Kevin E Crutchfield

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OBJECTIVE This study's purpose was to obtain a quantitative natural history of the cerebrovascular involvement in Fabry disease. BACKGROUND Fabry disease is an X-linked recessive disorder due to alpha-galactosidase A deficiency. Progressive accumulation of ceramidetrihexoside within the intima and media of cerebral blood vessels causes ischemic lesions in(More)
Fabry disease is a lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A). This enzymatic defect results in the accumulation of the glycosphingolipid globotriaosylceramide (Gb(3); also referred to as ceramidetrihexoside) throughout the body. To investigate the effects of purified alpha-gal A, 10(More)
Childhood ataxia with diffuse central nervous system hypomyelination syndrome (CACH) is a recently described leukodystrophy of unknown etiology. To characterize the neuropathological features and gain insight as to the pathogenesis of this disorder, we studied cerebral tissue from six patients with the CACH syndrome. Evaluation of toluidine blue-stained,(More)
OBJECTIVE The objective of this study is to characterize the brain abnormalities on head MRI of patients with mucolipidosis type IV. BACKGROUND Mucolipidosis type IV is an autosomal recessive lysosomal storage disease of unknown etiology. Patients develop corneal clouding, retinal degeneration, spastic quadriparesis, and mental retardation. Patients with(More)
OBJECTIVE To determine if serum vascular endothelial growth factor (VEGF) and ultrasonic monitoring of vascular dynamics with dynamic vascular analysis at sea level and high altitude correlate with acute mountain sickness symptoms. METHODS Nine volunteers participated in a staged ascent from sea level to 4300 m undergoing complete transcranial Doppler(More)
  • Charles H Tegeler, Kevin Crutchfield, +5 authors Greg Evans
  • Journal of neuroimaging : official journal of the…
  • 2013
BACKGROUND Transcranial Doppler (TCD) ultrasonography has been extensively used in the evaluation and management of patients with cerebrovascular disease since the clinical application was first described in 1982 by Aaslid and colleagues TCD is a painless, safe, and noninvasive diagnostic technique that measures blood flow velocity in various cerebral(More)
BACKGROUND AND PURPOSE There is an unmet need to classify cerebrovascular conditions physiologically and to assess cerebrovascular system performance. The authors hypothesized that by simultaneously considering the dynamic parameters of flow velocity, acceleration, and pulsatility index (PI) (impedance) in individual Doppler spectrum waveforms, they could(More)
Background The underlying pathophysiology in Normal Pressure Hydrocephalus (NPH) most likely involves changes in brain parenchyma, vascular resistance, and CSF circulation resulting in decreased intracranial compliance and diminished cerebral blood flow (CBF). Changes in blood vessel reactivity may play an important role in the pathophysiology and diagnosis(More)
OBJECTIVES Blunt cervical vascular injuries, often missed with current screening methods, have substantial morbidity and mortality, and there is a need for improved screening. Elucidation of cerebral hemodynamic alterations may facilitate serial bedside monitoring and improved management. Thus, the objective of this study was to define cerebral flow(More)
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