Aphasia or dementia: the cautionary tale of Dr. JJ.

This case study involves the differential diagnosis of aphasia versus dementia. However, the case of Dr. JJ is instructive from a number of aspects, presented here as "cautionary notes" which are intended to remind clinicians of sometimes-forgotten interpersonal issues in the diagnosis of disorders of speech and language. This case also is intended to illustrate that merely distinguishing between dementia and aphasia is sometimes inadequate, particularly when potentially transmittable (and in some cases, curable) dementias might be involved.

Serum autoantibodies in patients with Alzheimer's disease and vascular dementia and in nondemented control subjects.

BACKGROUND AND PURPOSE: In this study we sought to evaluate the clinical significance of serum autoantibodies to dementing processes. METHODS: We assessed 40 age-matched subjects: 10 patients with probable Alzheimer's disease, 10 with possible Alzheimer's disease with cerebrovascular disease, 10 with vascular dementia, and 10 nondemented control subjects. Serum from each subject was tested for the presence of antithyroglobulin antibody, thyroid antimicrosomal antibody, gastric anti-parietal cell antibody, anti-smooth muscle antibody, antinuclear antibody, rheumatoid factor, antineuronal antibody, and anticardiolipin antibody. In addition, we investigated the sera of these patients for the presence of an antivascular antibody directed against the vascular basement membrane proteoglycan antigen and for circulating immune complexes. RESULTS: Autoantibodies were present in 100% of the patients with possible Alzheimer's disease with cerebrovascular disease, 80% of those with vascular dementia, 40% of those with probable Alzheimer's disease, and 30% of the nondemented control subjects. The highest number of autoantibodies was observed in patients with vascular dementia and possible Alzheimer's disease with cerebrovascular disease. Antinuclear antibody was present in 60% of vascular dementia patients and antineuronal antibody in 50% of these patients. However, no individual autoantibody could differentiate Alzheimer's disease from cerebrovascular disorders. Immune complexes were detected in the serum of 20-30% of each patient group. Neither the patient nor the control sera was found to contain antiendothelial antibody. CONCLUSIONS: Despite the relatively small number of individuals examined in each category, the elevated number of autoantibodies associated with possible Alzheimer's disease with cerebrovascular disease and vascular dementia indicates a possible link between the presence of autoantibodies and cerebrovascular disorders in dementia.

Reliability of NINCDS-ADRDA clinical criteria for the diagnosis of Alzheimer's disease.

Although the NINCDS-ADRDA Work Group has recently developed uniform clinical criteria for the diagnosis of Alzheimer's disease (AD), inter-rater reliability studies of these criteria are few. We report a study in which 2 neurologists and 2 psychiatrists independently reviewed clinical data abstracted from the records of 30 demented subjects and 10 nondemented control subjects participating in a longitudinal study of AD at the University of Pittsburgh. We recorded the clinical data on a standardized form; the subjects' identity and clinical and pathologic diagnoses were omitted. Each physician diagnosed each case according to the NINCDS-ADRDA criteria. We calculated the inter-rater agreement for all possible 2-way combinations of clinicians with the Kappa statistic, which ranged from 0.36 (fair agreement) to 0.65 (substantial agreement). We conclude that current NINCDS-ADRDA criteria enable moderate levels of agreement among clinicians in general.

Epileptic nystagmus.

We report a 52-year-old woman with episodes of vertigo accompanied by right beating nystagmus and left posterior temporoparietal sharp waves and spikes on electroencephalogram without change in level of consciousness. Electrooculography demonstrated that the episodes of nystagmus were not preceded by gaze deviation and that nystagmus slow component velocities were linear. This patient's epileptic nystagmus may have been the result of excitation of cerebral ocular pursuit pathways.

The neurologic manifestations of fat embolism.

We reviewed the clinical features of 12 patients with fat embolism seen in 10 years. Based on neurologic manifestations, patients were grouped into those with focal cerebral syndromes and those with diffuse encephalopathy. Patients with focal signs tended to present earlier with neurologic findings alone, did not have fever, and had less risk of pulmonary disorder than patients with encephalopathy.

Recurrent cerebral infarctions in two brothers with antiphospholipid antibodies that block coagulation reactions.

Inhibitors blocking coagulation reactions, often called lupus anticoagulants, are readily identifiable but rarely considered as risk factors for cerebral infarction. These inhibitors are inconsistently found in a number of diseases (often autoimmune) and after treatment with ceretain drugs and appear to be closely associated with, or identical to, antibodies to certain phospholipids. We have observed two brothers with these inhibitors who both experienced recurrent cerebral infarctions. Such familial occurrence has rarely been reported. In addition, some other family members were found to have depressed factor XII levels. Using the technique of double immunodiffusion, we found that the serum from these brothers formed precipitin lines against certain phospholipid substrates, lending further support to the antiphospholipid nature of this inhibitor.

Various consequences of subcortical stroke. Prospective study of 16 consecutive cases.

Sixteen consecutive cases of subcortical stroke were studied prospectively and systematically. Behavioral and neurological manifestations of different sides, sites, and causes of lesion were evaluated by means of daily, standard observations during the patient's hospitalization and formal testing at monthly intervals thereafter. Language and cognitive impairments were observed following hemorrhagic and nonhemorrhagic strokes to both the left and right thalami and basal ganglia. The patterns were unlike those characteristic of traditional aphasia syndromes. One patient was asymptomatic; four demonstrated only dysarthria or dysarthria with abnormal affect; and 11 displayed a combination of speech, language, and cognitive deficits. Language skills recovered more rapidly and completely than did cognitive skills. Recovery was most dramatic within the first six to eight weeks after onset.

Stroke due to vertebral artery injury.

Minimal neck injury produced vertebral artery damage in two women and three men, 20 to 57 years of age. Serious neurologic deterioration occurred in two patients when positioned for roentgenographic procedures. Dissection or thrombosis of one or both vertebral arteries while in the transverse foramina occurred in three, one at the site of an osteophyte. The arteriogram of one patient showed a traumatic aneurysm at C-7, and a second patient had segmental arterial stenosis with bilateral traumatic aneurysms at C-5. The location of the arterial injury is not restricted to the occipitoatlantoaxial level but includes the course through the transverse foramina. The use of anticoagulation appears to be important in the immediate management of this process.

Rapid recovery from aphasia: a detailed language analysis.

The paper presents a daily analysis of the language recovery of a patient who was globally aphasic at the time of her first observation and who had recovered language, as measured by the Western Aphasia Battery, at the time of her discharge 14 days later. The paper emphasizes the relatively regular growth of normal syntactic, lexical, and pragmatic features coupled with similar regular decreases in aphasic features. Observations of both phenomena are necessary to describe language recovery.

The dissolution of language in Pick's disease with neurofibrillary tangles: a case study.

This paper is a detailed retrospective history of a patient who began to have difficulty with speaking and comprehension in 1967, and whose neuropathological examination at time of death 12 1/2 years later was consistent with a diagnosis of Pick's disease, complicated by neurofibrillary tangles. It follows the deteriorating course of his language abilities in the context of relatively less-impaired general cognitive abilities, using two sources of information. The first is his own written record of his deteriorating abilities, shown in letters and notes he wrote over this period. The second is through an oral history obtained from the patient's family. The purpose of this report is to illustrate in detail the course of a degenerative condition and to suggest the utility of such descriptional records in increasing understanding of language deterioration in dementia.

Clinical experience with the use of xenon-enhanced CT blood flow mapping in cerebral vascular disease.

Cerebral blood flow mapping with the xenon-enhanced/CT method has become a useful clinical tool in the management of patients with occlusive cerebral vascular disease. Studies involving 4-5 minutes of inhaling a xenon/oxygen mixture (less than or equal to 35%) can now be performed routinely with acceptable patient tolerance and compliance. Four cases with acute and chronic ischemic injuries are reported here to illustrate the manner in which this method has been used to characterize flow pattern in such patients and the relevance of this flow information to clinical patient management.

Chronic subdural hematoma with transient neurological deficits: a review of 15 cases.

Fifteen patients with chronic subdural hematomas had transient neurological deficits. The important clinical features were aphasia in 9 patients, absence of headache in 8, and head injury in 8. Computed tomographic scan correctly identified all 15 cases of chronic subdural hematoma and should be considered mandatory before initiating anticoagulation in patients with transient ischemic attacks.

Cerebral blood flow studies in man using recirculation corrected height/area (H/A) computations for intravenous injection of 133Xenon.

Our height/area (recirculation-corrected) non-invasive cerebral blood flow method compares well with k1 values and k average as determined from k1, k2, p1 and p2 by Obrist computations. The height/area method provides regional flow measurements with less variability than k1 due to low regional head counts, head motion and end-tidal gas sample variations. Derivation of the mathematical model is for a single flow compartment and suited for tomographic measurements such as PET. Collimated detector data using our computation provides a good approximation of flow even for multiple compartments. Our approach permits a lower counting rate by either a smaller isotope dose or better detector collimation than multicompartment curve fitting methods.