Biomedical subjects
Publications and source records attributed to R Mayeux.
Nearly every patient with a movement disorder has some type of behavioral dysfunction, whether it is personality change or intellectual impairment. Since these behaviors have confounding effects on the management of patients, it behooves clinicians to maintain awareness of the spectrum of mental changes in these diseases of the basal ganglia.
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The concept of "subcortical dementia" is controversial, lacking clinical validation and having only a questionable pathological basis. Over 100 patients with Alzheimer's, Parkinson's or Huntington's disease, subdivided into three functional disability stages, were given a brief quantitative neuropsychological assessment. Patients with Huntington's or Parkinson's disease were less intellectually impaired than those with Alzheimer's disease at each functional stage. Criteria for dementia were present in all of the Alzheimer's patients but in only half of the Huntington's and Parkinson's disease groups. Patients with similar overall intellectual function scores had no distinct pattern of neuropsychological test performance. Depression, absent in patients with Alzheimer's disease, was present in half the patients with Huntington's and Parkinson's disease and was correlated with intellectual decline. The concept of subcortical dementia is misleading. The pattern of neuropsychological impairment is not distinct, and the neuropathological basis of dementia in these diseases may result from a combination of cortical and subcortical degeneration.
We studied the ability of Parkinsonian patients and controls to generate voluntary movements on a tracing task. Subjects were videotaped while tracing designs of increasing complexity, presented on a vertical, transparent screen. Some designs were presented in a degraded form and subjects filled in their missing sections. Subjects also received a constructional task and a test of general intellectual ability. The quality of errors on the tracing task differed in the Parkinsonian and control groups. Parkinsonian patients made two distinct types of errors. One probably related to the motor disorders of the disease, but another seemed to be related to a higher level of control over sequential and predictive movements. The latter correlated with performance on the constructional and general intellectual tasks. These results suggest that Parkinson's disease may affect basal ganglia structures that are necessary for voluntary movements which require sequencing or planning. Clinically this may be observed in perceptual motor tasks since they require both voluntary movement and sequential organisation of behaviour.
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A patient with paroxysmal dystonic choreoathetosis also had familial ataxia. His brother was similarly affected but had rare paroxysmal episodes. No secondary or symptomatic forms of this type of paroxysmal dyskinesia have ever been reported. Episodes were briefly controlled with acetazolamide and were almost completely eliminated by clonazepam therapy.
Articulatory disturbances are frequently described in Parkinson disease, but language disorders are not. We have occasionally encountered parkinsonian patients with word-finding difficulty unrelated to memory loss, intellectual impairment, or dysarthria. To examine this, 22 medically stable parkinsonian patients were given the vocabulary subtest of the WAIS, the Boston Naming Test, measures of verbal fluency, and sentence repetition. Signs and symptoms of parkinsonism were rated. WAIS vocabulary subtest scores were above the mean for normal aged subjects, but confrontation naming was one standard deviation below norms for age and education. Naming was facilitated by cues in most patients. Only sentence repetition correlated with dysarthria. Category naming was impaired and correlated significantly with the severity of parkinsonism, especially bradykinesia. This suggests that a type of anomia may occur in Parkinson disease. it shares the clinical characteristics of the "tip-of-the-tongue" phenomenon and "word production anomia" seen in some aphasics.
To determine the prevalence of depression in Parkinson disease (PD) we evaluated 55 consecutive patients without dementia and 31 of their spouses. All subjects completed the Beck depression inventory and a quantitative mini-mental state examination. Using the Beck criteria, 47% of the patients and 12% of the spouses rated themselves as significantly depressed. Mental state scores were significantly lower in the patients. There was a correlation between the severity of depression and cognitive impairment, particularly for calculation, digit span, and visuomotor skills. The severity of parkinsonism, particularly bradykinesia, also paralleled cognition. There was a slight but significant relationship between parkinsonism and depression. These results confirm the high incidence of depression in PD, and suggest that depression in Parkinson patients may be accompanied by mild intellectual impairment and inattention which is independent of the severity of the illness.
Prior reports of neuroleptic malignant syndrome (NMS) concerned patients with psychiatric disorders, usually schizophrenia, who were taking dopamine receptor blocking agents. We report the syndrome in a patient with Huntington disease who was treated with dopamine-depleting agents. He had a negative evaluation for malignant hyperthermia (MH), and we suggest that NMS differs from MH. The occurrence of NMS caused by dopamine-depleting agents suggests that anticholinergic properties of phenotiazines are not the only cause. Central dopaminergic systems probably participate in thermoregulation, and dopamine depletion probably plays a pathogenetic role in this syndrome.
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A unique case reported of a patient with right-sided Parkinson's disease and left-sided tardive dyskinesia. This situation occurred because the patient's parkinsonian tremor was treated with antipsychotic drugs. After several months she developed tardive dyskinesia on the left side of the body. Successful treatment was achieved nine years later, using dopamine-depleting drugs (combination reserpine and alpha-methylparatyrosine) to suppress the tardive dyskinesia and trihexyphenidyl to reduce the parkinsonism. Control of the symptoms was complicated with parkinsonism symptoms later increased on the right and developed on the left, due to the dopamine-depleting drugs. A small amount of carbidopa/levodopa restored the proper balance of symptoms, effectively reducing the parkinsonism while not aggravating the tardive dyskinesia. This unique case provides insight into the pathogeneis of Parkinson's disease, the pathogenesis of tardive dyskinesia, their successful therapeutic approaches, and possibly the effect of drugs in blocking the progression of Parkinson's disease.
Memory and language were evaluated in patients with temporal lobe epilepsy and generalized epilepsy. Subjects were matched for age, duration of illness, and seizure frequency, and grouped according to the electroencephalographic results and seizure type into right temporal, left temporal, and generalized. In formal tests of intelligence, auditory and visual memory, and language, a significant difference was noted only on a confrontation naming test. The mean score on this test was considerably lower in the left temporal group; right temporal and generalized groups scored in the normal range. This correlated with impairment on many verbal subtests of intelligence and memory. These results suggest that the interictal memory impairment of temporal lobe epilepsy may be an anomia and that the anomia may contribute to impairment of verbal learning and memory; both circumlocution and circumstantiality may compensate for anomia.
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Three patients with complex partial seizures experienced prolonged episodes of aimless wandering followed by retrograde amnesia for the experience, a phenomenon called poriomania. Our experience and the literature suggest that this behavior is a prolonged postictal automatism and is not psychogenic. Adjustments of anticonvulsant therapy eliminated this behavior in all three of our patients.
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