Apolipoprotein E allele frequencies in argyrophilic grain disease

Glanzmann's thrombasthenia is a rare inherited hematological disorder defined by deficiency or abnormality of the glycoprotein (GP) IIb-IIIa complex. Presenting symptoms are hemorrhagic events, mainly epistaxis, purpura, or menorrhagia. We describe the clinical course and management of a 14-year-old girl with Glanzmann's thrombasthenia and severe menorrhagia. Following treatment with 20 U of packed red blood cells, 37 U of platelets, 7 U of fresh frozen plasma, cryoprecipitate, intravenous estrogens, and methylergotrine maleate with no improvement, the uterine cavity was packed for 48 hr. This unusual procedure halted the bleeding and avoided the necessity for a hysterectomy. When treating acute menorrhagia in patients with Glanzmann's thrombasthenia, the physician should be familiar with the characteristics and all treatment modalities for this disorder.     

A comparison of category and letter fluency in Alzheimer's disease and Huntington's disease.

44 patients with dementia of the Alzheimer type (DAT), 44 elderly normal control (ENC) Ss demographically matched to the DAT group, 42 patients with Huntington's disease (HD), and 42 middle-aged normal control (MNC) Ss demographically matched to the HD group were administered letter and category fluency tasks. DAT patients showed an overproportional impairment on category than on letter fluency tasks, whereas HD patients were equally impaired. Analyses based on receiver operating characteristic curves revealed that category fluency correctly classified significantly more DAT and ENC Ss than did letter fluency, whereas the 2 fluency tasks did not differ in this respect for HD and MNC Ss. Results suggest that HD patients' failures on fluency tasks are caused by impaired initiation/retrieval capacities. In contrast, DAT patients' greater category than letter fluency deficits are primarily due to a breakdown in the structure of semantic knowledge. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Norms for change in episodic memory as a prerequisite for the diagnosis of mild cognitive impairment (MCI).

The new diagnostic criteria for mild cognitive impairment (MCI) from the International Working Group on Mild Cognitive Impairment (Winblad et al., 2004, p. 243) list “evidence of decline over time in objective cognitive tasks” as one diagnostic sign, implying the repeated neuropsychological testing. This study aimed to compare different assessment methods of longitudinal change based on the performances of 366 cognitively healthy participants (237 men, 129 women) examined with a German version of the California Verbal Learning Test (Delis, Kramer, Kaplan, & Ober, 1987) at baseline and 2 years later. Age, education, gender, and baseline performance were taken into account. Results revealed marked practice effects after 2 years. Normal ranges for change that controls for practice effects and regression to the mean proved to be superior to other reliable change indexes. This new method allows for more valid interpretation of change in neuropsychological functioning and thus diagnosis of MCI. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Preclinical cognitive markers of dementia of the Alzheimer type.

56 nondemented elderly normal control (NC) Ss were studied at 3 consecutive annual administrations of the California Verbal Learning Test (CVLT). NC Ss with a positive family history for progressive dementia performed significantly worse than individuals with a negative family history for progressive dementia on several quantitative and qualitative indices of the CVLT and were more likely to undergo changes in diagnostic status over time (i.e., develop dementia of the Alzheimer type [DAT]). Stepwise discriminant function analyses of critical CVLT indices of the NC Ss and of 25 patients with mild DAT classified 5 NC Ss as DAT patients 1–2 yrs prior to their eventual changes in diagnostic status. Results suggest that specific memory deficits may serve as preclinical cognitive markers for DAT, especially in individuals with risk factors for DAT such as a positive family history. (PsycINFO Database Record (c) 2010 APA, all rights reserved)