François Boiler

Diagnosis of dementia: Clinicopathologic correlations

Based on 54 demented patients consecutively autopsied at the University of Pittsburgh, we studied the accuracy of clinicians in predicting the pathologic diagnosis. Thirty-nine patients (72.2%) had Alzheimers disease, while 15 (27.7%) had other CNS diseases (four multi-infarct dementia; three Creutzfeldt-Jakob disease; two thalamic and subcortical gliosis; three Parkinsons disease; one progressive supranuclear palsy; one Huntingtons disease; and one unclassified). Two neurologists independently reviewed the clinical records of each patient without knowledge of the patients identity or clinical or pathologic diagnoses; each clinician reached a clinical diagnosis based on criteria derived from those of the NINCDS/ADRDA. In 34 (63%) cases both clinicians were correct, in nine (17%) one was correct, and in 11 (20%) neither was correct. These results show that in patients with a clinical diagnosis of dementia, the etiology cannot be accurately predicted during life.

Risk of dementia in relatives of patients with Alzheimer's disease

Using a family history questionnaire, we investigated the occurrence of dementia among relatives of patients with a clinical diagnosis of Alzheimers disease (AD) and among the relatives of age-matched control subjects. Cumulative lifetime risk of developing AD-type dementia was greater among relatives of AD probands and was consistent with an autosomal dominant genetic mode of transmission. Although the lifetime risk of AD-type dementia was similar among relatives of early-onset and late-onset AD probands, relatives of early-onset probands tended to have an earlier onset of dementia than did relatives of late-onset AD probands. This result raises the possibility that age at onset of dementia in AD may be genetically determined.

Mental status of patients with parkinson disease

abstract This paper presents a detailed review of the literature related to the mental status of patients with Parkinson Disease (PD). Even though Parkinson stated that the intellect remains intact, several authors have shown that dementia is found in a sizeable percentage of patients with PD. Several papers written after 1970 suggest that this percentage is between 40 and 50. Both neuropsychological and neuropathological data indicate that the majority of demented PD patients show findings indistinguishable from Alzheimer Disease (AD). This finding is not related to aging and remains unexplained, even though it has been suggested that this mental status change may be related to dopaminergic drugs.

The token test: Validity and diagnostic power in Alzheimer's disease

The Token Test was introduced by DeRenzi and Vignolo (1962) with the intent of providing a sensitive and specific measure of auditory comprehension. Nevertheless, many nonlinguistic factors are also important in determining test performance. We assessed Token Test performance in patients classified as “probable Alzheimers disease” according to National Institute of Neurological and Communicative Disorders and Stroke‐Alzheimers Disease and Related Disorders Association criteria. We used a short version of the Token Test, the Mini‐Mental State Examination (MMSE, an indicator of global cognitive integrity), and a battery of tests designed to measure many specific neuropsychological functions. We show that this version of the Token Test correlates weakly with a measure of simple auditory‐verbal comprehension but highly with the MMSE. On the basis of an item analysis, it is suggested that demented patients do poorly on the Token Test mainly due to perseveration rather than due to difficulty with auditory‐ver...

A behavioral evaluation of brain‐scan estimates of lesion size

SUMMARYThe possibility that the brain scan can be used to estimate the magnitude of a cerebral lesion is tested by correlating the volume of a lesion calculated from its scan image with [1] a functional deficit in simple reaction time and [2] its volume measured from pathological data. In 20 patients with unilateral lesions of the right hemisphere, significant correlations were obtained with both auditory and visual reaction times. For cases of primary tumor, the correlations were highest and indicate that reaction time is elevated in direct proportion to lesion volume. An error of about 30% in estimating actual lesion volume from the scan is indicated. For metastases and vascular lesions, the correlations between scan volume and reaction time were positive but not statistically significant. In 23 cases with lesions of the left hemisphere, on the other hand, reaction time and scan volume were not correlated, although reaction times were higher than for normal subjects. In 7 cases of primary tumor that came to autopsy, a correlation of +.87 was obtained between lesion volume estimated from the brain scan and from pathological data.

Repetition in Alzheimer's disease: A longitudinal study

Abstract A longitudinal study of repetition in Alzheimers disease involving 24 patients and 35 controls over 3 years of evaluation is reported. A number of issues are addressed: Repetition ability in AD patients over time, the relationship between overall severity of dementia and performance on repetition tasks, the resemblance between the language of early AD patients and transcortical sensory aphasia symptoms, the dependence on short-term memory of repetition in Alzheimers Disease, the occurrence of linguistic processing during repetition of an oral message, and the bearing of repetition performance in AD on the notion that imitative responses index linguistic knowledge, particularly syntax. The analysis of the collected data suggests that patients with relatively mild AD perform sentence repetition well, and that this ability deteriorates over time, but that in a majority of patients, repetition remains better preserved than many other skills. AD patients do not seem to demonstrate a transcortical aphasia. Short-term memory, auditory comprehension at least to the level of retaining the gist of the sentence, and knowledge of syntax are implicated as explanations for the repetition ability in the face of a more general deterioration of function.

Alzheimer’s disease, cerebral amyloid angiopathy, and dementia of acute onset

abstractThe coexistence of cerebral amyloid angiopathy (CAA) and Alzheimer’s disease (AD) should be considered in the differential diagnosis of cases with acute onset of dementia when other causes have been excluded. We report clinical and neuropathological findings in a 78-year-old man who developed dementia of acute onset with an apparent rapid course three months before his death. Postmortem microscopic examination of the brain revealed senile (neuritic) plaques and neurofibrillary tangles in the hippocampus and cerebral cortex. CAA affected vessels of the neocortex and leptomeninges, most severely in the frontal and parietal areas. (Aging 3: 171–175,1991)