhatre Copyright © 2014 Tiffany Vu and Akhil Chhatre. All rights reserved. Susac's Syndrome in a Patient Diagnosed with MS for 20 Years: A Case Report Sun, 02 Feb 2014 09:10:25 +0000 Susac’s syndrome is an uncommon neurologic disorder of unknown cause. It has been described as a clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusions. Clinically the diagnosis is difficult when the patient presents only a portion of a triad. We present a case with vision loss and sensorineural deafness and who had been diagnosed with MS for 20 years. Susac’s syndrome is presumed to be an autoimmune endotheliopathy. Neurologic symptoms and signs are diffuse and multifocal, acute or subacute in onset, and progress during the active phase of the disease. In some patients the onset was stroke like and in others that of subacute dementia. Headache, often with migrainous features, was a prominent feature initially in more than one half of the patients. A high index of suspicion leading to correct diagnosis and early appropriate therapy may reduce the permanent sequel seen with this disease. Misdiagnosis is common. In patients in whom diagnosis and treatment are delayed permanent morbidity is higher in terms of visual loss, hearing loss, and neurologic debility. In patients in whom rapid diagnosis has led to early administration of immunosuppressive therapy, recovery can be almost complete. Bijen Nazliel, Asli Akyol, Hale Zeynep Batur Caglayan, Irem Yildirim-Capraz, and Ceyla Irkec Copyright © 2014 Bijen Nazliel et al. All rights reserved. Nonalcoholic Wernicke’s Encephalopathy Associated with Unintentional Weight Loss, Cholecystectomy, and Intractable Vomiting: The Role of Dual Thiamine and Corticosteroid Therapy Sun, 19 Jan 2014 14:09:22 +0000 A 23-year-old male with one month of intractable vomiting, subsequent cholecystitis status post cholecystectomy, and overall 40-pound weight loss over the last few months presented with altered mental status and seizures. MRI showed signal abnormalities involving the hypothalamus, periaqueductal gray matter, 4th ventricle, and bilateral thalami, indicative of Wernicke’s encephalopathy. The patient was started on empiric IV thiamine and methylprednisolone; thiamine levels were subsequently found to be low. Infectious disease workup was negative. Within a few days of this therapy, the patient’s neurological status steadily improved with increased responsiveness and communication. Repeat MRI 7 days after admission showed significant resolution of the signal abnormalities. Over the next several weeks the patient became fully conversational, cognitively intact, and increasingly ambulatory. Nonalcoholic Wernicke’s encephalopathy is rare; there have been reports relating it separately to vomiting and invasive surgery. In this case report, we associate it with both recurrent vomiting and minimally invasive cholecystectomy. We also discuss combinatorial therapy of thiamine and corticosteroids, which is poorly defined in the literature. Though there is no consensus-based optimal treatment of Wernicke’s encephalopathy, this adds to the discussion of using dual therapy and supports that the use of empiric corticosteroids does not harm the patient. Vivek Verma, Chenell Donadee, Leslie Gomez, and Marina Zaretskaya Copyright © 2014 Vivek Verma et al. All rights reserved. Dramatic Intracerebral Hemorrhagic Presentations of Reversible Cerebral Vasoconstriction Syndrome: Three Cases and a Literature Review Sun, 12 Jan 2014 10:12:24 +0000 Reversible cerebral vasoconstriction syndrome (RCVS) refers to a number of disorders characterized by severe and sudden-onset (“thunderclap”) headaches and angiographic features of reversible, segmental, multifocal vasoconstriction of cerebral arteries. Although RCVS generally resolves without significant sequelae, a rare and possibly underrecognized hemorrhagic presentation has a worse potential course. We report three cases of hemorrhagic RCVS and review the literature. Three females (42, 54, and 33 years old, resp.) presented with severe headache, neurological deficits, and dramatic intracerebral hemorrhage (ICH). Patient 1 presented comatose with a 9 × 4 × 6.6 cm left deep intraparenchymal hemorrhage (IPH) and 1 cm midline shift. She underwent emergent surgical intervention. Patient 2 had a 3.3 × 1.5 cm left superior frontal IPH that enlarged to 4 × 2.5 cm within 12 hours with worsening headache and neurological deficits. She was successfully managed nonoperatively. Patient 3, after uncomplicated pregnancy and delivery, presented with a 1.5 cm left superior parietal IPH on postpartum day 7. Two days later, she acutely developed right hemiplegia. Repeat CT demonstrated a new 3.3 × 1.7 cm left frontal IPH. She was also successfully managed nonoperatively. Many diverse conditions are grouped within the category of RCVS. Dramatic ICH remains a rare and possibly underrecognized presenting feature. Prompt diagnosis and management are essential for obtaining the best outcome. Joel M. Stary, Bonnie H. Wang, Seong-Jin Moon, and Huan Wang Copyright © 2014 Joel M. Stary et al. All rights reserved. High Grade Glioma Mimicking Voltage Gated Potassium Channel Complex Associated Antibody Limbic Encephalitis Sun, 12 Jan 2014 00:00:00 +0000 Though raised titres of voltage gated potassium channel (VGKC) complex antibodies have been occasionally associated with extracranial tumours, mainly presenting as Morvan's Syndrome or neuromyotonia, they have not yet been reported to be associated with an intracranial malignancy. This is especially important as misdiagnosis of these conditions and delay of the appropriate treatment can have important prognostic implications. We describe a patient with a high grade glioma presenting with clinical, radiological, and serological features consistent with the diagnosis of VGKC antibody associated limbic encephalitis (LE). This is the first association between a primary brain tumour and high titre of VGKC complex antibodies. Clinicoradiological progression despite effective immunosuppressive treatment should prompt clinicians to look for alternative diagnoses. Further studies to elucidate a possible association between VGKC complex and other surface antigen antibodies with primary brain tumours should be carried out. Dilan Athauda, R. S. Delamont, and E. De Pablo-Fernandez Copyright © 2014 Dilan Athauda et al. All rights reserved. Understanding Cerebellar Liponeurocytomas: Case Report and Literature Review Wed, 08 Jan 2014 09:57:02 +0000 Cerebellar liponeurocytomas were recognized in the 2000 WHO 3rd edition of CNS tumors as a distinct grade I pathological entity, a tumor with a more favorable prognosis than medulloblastoma. But reports of long-term recurrences and some possible aggressive behavior led to an upgrade on the latest WHO 4th edition of CNS tumors. The case of a 64-year-old female patient is reported in this paper. More than 30 cases of this lately recognized pathological entity have been reported to date. The diagnostic, radiological, and pathological features associated with this tumor are discussed through a literature review. M. Y. Oudrhiri, N. Raouzi, I. El Kacemi, N. El Fatemi, R. Gana, M. R. Maaqili, and F. Bellakhdar Copyright © 2014 M. Y. Oudrhiri et al. All rights reserved. Syncope Associated with Subthalamic Nucleus Deep Brain Stimulation in a Patient with Parkinson’s Disease Sun, 22 Dec 2013 14:03:56 +0000 In advanced Parkinson's disease (PD), deep brain stimulation (DBS) may be an alternative option for the treatment of motor symptoms. Side effects associated with subthalamic nucleus (STN) DBS in patients with PD are emerging as the most frequent sensory and motor symptoms. DBS-related syncope is reported as extremely rare. We wanted to discuss the mechanisms of syncope associated with STN DBS in a patient with Parkinson's disease. Case report. Sixty-three-year-old female patient is followed up with diagnosis of idiopathic Parkinson's disease for 6 years in our clinic. The patient has undergone STN DBS due to painful dystonia and drug resistant tremor. During the operation, when the left STN was stimulated at 5 milliampere (mAmp), the patient developed presyncopal symptoms. However, when the stimulation was stopped symptoms improved. During the early period after the operation, when the right STN was stimulated at 1.3 millivolts (mV), she developed the pre-yncopal symptoms and then syncope. Our case shows that STN DBS may lead to directly autonomic symptoms resulting in syncope during stimulation-on (stim-on). Dursun Aygun, Ersoy Kocabicak, Onur Yildiz, Musa Kazim Onar, Hatice Guz, Omer Boke, Murat Kurt, and Yasin Temel Copyright © 2013 Dursun Aygun et al. All rights reserved. Symptomatic Pneumocephalus Associated with Lumbar Dural Tear and Reverse Trendelenburg Positioning: A Case Report and Review of the Literature Sun, 22 Dec 2013 11:59:42 +0000 Symptomatic pneumocephalus is a rare complication of degenerative lumbar spine surgery. This is a case report of a patient who developed transient diplopia associated with pneumocephalus following lumbar spine surgery complicated by a dural tear. The diplopia improved as the pneumocephalus resolved. Factors involved in the development of pneumocephalus include an unintended durotomy and intraoperative reverse Trendelenburg positioning that was utilized to decrease the risk of postoperative vision loss. When encountering cerebrospinal fluid (CSF) leakage intraoperatively, spine surgeons should level the operating table until closure of the dural defect to prevent potential complications associated with pneumocephalus. If postoperative patients complain of severe headaches or display a focal cranial neurologic deficit, then a computed tomography (CT) scan of the brain should be ordered and evaluated. Consulting neurologists should be aware of the circumstances surrounding this rare complication. Stephen M. Pirris and Eric W. Nottmeier Copyright © 2013 Stephen M. Pirris and Eric W. Nottmeier. All rights reserved. Longitudinally Extensive Transverse Myelitis with Intramedullary Metastasis of Small-Cell Lung Carcinoma: An Autopsy Case Report Sun, 22 Dec 2013 11:58:59 +0000 Background. Longitudinally extensive transverse myelitis (LETM) is characterized by spinal cord inflammation extending vertically through three or more vertebral segments. The widespread use of MRI revealed LETM more frequency than ever. We report the case of a patient with pathologically confirmed small-cell lung carcinoma metastasis into the spinal cord presenting as LETM. Case Presentation. A 74-year-old man developed rapidly progressive sensorimotor disturbance and vesicorectal dysfunction. T2-weighted magnetic resonance imaging of the spine revealed LETM at the level of from T3 to conus medullaris; gadolinium enhancement showed concurrent tumor in the thoracic spinal cord from T10 to T11. Systemic survey identified a nodular mass in the lung that was verified as small-cell carcinoma. Following initial failed treatment by high-dose steroid, the patient underwent an emergent microsurgical tumor resection. Histological examination was identical with the lung carcinoma. The patient died of tumor progression at the 47th day after admission. At autopsy, only changes of edema were found in the gray matter of the cord, while tumor cells were not noted in it. Conclusion. Metastasis may rarely present symptoms of LETM. Prompt identification of underlying etiology by contrast examination and systemic survey is crucial for the patient assumed as LETM. Kenya Nishioka, Ryota Tanaka, Satoshi Tsutsumi, Hideki Shimura, Yutaka Oji, Harumi Saeki, Yukimasa Yasumoto, Masanori Ito, Nobutaka Hattori, and Takao Urabe Copyright © 2013 Kenya Nishioka et al. All rights reserved. Central Nervous System Demyelination in a Charcot-Marie-Tooth Type 1A Patient Mon, 16 Dec 2013 09:44:07 +0000 Introduction. Central nervous system involvement, either clinical or subclinical, has been reported mainly in X-linked Charcot-Marie-Tooth (CMT-X) patients. Case Presentation. We present the case of a 31-year-old man with a genetically confirmed history of CMT1A who developed CNS involvement mimicking multiple sclerosis (MS). Clinical, imaging, and laboratory findings suggested an autoimmune CNS demyelination. Discussion. Although the simultaneous existence of CMT1A and MS could be coincidental we postulate that overexpression of PMP22, the target protein in CMT1A, might influence the immunological self-tolerance to CNS proteins via molecular mimicry, leading to a CNS autoimmune demyelinating disorder. Christos Koros, Maria-Eleftheria Evangelopoulos, Costas Kilidireas, and Elisabeth Andreadou Copyright © 2013 Christos Koros et al. All rights reserved. Sporadic Hemiplegic Migraine with ATP1A2 and Prothrombin Gene Mutations Wed, 11 Dec 2013 13:31:56 +0000 Background. Hemiplegic migraine is a rare type of migraine that may present in children and adolescents. Both familial and sporadic hemiplegic migraines have similar prevalence and clinical characteristics. Patient. We report an adolescent with sporadic hemiplegic migraine who previously had a similar attack in the past and who was initially evaluated for a possible acute ischemic event. Results. Magnetic resonance angiography showed dilatation of the left middle cerebral artery that resolved in a follow-up study. She was also found to have a ATP1A2 (c.2273 G>C) mutation and a heterozygous prothrombin mutation. Conclusions. We suggest that patients with sporadic hemiplegic migraine be tested for both ATP1A2 mutations which in some cases may be pathogenic, and prothrombin mutations which increase the stroke risk for this patient population. Jose Aceves, Diana Mungall, and Batool F. Kirmani Copyright © 2013 Jose Aceves et al. All rights reserved. Posterior Cerebral Infarction following Loss of Guide Wire Sun, 08 Dec 2013 15:18:21 +0000 Stroke after internal jugular venous cannulation typically leads to acute carotid or vertebral arteries injury and cerebral ischemia. We report the first case of delayed posterior cerebral infarction following loss of guide wire after left internal jugular venous cannulation in a 46-year-old woman with a history of inflammatory bowel disease. Our observation highlights that loss of an intravascular guide wire can be a cause of ischemic stroke in patients undergoing central venous catheterization. Jean-Marc Bugnicourt, Denis Belhomme, Bruno Bonnaire, Jean-Marc Constans, and Cécile Manaouil Copyright © 2013 Jean-Marc Bugnicourt et al. All rights reserved. Marked Seizure Reduction after MCT Supplementation Sun, 08 Dec 2013 11:29:35 +0000 We report the case of a 43-year-old man with history of nonsurgical partial epilepsy who previously failed multiple trials of antiepileptic drugs. Medium-chain triglycerides (MCT) were added to his regular diet in the form of pure oil. Subsequently, his seizure frequency was markedly reduced from multiple daily seizures to one seizure every four days. His seizures recurred after transient discontinuation of MCT over a period of ten days. His seizure improvement was achieved at a dose of four tablespoons of MCT twice daily with no reported side effects. He developed significant diarrhea and flatulence at higher doses. We conclude that MCT oil supplementation to regular diet may provide better seizure control in some patients. MCT oil supplementation may be a more tolerable alternative to the standard ketogenic diet. Raed Azzam and Nabil J. Azar Copyright © 2013 Raed Azzam and Nabil J. Azar. All rights reserved.