Case Reports in Neurological Medicine https://www.hindawi.com The latest articles from Hindawi © 2017 , Hindawi Limited . All rights reserved. Homonymous Superior Quadrantanopia due to Erdheim-Chester Disease with Asymptomatic Pituitary Involvement Tue, 23 May 2017 07:06:01 +0000 http://www.hindawi.com/journals/crinm/2017/2807461/ 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 http://www.hindawi.com/journals/crinm/2017/2593096/ 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 http://www.hindawi.com/journals/crinm/2017/7965758/ 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 http://www.hindawi.com/journals/crinm/2017/1045039/ 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 http://www.hindawi.com/journals/crinm/2017/5675732/ 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 http://www.hindawi.com/journals/crinm/2017/3247034/ 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 http://www.hindawi.com/journals/crinm/2017/4318450/ 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 http://www.hindawi.com/journals/crinm/2017/1835796/ 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 http://www.hindawi.com/journals/crinm/2017/2736402/ 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 http://www.hindawi.com/journals/crinm/2017/4919758/ 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 http://www.hindawi.com/journals/crinm/2017/5146723/ 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 http://www.hindawi.com/journals/crinm/2017/1471096/ 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 http://www.hindawi.com/journals/crinm/2017/3861804/ Chadi Tannoury and Anthony Degiacomo Copyright © 2017 Chadi Tannoury and Anthony Degiacomo. All rights reserved. Sacral Neuromodulation: Foray into Chronic Pelvic Pain in End Stage Endometriosis Mon, 06 Mar 2017 00:00:00 +0000 http://www.hindawi.com/journals/crinm/2017/2197831/ Excision of all endometriotic lesions is the method of choice in the treatment of severe endometriosis resistant to medical therapy. The infiltrating nature of the disease as well as extensive surgery may, however, cause chronic pain that cannot be relieved by either surgery or hormonal treatment. As a pilot treatment, we tested the effect of sacral neuromodulation (SNM) for four endometriosis patients suffering chronic pelvic pain and pelvic organ dysfunction after radical surgical treatment. Three out of four patients reported improvement in their symptoms during the neuromodulation testing period and a permanent pulse generator was installed. After 2.5 years, all three patients report better quality of life and want to continue with SNM. Maija Lavonius, Pia Suvitie, Pirita Varpe, and Heikki Huhtinen Copyright © 2017 Maija Lavonius et al. All rights reserved. Central Hyperthermia Treated with Bromocriptine Tue, 28 Feb 2017 00:00:00 +0000 http://www.hindawi.com/journals/crinm/2017/1712083/ Introduction. Central hyperthermia is common in patients with brain injury. It typically has a rapid onset with high temperatures and marked fluctuations and responds poorly to antibiotics and antipyretics. It is also associated with worse outcomes in the brain injured patient. Recognizing this, it is important to aggressively manage it. Case Report. We report a 34-year-old male with a right thalamic hemorrhage extending to the midbrain and into the ventricles. During his admission, he developed intractable fevers with core temperatures as high as 39.3°C. Infectious workup was unremarkable. The fever persisted despite empiric antibiotics, antipyretics, and cooling wraps. Bromocriptine was started resulting in control of the central hyperthermia. The fever spikes were reduced to minor fluctuations that significantly worsened with any attempt to wean off the bromocriptine. Conclusion. Diagnosing and managing central hyperthermia can be challenging. The use of bromocriptine can be beneficial as we have reported. P. Natteru, P. George, R. Bell, P. Nattanmai, and C. R. Newey Copyright © 2017 P. Natteru et al. All rights reserved. Hemifacial Pain and Hemisensory Disturbance Referred from Occipital Neuralgia Caused by Pathological Vascular Contact of the Greater Occipital Nerve Sun, 26 Feb 2017 06:48:36 +0000 http://www.hindawi.com/journals/crinm/2017/3827369/ Here we report a unique case of chronic occipital neuralgia caused by pathological vascular contact of the left greater occipital nerve. After 12 months of left-sided, unremitting occipital neuralgia, a hypesthesia and facial pain developed in the left hemiface. The decompression of the left greater occipital nerve from pathological contacts with the occipital artery resulted in immediate relief for hemifacial sensory change and facial pain, as well as chronic occipital neuralgia. Although referral of pain from the stimulation of occipital and cervical structures innervated by upper cervical nerves to the frontal head of V1 trigeminal distribution has been reported, the development of hemifacial sensory change associated with referred trigeminal pain from chronic occipital neuralgia is extremely rare. Chronic continuous and strong afferent input of occipital neuralgia caused by pathological vascular contact with the greater occipital nerve seemed to be associated with sensitization and hypersensitivity of the second-order neurons in the trigeminocervical complex, a population of neurons in the C2 dorsal horn characterized by receiving convergent input from dural and cervical structures. Byung-chul Son and Jin-gyu Choi Copyright © 2017 Byung-chul Son and Jin-gyu Choi. All rights reserved. Rheumatoid Meningitis Occurring during Etanercept Treatment Mon, 13 Feb 2017 10:04:23 +0000 http://www.hindawi.com/journals/crinm/2017/7638539/ We report a 65-year-old man who had repetitive seizures 6 months after receiving etanercept, methotrexate, and prednisolone for rheumatoid arthritis. Mononuclear cells were mildly increased in the cerebrospinal fluid (CSF). Brain magnetic resonance imaging (MRI) showed high intensity along sulci of the frontal and parietal lobes. Brain biopsy revealed lymphocyte and plasma cell infiltration in the meninges, confirming the diagnosis of rheumatoid meningitis. After steroid pulse therapy, seizures resolved and clinical findings improved. When etanercept was replaced by tocilizumab, rheumatoid meningitis did not recur. Although TNF-α inhibitors can control joint symptoms of rheumatoid arthritis, they may induce rheumatoid meningitis. Koji Tsuzaki, Takashi Nakamura, Hiroyuki Okumura, Naoko Tachibana, and Toshiaki Hamano Copyright © 2017 Koji Tsuzaki et al. All rights reserved. Improvement and Neuroplasticity after Combined Rehabilitation to Forced Grasping Mon, 06 Feb 2017 11:15:40 +0000 http://www.hindawi.com/journals/crinm/2017/1028390/ The grasp reflex is a distressing symptom but the need to treat or suppress it has rarely been discussed in the literature. We report the case of a 17-year-old man who had suffered cerebral infarction of the right putamen and temporal lobe 10 years previously. Forced grasping of the hemiparetic left upper limb was improved after a unique combined treatment. Botulinum toxin type A (BTX-A) was first injected into the left biceps, wrist flexor muscles, and finger flexor muscles. Forced grasping was reduced along with spasticity of the upper limb. In addition, repetitive facilitative exercise and object-related training were performed under low-amplitude continuous neuromuscular electrical stimulation. Since this 2-week treatment improved upper limb function, we compared brain activities, as measured by near-infrared spectroscopy during finger pinching, before and after the combined treatment. Brain activities in the ipsilesional sensorimotor cortex (SMC) and medial frontal cortex (MFC) during pinching under electrical stimulation after treatment were greater than those before. The results suggest that training under electrical stimulation after BTX-A treatment may modulate the activities of the ipsilesional SMC and MFC and lead to functional improvement of the affected upper limb with forced grasping. Michiko Arima, Atsuko Ogata, Kazumi Kawahira, and Megumi Shimodozono Copyright © 2017 Michiko Arima et al. All rights reserved. Is Intravenous Heparin a Contraindication for TPA in Ischemic Stroke? Sun, 05 Feb 2017 10:36:37 +0000 http://www.hindawi.com/journals/crinm/2017/9280961/ There are approximately 2 million cardiac catheterizations that occur every year in the United States and with an aging population this number continues to rise. Adverse events due to this procedure occur at low rates and include stroke, arrhythmia, and myocardial infarctions. Due to the high volume of procedures there are a growing number of adverse events. Stroke after cardiac catheterization (SCC) has an incidence between 0.27 and 0.5% and is one of the most debilitating complications leading to high rates of mortality and morbidity. Given the relatively uncommon clinical setting of stroke after cardiac catheterization, treatment protocols regarding the use of IV or IA thrombolysis have not been adequately developed. Herein, we describe a case of a 39-year-old male who developed a stroke following a cardiac catheterization where IV thrombolysis was utilized although the patient was on heparin prior to cardiac catheterization. Zain Kulairi, Nisha Deol, Renee Tolly, Rohan Manocha, and Maliha Naseer Copyright © 2017 Zain Kulairi et al. All rights reserved. A Case of “Refractory” Neuropsychiatric Lupus Responsive to Anticoagulation Mon, 30 Jan 2017 08:32:22 +0000 http://www.hindawi.com/journals/crinm/2017/5726180/ Neuropsychiatric disorder is a severe complication in 14% to 75% of systemic lupus erythematosus (SLE) patients, which can result in significant morbidity. A 15-year-old female SLE patient with coexistence of dural sinus thrombosis and intracerebral hemorrhage resistant to two pulses of high dose of glucocorticoid was treated with anticoagulation of the low-molecular-weight [LMW] heparin subcutaneously followed by warfarin. The patient demonstrated a remarkable clinical response. Rui Wu and Sun Hu Copyright © 2017 Rui Wu and Sun Hu. All rights reserved. A Pediatric Tumor Found Frequently in the Adult Population: A Case of Anaplastic Astroblastoma in an Elderly Patient and Review of the Literature Mon, 23 Jan 2017 07:51:31 +0000 http://www.hindawi.com/journals/crinm/2017/1607915/ Astroblastomas are rare, potentially curable primary brain tumors which can be difficult to diagnose. We present the case of astroblastoma in a 73-year-old male, an atypical age for this tumor, more classically found in pediatric and young adult populations. Through our case and review of the literature, we note that this tumor is frequently reported in adult populations and the presentation of this tumor in the elderly is well described. This tumor is an important consideration in the differential diagnosis when managing both pediatric and adult patients of any age who present with the imaging findings characteristic of this rare tumor. Christopher Payne, Ali Batouli, Kristen Stabingas, Dunbar Alcindor, Khaled Abdel Aziz, Cunfeng Pu, Elizabeth Tyler-Kabara, Robert Williams, and Alexander Yu Copyright © 2017 Christopher Payne et al. All rights reserved. Hemorrhagic Colloid Cyst Presenting with Acute Hydrocephaly Sun, 22 Jan 2017 07:43:00 +0000 http://www.hindawi.com/journals/crinm/2017/2978080/ Colloid cysts are benign slow-growing cystic lesions located on the roof of the third ventricle that usually present with symptoms related to gradual rise of intracranial pressure. They mostly remain asymptomatic and sometimes grow progressively and cause diverse symptoms associated with increased intracranial pressure such as headache, diplopia, and sixth cranial nerve palsy. Here we report a 47-year-old female who presented to the emergency department with acute severe headache and nausea/vomiting. On MRI examination acute hydrocephaly due to hemorrhagic colloid cyst was detected. Acute hemorrhage in colloid cysts is extremely rare and may present with symptoms of acute increase in the intracranial pressure. Intracystic hemorrhage is very rarely reported as a complication of colloid cyst presenting with paroxysmal symptoms of acute hydrocephaly. Reza Akhavan, Behrouz Zandi, Masoud Pezeshki-Rad, Donya Farrokh, and Bita Abbasi Copyright © 2017 Reza Akhavan et al. All rights reserved. Seizure following the Use of the COX-2 Inhibitor Etoricoxib Sun, 22 Jan 2017 00:00:00 +0000 http://www.hindawi.com/journals/crinm/2017/1410759/ We describe a case of epileptic seizures occurring after the use of a COX-2 inhibitor. A 61-year-old man was admitted to our department because of a generalized tonic-clonic seizure. EEG showed generalized slowdown of the activity. Neuroimaging and blood samples studies did not evidence alterations, but a careful pharmacological history revealed that the patient had taken the COX-2 inhibitor etoricoxib to treat lumbago few days before the onset of clinical symptoms. No seizures were reported after etoricoxib discontinuation and an EEG resulted to be normal two months after this. Conclusion. Knowing the pharmacological history of a patient is important for understanding the clinical presentation and selecting appropriate treatment. This is, to the best of our knowledge, the first reported case of generalized seizures associated with the use of COX-2 inhibitors. Valentina Arnao, Marianna Riolo, Brigida Fierro, and Paolo Aridon Copyright © 2017 Valentina Arnao et al. All rights reserved. Nonconvulsive Status Epilepticus Resembling Clinical Absence with Atypical EEG Pattern Thu, 19 Jan 2017 00:00:00 +0000 http://www.hindawi.com/journals/crinm/2017/6987821/ Objective. We are reporting two cases: a patient with steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) and another patient with secondary progressive multiple sclerosis (SPMS), both presenting with altered mental status (AMS) and later diagnosed with nonconvulsive atypical absence status epilepticus (AS), with atypical EEG changes. Methods. A report of two cases. Results. A patient with history of SREAT and the other with SPMS had multiple admissions due to AMS. For both, EEG revealed the presence of a high voltage generalized sharply contoured theta activity. A diagnosis of NCSE with clinical features of AS was made based on both clinical and EEG features. There was significant clinical and electrographic improvement with administration of levetiracetam for both patients in addition to sodium valproate and Solumedrol for the SREAT patient. Both patients continued to be seizure free on follow-up few months later. Conclusions. This is a report of two cases of atypical AS, with atypical EEG, in patients with different neurological conditions. Prompt clinical and EEG recovery occurred following appropriate medical treatment. We think that this condition might be underreported and could significantly benefit from prompt treatment when appropriately diagnosed. Channaiah Srikanth Mysore, Najib Murr, Rana Zabad, and John Bertoni Copyright © 2017 Channaiah Srikanth Mysore et al. All rights reserved. Variability in Clinical Presentation of Neonatal Stroke: Report of Four Cases Tue, 17 Jan 2017 00:00:00 +0000 http://www.hindawi.com/journals/crinm/2017/5215686/ Neonatal stroke can be a cause of long term neurodevelopmental disability, seizures, and impaired cognitive function. We present four cases of neonatal stroke, associated with different risk factors and clinical presentations. Two of these newborns were born to mothers with no prenatal care. Sonia Joseph, Dimitrios Angelis, Robert Bennett, Bhargavi Kola, and Amanda Hughes Copyright © 2017 Sonia Joseph et al. All rights reserved. A Case Report of Nonvasculitic Autoimmune Inflammatory Meningoencephalitis with Sensory Ganglionopathy: A Rare Presentation of Sjögren Syndrome Sun, 15 Jan 2017 08:59:15 +0000 http://www.hindawi.com/journals/crinm/2017/5696512/ A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids. A simultaneous improvement of the cognitive abilities allowed for the detection of sensory ataxia of the lower limbs. Sensory ganglionopathy was then detected with electrophysiological studies. A diagnosis of Sjögren syndrome was suspected and confirmed by salivary gland scintigraphy, Schirmer’s test, and submaxillary gland biopsy. We report a case of Sjögren syndrome associated with central and peripheral nervous system involvement, without sicca symptoms preceding the neurological clinical picture. The coexistence of ganglionopathy and a favourable response to immunosuppression are key features that can lead to the correct diagnosis in cases with atypical CNS symptoms, mimicking a rapidly progressive dementia. João Peres, Simão Cruz, Rita Oliveira, Luís Santos, and Ana Valverde Copyright © 2017 João Peres et al. All rights reserved. Evolution of Cerebral Atrophy in a Patient with Super Refractory Status Epilepticus Treated with Barbiturate Coma Sun, 15 Jan 2017 08:22:52 +0000 http://www.hindawi.com/journals/crinm/2017/9131579/ Introduction. Status epilepticus is associated with neuronal breakdown. Radiological sequelae of status epilepticus include diffusion weighted abnormalities and T2/FLAIR cortical hyperintensities corresponding to the epileptogenic cortex. However, progressive generalized cerebral atrophy from status epilepticus is underrecognized and may be related to neuronal death. We present here a case of diffuse cerebral atrophy that developed during the course of super refractory status epilepticus management despite prolonged barbiturate coma. Methods. Case report and review of the literature. Case. A 19-year-old male with a prior history of epilepsy presented with focal clonic seizures. His seizures were refractory to multiple anticonvulsants and eventually required pentobarbital coma for 62 days and midazolam coma for 33 days. Serial brain magnetic resonance imaging (MRI) showed development of cerebral atrophy at 31 days after admission to our facility and progression of the atrophy at 136 days after admission. Conclusion. This case highlights the development and progression of generalized cerebral atrophy in super refractory status epilepticus. The cerebral atrophy was noticeable at 31 days after admission at our facility which emphasizes the urgency of definitive treatment in patients who present with super refractory status epilepticus. Further research into direct effects of therapeutic coma is warranted. Christopher R. Newey, Pravin George, Premkumar Nattanmai, Christine Ahrens, Stephen Hantus, and Aarti Sarwal Copyright © 2017 Christopher R. Newey et al. All rights reserved. Hyperechogenicity of the Substantia Nigra in Parkinson’s Disease: Insights from Two Brothers with Markedly Different Disease Durations Wed, 11 Jan 2017 00:00:00 +0000 http://www.hindawi.com/journals/crinm/2017/3673159/ We present clinical features and substantia nigra morphology for two brothers with Parkinson’s disease (PD) aged 60 and 59 years. The brothers were diagnosed at 41 and 50 years of age, respectively. Both patients exhibited an abnormally large area of substantia nigra echogenicity bilaterally when viewed with transcranial ultrasound. The abnormality was similar in both brothers despite one having a much longer disease duration than the other. These findings further highlight that transcranial ultrasound is not associated with severity of clinical symptoms, but it might assist in the diagnosis of PD provided that it is combined with other variables known to precede PD. Julie M. Hall, Matthew J. Georgiades, Deborah A. Hammond, Xiaoting Feng, Ahmed A. Moustafa, Simon J. G. Lewis, and Gabrielle Todd Copyright © 2017 Julie M. Hall et al. All rights reserved. Chronic Subdural Hematoma Associated with Thrombocytopenia in a Patient with Human Immunodeficiency Virus Infection in Cameroon Tue, 10 Jan 2017 08:02:58 +0000 http://www.hindawi.com/journals/crinm/2017/5395829/ Hematological abnormalities including thrombocytopenia are common in patients living with HIV infection. Patients with HIV infection related thrombocytopenia present generally with only minor bleeding problems. But cases of subdural hematoma are very rare. A 61-year-old female with a history of HIV infection of 9 years’ duration presented with a 3-month history of generalized headache associated with visual blurring and anterograde amnesia. There was no history of trauma or fever. She was treated empirically for cerebral toxoplasmosis for 6 weeks without any improvement of the symptoms. One week prior to admission, she developed weakness of the left side of the body. Clinical examination revealed left-sided hemiparesis. Computed tomography scan of the brain showed a 25 mm chronic right frontoparietotemporal subdural hematoma compressing the lateral ventricle with midline shift. There was no appreciable cerebral atrophy. A complete blood count showed leucopenia and thrombocytopenia at 92,000 cells/mm3. Her CD4-positive cell count was 48 cells/mm3 despite receiving combination antiretroviral therapy for 9 years. A complete blood count analysis suggestive of thrombocytopenia should raise suspicion of possibilities of noninfectious focal brain lesions like subdural hematoma amongst HIV infected patients presenting with nonspecific neurological symptoms. This will enable prompt diagnosis and allow early appropriate intervention. Clovis Nkoke, Engelbert Bain Luchuo, Denis Teuwafeu, Ines Nepetsoun, and Cyrille Nkouonlack Copyright © 2017 Clovis Nkoke et al. All rights reserved. Treatment Challenges of a Primary Vertebral Artery Aneurysm Causing Recurrent Ischemic Strokes Tue, 10 Jan 2017 00:00:00 +0000 http://www.hindawi.com/journals/crinm/2017/2571630/ Background. Extracranial vertebral artery aneurysms are a rare cause of embolic stroke; surgical and endovascular therapy options are debated and long-term complication may occur. Case Report. A 53-year-old man affected by neurofibromatosis type 1 (NF1) came to our attention for recurrent vertebrobasilar embolic strokes, caused by a primary giant, partially thrombosed, fusiform aneurysm of the left extracranial vertebral artery. The aneurysm was treated by endovascular approach through deposition of Guglielmi Detachable Coils in the proximal segment of the left vertebral artery. Six years later the patient presented stroke recurrence. Cerebral angiography and Color Doppler Ultrasound well characterized the unique hemodynamic condition developed over the years responsible for the new embolic event: the aneurysm had been revascularized from its distal portion by reverse blood flow coming from the patent vertebrobasilar axis. A biphasic Doppler signal in the left vertebral artery revealed a peculiar behavior of the blood flow, alternately directed to the aneurysm and backwards to the basilar artery. Surgical ligation of the distal left vertebral artery and excision of the aneurysm were thus performed. Conclusion. This is the first described case of NF1-associated extracranial vertebral artery aneurysm presenting with recurrent embolic stroke. Complete exclusion of the aneurysm from the blood circulation is advisable to achieve full resolution of the embolic source. Davide Strambo, Luca Peruzzotti-Jametti, Aurora Semerano, Giovanna Fanelli, Franco Simionato, Roberto Chiesa, Enrico Rinaldi, Vittorio Martinelli, Giancarlo Comi, Marco Bacigaluppi, and Maria Sessa Copyright © 2017 Davide Strambo et al. All rights reserved.