Objective: To evaluate the utility of mismatch negativity (MMN), a neurophysiologic marker of non-motor cognitive processing, in amyotrophic lateral sclerosis (ALS).
Methods: 89 patients, stratified into 4 different phenotypic presentations of ALS (67 spinal-onset, 15 bulbar-onset, 7 ALS-FTD, 7 gene careers), and 19 matched controls underwent 128-channel EEG data recording. Subjects were presented with standard auditory tones interleaved with pitch-deviant tones in three recording blocks.
We describe a unique patient who experienced a progressive autoimmune coma from age 14 to 17. The patient awoke after treatment with immunosuppressant medication. Although alertness, verbalization, and mobilization markedly improved, the patient reported persistent cognitive difficulties.
View Article and Find Full Text PDFNeuropsychologia
February 2013
Awareness of deficits after brain injury represents a significant clinical and theoretical challenge, but relatively little is known about the neuroanatomical correlates of specific types of deficit awareness. We examined the awareness correlates of left versus right prefrontal cortex lesions in comparison to left and right posterior lesions including two types of awareness measures--metacognitive and online error monitoring. Frontal lobe frontal lesion patients exhibited impaired metacognitive awareness and also showed deficits in monitoring errors as they occurred.
View Article and Find Full Text PDF22q11.2 Deletion Syndrome (22q11DS) is a common microdeletion disorder associated with mild to moderate intellectual disability and specific neurocognitive deficits, particularly in visual-motor and attentional abilities. Currently there is evidence that the visual-motor profile of 22q11DS is not entirely mediated by intellectual disability and that these individuals have specific deficits in visual-motor integration.
View Article and Find Full Text PDF129 Wada procedures were reviewed to examine the suitability of propofol (n=54) as a replacement to amobarbital (n=75) for use as an anaesthetic in the Wada test. Suitability was considered with respect to length of hemiplegia induced, the frequency of side effects and patient memory scores. Data was retrospectively collected from records of patients who had undergone the Wada procedure between 2004 and 2009 in Beaumont Hospital, Dublin.
View Article and Find Full Text PDFAmyotroph Lateral Scler
August 2010
We present the clinical, electrophysiological and molecular genetic findings of a 58-year-old male with genetically confirmed Huntington's disease (HD) and concurrent clinically definite ALS by El Escorial criteria. The patient presented with asymmetric upper limb amyotrophy and weakness, and subsequently developed chorea and cognitive change. Genetic testing confirmed the presence of expanded trinucleotide repeats in huntingtin, consistent with a diagnosis of Huntington's disease.
View Article and Find Full Text PDFAmyotrophic lateral sclerosis (ALS) is a motor neuron disease that has sporadic and inherited forms. ALS is the most common neurodegenerative disorder of young and middle-aged adults, and few treatments are available. Although the degeneration predominantly affects the motor system, cognitive and behavioural symptoms have been described for over a century, and there is evidence that ALS and frontotemporal dementia overlap clinically, radiologically, pathologically, and genetically.
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