Casuistic contribution to the differential diagnosis of the myoclonic syndrome]

Involuntary muscle twitches, which are accompanied by a more or less pronounced effect of motion and which may last for several hours, are a common guiding symptom of a number of neurological syndromes. This paper reports the case of a 24-year-old male who had been suffering since his sixth year from episodic muscular spasms accompanied by attacks of petit mal. These are regarded as representing a myoclonic-epileptic syndrome. In this connection, a distinction has to be made, by differential diagnosis, between the "true" myoclonic syndrome which is characterized by the absence of complications due to attacks of grand mal or petit mal, Kojewnikoff's epilepsy, and the myoclonic-epileptic dementia form of syndrome.     

The usefulness of the Western Aphasia Battery for differential diagnosis of Alzheimer dementia and focal stroke syndromes: preliminary evidence.

We assessed the usefulness of the Western Aphasia Battery for distinguishing the language disturbances caused by Alzheimer dementia (AD) from those caused by stroke. Using discriminant function analyses, the multiple variable "aphasia quotient--reading quotient--writing quotient" classified 29 (72.5%) of the 40 patients correctly. These 29 patients included 8 of 10 patients with left hemisphere infarction and fluent aphasia; 6 of 10 with AD; 5 of 10 patients with right hemisphere infarction; and all 10 of the neurologically normal control subjects. The patients with AD and those with right hemisphere stroke were the most difficult to classify using the aphasia battery.     

Diagnosis and differential diagnosis of myasthenia gravis]

The author describes the possibilities of diagnosis and differential diagnosis of myasthenia gravis. The data is based on the literature and on case reports of personal observations of more than 270 patients with myasthenia. He inaugurates his own classification of myasthenia comprising of 8 types, among which there is one particular clinical type, described first by the author. The different problematic types of myasthenia are discussed together with the most important diagnostic tests and the principles of differential diagnosis. The clinical and pathological study is completed by illustrations.     

Systems measures of word use and differential diagnosis

This paper presents findings on the power of two systems measures of word use to discriminate four patient groups. The first is the slope of accumulated entropy or complexity of the sample (known to be logarithmic over time). The other is the average Poisson rate for the use of new words (known to be a Poisson process). The four groups consisted of cancer, depressed, paranoid, and somatization patients. The two word measures discriminate the first three groups from the fourth. There is evidence that larger samples will identify a similar discriminator for each of the first three groups from one another. The results are discussed in terms of how these methods may offer fresh insight into the relationship between language use and psychopathology.     

Facioscapulohumeral neuromuscular syndromes--problems of differential and early diagnosis and genetic counseling

Among 20 kinships affected by facioscapulohumeral and scapulohumeroperoneal neuromuscular syndromes 6 probands with autosomal dominant transmission and 5 obviously sporadic cases revealed myopathic changes on biopsy and were classified to have facioscapulohumer (operone)al muscle dystrophies. 5 autosomal dominant cases and one sporadic case with neurogenic muscle changes and 3 other non-dystrophic phenocopies emphasize the necessity of muscle biopsy for nosological assignment. Definite correlations between mode of inheritance, clinical and morphological findings could not be found. Pathological ultrasound and EMG findings in parents and children of seemingly sporadic cases suggest to assume autosomal dominant inheritance with incomplete penterance in such cases.     

Some suggestions on epileptic psychoses, their differential diagnosis and therapy

From a present point of view, some clinical suggestions are put forward concerning the symptomatology, differential diagnostics, and therapy of psychotic states in epileptic patients (twilight states, states of emotional deterioration and epileptic psychoses proper).     

SD-squared: on the association between semantic dementia and surface dyslexia

Within the connectionist triangle model of reading aloud, interaction between semantic and phonological representations occurs for all words but is particularly important for correct pronunciation of lower frequency exception words. This framework therefore predicts that (a) semantic dementia, which compromises semantic knowledge, should be accompanied by surface dyslexia, a frequency-modulated deficit in exception word reading, and (b) there should be a significant relationship between the severity of semantic degradation and the severity of surface dyslexia. The authors evaluated these claims with reference to 100 observations of reading data from 51 cases of semantic dementia. Surface dyslexia was rampant, and a simple composite semantic measure accounted for half of the variance in low-frequency exception word reading. Although in 3 cases initial testing revealed a moderate semantic impairment but normal exception word reading, all of these became surface dyslexic as their semantic knowledge deteriorated further. The connectionist account attributes such cases to premorbid individual variation in semantic reliance for accurate exception word reading. These results provide a striking demonstration of the association between semantic dementia and surface dyslexia, a phenomenon that the authors have dubbed SD-squared.     

Clinical tests of memory in dementia, depression, and healthy aging

In Study 1, carefully screened elderly adults with primary degenerative dementia or major depression were compared to healthy aged control subjects on three tests of learning and memory: the Benton Visual Retention Test, Inglis Paired-Associate Learning Test, and the Fuld Object-Memory Evaluation (OME). The sharpest distinction in performance among the groups was observed on the OME, and discriminant equations based on this test correctly classified a high percentage (greater than or equal to 90%) of participants. Study 2 applied the classification rules derived in the first investigation to an unselected series of geropsychiatry inpatients referred for neuropsychological evaluation. There was agreement between memory test classification and general categories of clinical discharge diagnosis (organic vs. functional) for 21 of 25 patients, and with status at follow-up approximately 18 months later. Predictive value computations suggested that the OME is more accurate in confirming true dementia than in detecting dementia syndromes associated with functional disorders.     

Relationship between anxiety and premenstrual syndrome

The relationship between symptoms of the premenstrual syndrome and anxiety was assessed for 68 women. The syndrome as measured by a modified version of the Moos Menstrual Distress Questionnaire, was correlated significantly with trait anxiety, as measured by Spielberger's State-Trait Anxiety Inventory. Trait anxiety was correlated with all three subtypes from the questionnaire: behavioral, psychological, and physical, most strongly for the psychological subtype. These findings suggest a role for stress-management training in the treatment of the premenstrual syndrome.     

Clinical manifestations and stages of Rett syndrome

The presentation and clinical diagnosis of Rett syndrome at various ages and stages are reviewed. In addition to the classical form, variability in phenotype between different atypical Rett forms is given. Obligatory, supportive, and differential diagnostic criteria are summarized. Long-term follow-up findings in ageing Rett women are addressed.