Neurology
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Batten disease (juvenile neuronal ceroid lipofuscinosis [JNCL]) is an autosomal recessive neurodegenerative disorder characterized by blindness, seizures, and relentless decline in cognitive, motor, and behavioral function. Onset is in the early school years, with progression to death typically by late adolescence. Development of a clinical instrument to quantify severity of illness is a prerequisite to eventual assessment of experimental therapeutic interventions. ⋯ The Unified Batten Disease Rating Scale (UBDRS) is a reliable instrument that effectively tests for neurologic function in blind and demented patients. In its current form, the UBDRS is useful for monitoring the diverse clinical findings seen in Batten disease.
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To compare brain activity identified by fMRI in subjects with Alzheimer disease (AD) and older healthy controls (HCs) performing an episodic/working memory (EWM) and semantic memory (SM) task. ⋯ Subjects with Alzheimer disease (AD) recruited brain regions for easier episodic/working memory (EWM) tasks used by healthy controls (HCs) for more difficult EWM tasks. AD subjects recruited brain regions for semantic memory tasks used by HCs for more difficult EWM tasks. The authors propose a functional "memory reserve" model of compensatory recruitment according to task difficulty and underlying neuropathology.
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Atherosclerosis may be linked to cognitive decline and depression in old age. ⋯ In the population at large, generalized atherosclerosis contributes to cognitive decline in old age but not to depression.
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Hypocretin-1 is involved in the regulation of the sleep-wake cycle. The authors prospectively assessed CSF hypocretin-1 levels in 44 consecutive patients with acute traumatic brain injury (TBI). Compared with controls, hypocretin-1 levels were abnormally lower in 95% of patients with moderate to severe TBI and in 97% of patients with posttraumatic brain CT changes. Hypocretin-1 deficiency after TBI may reflect hypothalamic damage and be linked with the frequent development of posttraumatic sleep-wake disorders.