SUMMARY A clinical study of 42 patients with essential tremor is presented. In the case of 12 patients the family history strongly suggested an autosomal dominant mode of transmission, in four the mode of inheritance was indeterminate, and the remaining 26 patients were sporadic cases without an established genetic basis. The tremor involved the upper extremities in 41 patients, the head in 25, lower limbs in 15, and trunk in two. Seven patients showed involvement of speech. Variations were found in the speed and regularity of the tremor. Leg involvement took a variety of forms: (1) direct involvement by tremor; (2) a painful limp associated with forearm tremor; (3) associated dyskinetic movements; (4) ataxia; (5) foot clubbing; and (6) evidence of peroneal muscular atrophy. Several minor symptoms hyperhidrosis, cramps, dyskinetic movements, and ataxia-were associated with essential tremor. Other features were linked phenotypically to the ataxias and system degenerations. Apart from minor alterations in tone, expression, and arm swing, features of Parkinsonism were notably absent.

SUMMARY Study of the speech disorders of Parkinsonism provides a paradigm of the integration of phonation, articulation and language in the production of speech. The initial defect in the untreated patient is a failure to control respiration for the purpose of speech and there follows a forward progression of articulatory symptoms involving larynx, pharynx, tongue and finally lips. There is evidence that the integration of speech production is organised asymmetrically at thalamic level. Experimental or therapeutic lesions in the region of the inferior medial portion of ventro-lateral thalamus may influence the initiation, respiratory control, rate and prosody of speech. Higher language functions may also be involved in thalamic integration: different forms of anomia are reported with pulvinar and ventrolateral thalamic lesions and transient aphasia may follow stereotaxis. The results of treatment with levodopa indicate that neurotransmitter substances enhance the clarity, volume and persistence of phonation and the latency and smoothness of articulation. The improvement of speech performance is not necessarily in phase with locomotor changes. The doserelated dyskinetic effects of levodopa, which appear to have a physiological basis in observations previously made in post-encephalitic Parkinsonism, not only influence the prosody of speech with near-mutism, hesitancy and dysfluency but may affect word-finding ability and in instances of excitement (erethism) even involve the association of long-term memory with speech. In future, neurologists will need to examine more closely the role of neurotransmitters in speech production and formulation.

A 40-yr-old woman, K.S., is reported, who shows a severe loss of memory for people following a history of epilepsy and right anterior temporal lobectomy. Despite this memory problem, K.S. is not clinically amnesic, has a Memory Quotient of 122 on the Wechsler Memory Scale in line with her IQ of 119, and performs well on conventional tests of recognition and recall. She does not have a generalized semantic memory deficit for living things, but her deficit extends beyond people to include famous animals, buildings and product names. Autobiographical memory is good, except where memory for people is concerned. The nature of the memory store that is impaired in K.S. is discussed, as are the implications of her case for theories of the organization of long-term memory.

No abstract

The presence of positive antibody titres to Toxoplasma and Toxocara in an adult epileptic population has been examined in relation to other observations of aetiological importance. With Toxoplasma, and more particularly with Toxocara, a higher incidence of positive antibody titres was recorded than in nonepileptic populations. Comparison with previous studies in childhood epilepsy indicate that the incidence of positive titres increased with age throughout adult life. Despite attention to the age of onset of epilepsy, presumed aetiological factors, and electroencephalographic and clinical observations, no causal relationship between parasitic infection and the aetiology of epilepsy was established.

Botulism can arise from preformed toxin, wound infection or intestinal toxico-infection. All three forms can occur in humans as well as in animals. The examination of botulism in veterinary practice can alert the medical profession to the hazards which can occur with the introduction of dietary alterations and hermetic sealing of foodstuffs. There is also the possibility that the development of pica through lack of essential nutrients could lead to the ingestion of contaminated substances rendering the child (or even adult) susceptible to botulinum intoxication. A positive gain has been that research leading to the elimination of shaker foal disease has provided a comprehensive analysis of factors which may underline the risk of toxico-infection in infants.

In 1932 Monroe wrote that “inability to read may be one of the prolonged school problems to which parents and agencies become adapted until the child drifts into truancy and incorrigibility”. This observation, if true, is highly pertinent to the problems of present day society, and, for this reason, a personal study was made of delinquent boys between the ages of 12 and 17 years referred by the courts for assessment at a London Remand Home and Classifying Centre.

Summary A chronic degenerative neurological disorder is described which is characterized by tics, grimacing, involuntary movements, a severe disturbance of swallowing and a concurrent malformation of the erythrocytes. This condition is clearly different from familial hypo-betalipoproteinaemia and abetalipoproteinaemia, the two previously described neurological disorders associated with abnormal red cells.