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International Journal of Clinical and Experimental Neurology

ISSN (Print): 2379-7789

ISSN (Online): 2379-7797

Editor-in-Chief: Zhiyou Cai, MD

Website: http://www.sciepub.com/journal/IJCEN

   

Article

Neuropsychiatric Symptoms of Urbach-Wiethe Disease

1School of Psychology, Kean University, Union, NJ, USA

2Department of Nursing, Kean University, Union, NJ, USA


International Journal of Clinical and Experimental Neurology. 2015, 3(2), 45-50
doi: 10.12691/ijcen-3-2-3
Copyright © 2015 Science and Education Publishing

Cite this paper:
Richard P. Conti, Jacqueline M. Arnone. Neuropsychiatric Symptoms of Urbach-Wiethe Disease. International Journal of Clinical and Experimental Neurology. 2015; 3(2):45-50. doi: 10.12691/ijcen-3-2-3.

Correspondence to: Richard  P. Conti, School of Psychology, Kean University, Union, NJ, USA. Email: rconti@kean.edu

Abstract

Urbach–Wiethe (or lipoid proteinosis) disease (UWD) is a rare autosomal recessive disorder characterized by dermatological, psychiatric, and neurological symptoms. Presentation occurs during childhood, but can be observed from birth. While benign, the disease is progressive and chronic with no known cure. Treatment modalities are palliative for symptoms. The extant literature consists mainly of anecdotal reports and case studies that are limited by small sample sizes and paucity of controlled studies. Incidence and prevalence rates are unknown. There are less than 500 documented cases reported worldwide, and of those, less than 50 cases demonstrate neurological and neuropsychiatric conditions. Worldwide occurrence of the disease is documented, with the largest cohort living in a remote area of South Africa. The affected individuals are mainly Caucasian, born to consanguineous parents, and from Dutch or German heritage. Patients affected have been reported in China, Pakistan and Iran. Current and earlier studies focus primarily on the most visible signs of disease, dystonia and dermatological symptoms, while other studies have reported calcification in the amygdala, hippocampus, parahippocampal gyrus, and the striatum. While central nervous system involvement can lead to a wide range of clinical manifestations such as epilepsy and neuropsychiatric symptoms, there is not a consensus of reported cases with amygdala calcifications accompanied by neurological symptoms. Quantitative research is warranted to further identify the role and relationship between amygdala calcification and neurologic and neuropsychiatric symptoms, while qualitative research will afford insights into the lived experience of individuals and families living with UWD.

Keywords

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Article

Age-related Volumetric Changes of Prefrontal Gray and White Matter from Healthy Infancy to Adulthood

1Department of Psychology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan

2Department of Pediatrics, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan

3Department of Radiology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan

4Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, Pennsylvania, USA

5Department of Psychiatry, University of Pennsylvania, Philadelphia, Pennsylvania, USA


International Journal of Clinical and Experimental Neurology. 2016, 4(1), 1-8
doi: 10.12691/ijcen-4-1-1
Copyright © 2016 Science and Education Publishing

Cite this paper:
Mie Matsui, Chiaki Tanaka, Lisha Niu, Kyo Noguchi, Warren B. Bilker, Michael Wierzbicki, Ruben C. Gur. Age-related Volumetric Changes of Prefrontal Gray and White Matter from Healthy Infancy to Adulthood. International Journal of Clinical and Experimental Neurology. 2016; 4(1):1-8. doi: 10.12691/ijcen-4-1-1.

Correspondence to: Mie  Matsui, Department of Psychology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Japan. Email: mmatsui@las.u-toyama.ac.jp

Abstract

Despite increasing evidence of the role of the prefrontal cortex in providing the neural substrate of higher cognitive function and neurodevelopment, little is known about neuroanatomic changes in prefrontal subregions during human development. In this prospective study, we evaluated prefrontal gray and white matter volume in healthy infants, children, adolescents, and adults. Magnetic resonance imaging was performed on 107 healthy people aged one month to 25 years. Gray and white matter volumes of the dorsolateral, dorsomedial, orbitolateral, and orbitomedial prefrontal cortex were quantified. The results indicated that both children and early adolescents had larger dorsolateral gray matter volume than infants and adults. Dorsolateral white matter volumes in children, early adolescents, and late adolescents were larger than those of infants. Dorsomedial white matter volumes of early adolescents, late adolescents, and adults were also larger than those of infants. There was no significant difference among age groups in both orbital prefrontal regions. These findings suggest that there are two important stages of structural change of the prefrontal cortex from infancy to young adulthood. First, growth spurts of both gray matter and white matter during the first 2 years of life have been shown to occur specifically in the dorsal prefrontal cortex. Second, gray matter changes have been shown to be regionally specific, with changes in the dorsal, but not orbital, prefrontal cortex peaking during late childhood or early adolescence. Thus, developmental differences within sectors of the prefrontal lobe and evidence of neural pruning and myelination may be useful in understanding the mechanisms of neurodevelopmental disorders.

Keywords

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Article

Neurological Complications in Sickle Cell Disease

1Consultant Hematologist, Kuwait, Amiri hospital

2Consultant Neurologist & Director of Multiple Sclerosis clinic at Alamiri Hospital, Kuwait

3Senior specialist in Radiologist, Amiri Hospital

4Consultant Neurologist, Kuwait, Mubark Al-Kabeer Hospital


International Journal of Clinical and Experimental Neurology. 2016, 4(1), 9-18
doi: 10.12691/ijcen-4-1-2
Copyright © 2016 Science and Education Publishing

Cite this paper:
Hassan A. Al-Jafar, Raed Alroughani, Thamer A.Abdullah, Fatma Al-Qallaf. Neurological Complications in Sickle Cell Disease. International Journal of Clinical and Experimental Neurology. 2016; 4(1):9-18. doi: 10.12691/ijcen-4-1-2.

Correspondence to: Hassan  A. Al-Jafar, Consultant Hematologist, Kuwait, Amiri hospital. Email: Cbc9@hotmail.com

Abstract

Sickle cell disease is a common inherited blood disorder that affects red blood cells. It is a hemoglobinopathy characterized by hemoglobin polymerization, erythrocyte stiffening, and subsequent vaso-occlusions. These changes can lead to microcirculation obstructions, tissue ischemia, infarction and acute stroke. In addition, chronic cerebral ischemia and cerebral vascular anomalies are considered among the most disabling problems in sickle cell disease. Neurological complications of sickle cell disease include, Ischaemic Stroke, hemorrhagic stroke, transient ischemic attack, silent cerebral infarction, headache, Moyamoya disease, neuropathic pain, and neurocognitive impairment. Early diagnosis and proper management of sickle cell disease neurological complications require specialised hematological and neurological experties. The newly used medications under ongoing research foster the hope to overcome this devastating disease and its complications.

Keywords

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