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Clinical Neurology and Neurosurgery

Yinglu Liu, Zhao Dong, Shengyuan Yu
Phenylketonuria (PKU) is a prevalent inherited metabolic disorder caused by a phenylalanine hydroxylase (PAH) or tetrahydrobiopterin (BH4) deficiency, which leads to the accumulation of phenylalanine (PHE). High blood levels of PHE have a toxic effect on the brain and are associated with several neurological signs. Most cases of PKU are identified during infancy, and diagnosis of PKU in adult is rare. Here, we describe a 29-year-old patient with progressive dementia and muscular weakness mimicking X-linked adrenoleukodystrophy...
June 11, 2018: Clinical Neurology and Neurosurgery
Douglas T Hidlay, Ryan A McTaggart, Grayson Baird, Shadi Yaghi, Morgan Hemendinger, Eric L Tung, Eleanor L Dibiasio, Richard A Haas, Mahesh V Jayaraman
OBJECTIVES: Mechanical thrombectomy is the standard of care for stroke caused by an emergent large vessel occlusion in the anterior circulation, and the ability to rapidly review CTA is one hurdle in minimizing the time from diagnosis to intervention. We evaluated the diagnostic accuracy and confidence in review of stroke CTA for ELVO via a smartphone-based application as compared to PACS workstation. PATIENTS AND METHODS: Seventy-six head and neck CTA studies performed for stroke from one comprehensive and seven primary stroke centers were independently reviewed remotely on smartphone by two blinded interventional neuroradiologists in actual-use circumstances...
June 11, 2018: Clinical Neurology and Neurosurgery
Thomas P Pittelkow, Markus A Bendel, Daniel R Lueders, Lisa A Beck, Matthew J Pingree, Bryan C Hoelzer
OBJECTIVES: Exploratory research quantifying the change of spasticity among patients who underwent baclofen intrathecal drug delivery system (IDDS) implantation. PATIENTS AND METHODS: 88 patients with a baclofen IDDS were identified. Patient characteristics, spasticity scores pre/post intrathecal baclofen test dose, and IDDS perioperative implantation records were collected. The primary outcome was to quantify the extent to which there was a change in Modified Ashworth Scores (MAS) pre/post-intrathecal baclofen test dose administration...
June 10, 2018: Clinical Neurology and Neurosurgery
Erica Garbin Risson, Ana Paula Serpa, Jéssica Jacques Berger, Renata Fabiola Heil Koerbel, Andrei Koerbel
OBJECTIVE: Spinal cord stimulation has been proven highly effective in the treatment of Complex Regional Pain Syndrome (CRPS). The definitive implantation of a neurostimulator is usually preceded by a therapeutic test (trial), which has the purpose of identifying whether the patient would respond positively to neuromodulation or not. The present study aims to analyze the surgical results of spinal cord stimulation in type 1 CRPS patients who have not undergone trial. PATIENTS AND METHODS: From January 2011 to August 2017, 160 patients underwent implantation of spinal cord neurostimulator...
June 10, 2018: Clinical Neurology and Neurosurgery
Paulo José Lorenzoni, Renata Dal-Prá Ducci, Giuliano Ohde Dalledone, Claudia Suemi Kamoi Kay, Sérgio Monteiro de Almeida, Lineu Cesar Werneck, Rosana Hermínia Scola
HIV-associated motor neuron disease (MND), or amyotrophic lateral sclerosis (ALS)-like syndrome associated with HIV infection, is a rare manifestation of HIV infection. HIV-associated MND has only been identified in few cases to date. We analysed two Brazilian patients with HIV infection who developed MND. The diagnosis of HIV infection was concomitant with diagnosis of MND in one patient and it occurred eight years before the MND symptoms in another patient. The manifestation of MND in our patients with HIV infection was similar to classic ALS...
June 10, 2018: Clinical Neurology and Neurosurgery
Kavelin Rumalla, Kyle A Smith, Kenneth A Follett, Jules M Nazzaro, Paul M Arnold
OBJECTIVE: Deep brain stimulation (DBS) surgery has proven benefit for several movement disorders and medically-refractory psychiatric conditions and is considered a fairly safe procedure. We sought to determine the national rates, causes, predictors, and outcomes associated with 30-day and 90-day readmission. PATIENTS AND METHODS: The Nationwide Readmissions Database was queried (January-September 2013) using ICD-9-CM codes, identifying patients who underwent DBS for movement disorder (Parkinson's disease [PD], essential tremor [ET], or dystonia)...
June 10, 2018: Clinical Neurology and Neurosurgery
Moinay Kim, Jae Sung Ahn, Wonhyoung Park, Suk Kyung Hong, Sang Ryong Jeon, Sung Woo Roh, Seungjoo Lee
OBJECTIVE: Diffuse axonal injury (DAI) is known to be associated with poor outcome. DAI often associates with other intracranial injuries but their distinct features have not been established. In this retrospective cohort study, we compared clinical outcomes between pure and non-pure DAI patients. PATIENTS AND METHODS: Total of 1047 traumatic brain injury (TBI) patients visited our institute between 2011 and 2017. Age ranged between 15-85 years old and Glasgow coma scale (GCS) score less than 13 were included...
June 10, 2018: Clinical Neurology and Neurosurgery
Brittany N Burton, Jenny Q Hu, Aria Jafari, Richard D Urman, Ian F Dunn, W Linda Bi, Adam S DeConde, Rodney A Gabriel
OBJECTIVES: Updated multi-institutional database studies assessing perioperative risk factors on 30-day morbidity and mortality after skull base surgeries are limited. We aim to identify perioperative risk factors and report the incidence of 30-day morbidity and mortality in adult patients after skull base surgery. PATIENTS AND METHODS: We queried the 2007-2016 American College of Surgeons National Surgical Quality Improvement program database to identify patients who underwent anterior, middle, or posterior skull base surgery...
June 10, 2018: Clinical Neurology and Neurosurgery
Murat Mert Atmaca, Nerses Bebek, Elif Kocasoy-Orhan, Candan Gürses
OBJECTIVE: Epilepsia partialis continua (EPC) is a special form of cortical epilepsy. Several studies have described the ictal and interictal electroencephalography (EEG) findings in patients with EPC; however, lateralizing and localizing values of these findings have been evaluated rarely. This study investigated the correlation of semiologic and EEG findings, and outcomes in patients with EPC. PATIENTS AND METHODS: Clinical and EEG findings and outcomes, and their correlations were studied prospectively in 15 patients who were diagnosed as having EPC upon presentation to Istanbul Medical Faculty Hospital between January 2010 and April 2014, and retrospectively in 5 previously evaluated patients...
June 9, 2018: Clinical Neurology and Neurosurgery
Jianguo Shi, Zaifen Gao, Jun Gao, Gang Li, Yehong Chen
OBJECTIVE: The aim of this study is to identify the prognostic role of MRI and EEG in posterior cortex epilepsies (PCES) and to characterize their clinical features. PATIENT AND METHODS: We retrospectively studied 54 consecutive patients (18 females, 36 males) from April 2011 to November 2015, who had undergone PCES surgery. Electro-clinical, pathological and surgical data were evaluated. Of the patients, 18 (33.3%) patients underwent a cortical resection (corticectomy), 10 (18...
June 9, 2018: Clinical Neurology and Neurosurgery
Mesut Yilmaz, Canan Yasar, Selda Aydin, Okan Derin, Burcu Polat, Gulhan Ertan, Bahadir Ceylan, Ali Mert
Central nervous system infection caused by Human Herpesvirus 6 (HHV-6) is well known in immunocompromised; however, data regarding immunocompetent patients is limited to case series. We describe a 29-year-old immunocompetent pregnant woman with meningoencephalitis due to HHV-6 and review current literature. HHV-6 should be kept in mind in patients with meningoencephalitis of unknown etiology.
June 5, 2018: Clinical Neurology and Neurosurgery
Youssef A Al-Said, Saleh S Baeesa, Zaitoon Shivji, Husam Kayyali, Khalid Alqadi, Ghada Kadi, Edward J Cupler, Ahmad R Abuzinadah
OBJECTIVE: Electroencephalography (EEG) in the intensive care unit (ICU) is often done to detect non-convulsive seizures (NCS). The outcome of ICU patients with NCS strongly depends on the underlying etiology. The implication of NCS and other EEG findings on clinical outcome independent from their etiology is not well understood and our aim to investigate it. PATIENTS AND METHODS: We retrospectively identified all adult patients in the ICU who underwent EEG monitoring between January 2008 and December 2011...
June 5, 2018: Clinical Neurology and Neurosurgery
Pravin Salunke, Madhivanan Karthigeyan, Sushanta K Sahoo, Narasimhaiah Sunil
OBJECTIVE: Congenital atlanto-axial dislocation (CAAD) often manifests in younger age group. Softer bones, deformed joints and the concern of fusing multiple segments in growing age set apart this subset of patients from adults. Objectives of this manuscript are to discuss the challenges faced and subsequent solutions during multiplanar realignment and fusion (short segment) of C1-2 joints through a direct posterior approach in pediatric CAAD. PATIENTS & METHODS: 56 pediatric patients with CAAD were operated through a direct posterior approach in the last 4 years...
June 5, 2018: Clinical Neurology and Neurosurgery
Philipp Franck, Jaime L Bernstein, Leslie E Cohen, Roger Härtl, Ali A Baaj, Jason A Spector
OBJECTIVES: Patients with neoplastic disease involving the spine either from primary or metastatic disease present a unique challenge given these patients' frequent poor nutritional status at the time of surgery, the delivery of early post-operative chemotherapy or radiation, and placement of large amounts of hardware and avascular bone graft into a wound bed that is atrophic, previously operated or irradiated. As a result, wound morbidity has traditionally been high in this cohort of patients...
May 29, 2018: Clinical Neurology and Neurosurgery
Weihua Mai, Hanwei Liu, Xingwei Liu, Ling Chen, Wenying Zhou
OBJECTIVES: The aim of this study is to investigate the association of HDL-C with CCAS, as well as its intracranial or extracranial location in the Southern Chinese population. PATIENTS AND METHODS: 123 Southern Chinese patients with large-artery atherosclerotic(LAA) ischemic stroke were enrolled for the final analysis. Based on the stenosis severity defined by digital subtraction angiography, the patients were categorized into CCAS and non-CCAS groups. The degree of artery stenosis among patients of CCAS was classified into three grades...
May 29, 2018: Clinical Neurology and Neurosurgery
Panagiotis Kerezoudis, Sandy Goncalves, Joseph D Cesare, Mohammed Ali Alvi, Dennis P Kurian, Arjun S Sebastian, Ahmad Nassr, Mohamad Bydon
OBJECTIVES: Current surgical treatment options for one-time recurrent lumbar disc herniation (RLDH) include repeat discectomy or discectomy supplemented with fusion. Significant contention exists within the surgical spine community with regard to the most effective treatment modality. The objective of this study is to compare reoperation rates and patient reported outcomes following fusion versus repeat discectomy for RLDH. PATIENTS AND METHODS: The electronic literature search was performed in Ovid Medline/Pubmed, EMBASE and Cochrane, Scopus and China National Knowledge Infrastructure for human studies directly comparing repeat discectomy with fusion for ipsilateral or contralateral RLDH...
May 29, 2018: Clinical Neurology and Neurosurgery
Timothy Bardouille, Lindsey Power, Marc Lalancette, Ronald Bishop, Steven Beyea, Margot J Taylor, Benjamin T Dunkley
OBJECTIVES: Magnetoencephalography (MEG) provides functional neuroimaging data for pre-surgical planning in patients with epilepsy or brain tumour. For mapping the primary somatosensory cortex (S1), MEG data are acquired while a patient undergoes median nerve stimulation (MNS) to localize components of the somatosensory evoked field (SEF). In clinical settings, only one MEG imaging session is usually possible due to limited resources. As such, it is important to have an a priori estimate of the expected variability in localization...
May 26, 2018: Clinical Neurology and Neurosurgery
Ana Aires, Tiago Gomes, Paulo Linhares, Filipe Cunha, Maria José Rosas, Rui Vaz
Meige Syndrome (MS) is a disabling movement disorder which impairs daily routines such as eating and speaking and, when not responsive to best medical treatment, deep brain stimulation (DBS) of the globus pallidus interna (GPi) has been considered. Previous evidence has shown a significant improvement in motor dysfunction with DBS, however its benefit on disease-specific disability and quality of life has not been thoroughly studied. We describe two patients with severe MS submitted to GPi-DBS. Patient improvement was assessed preoperatively and 24 months after the surgery by applying the movement subscore of the Burke-Fahn-Marsden Dystonia Rating Scale (BFMRS) and Jankovic Rating Scale (JRS) for motor function and the BFMRS disability subscore and Blepharospasm Disability Scale (BDS) for disability...
May 25, 2018: Clinical Neurology and Neurosurgery
Susana Pinto, Mamede de Carvalho
OBJECTIVES: We investigated two different methodological approaches for determining nasal inspiratory pressure during a sniff (SNIP) in patients with Amyotrophic Lateral Sclerosis (ALS). PATIENTS AND METHODS: We included 37 ALS patients and 11 controls. SNIP was measured in the sitting position, performed in each nostril with (SNIPocclud) and without (SNIPopen) closing the contralateral nostril. The best of 3 consistent results was considered for analyses. Patients were also assessed with MIP and the revised functional ALS rating scale (ALSFRS-R)...
May 24, 2018: Clinical Neurology and Neurosurgery
Sergio Loaiza, Sonia Carvajal, Daniel Giraldo, Astrid Galvis, Leon Ortiz
OBJECTIVES: Advances in therapeutic schemes for High Grade Gliomas, such as the introduction of Stupp protocol, have raised interest in elucidating its effects on quality of life and cognitive function. This study aims to examine executive functions, memory and attention in patients with High Grade Gliomas, before initiation and after completion of Stupp protocol. PATIENTS AND METHODS: A before-after study was executed. Patients were selected from a consecutive sample of subjects with High Grade Gliomas who had not initiated Stupp protocol...
May 23, 2018: Clinical Neurology and Neurosurgery
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