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A 17 year old girl died after an illness characterized by progressive mental deterioration and severe myoclonic jerks. Extensive pathological, virological, and immunological studies failed to support the diagnosis of subacute sclerosing panencephalitis but were compatible with disseminated demyelinating disease. The spinal fluid was positive for measles virus antibodies when examined by the fluorescent antibody technique, by complement fixation, and by haemagglutination inhibition tests, but the antibody titres were not high. The concentration of vaccinia antibody in the serum was consistent with that found in the general population and none was detected in the spinal fluid. Animal and tissue culture studies failed to disclose a viral agent, but pathological sections revealed perivascular cellular infiltration, demyelination, rare inclusion bodies, and multinucleated giant cells. Although these studies were not definitive, a record of procedures used and results obtained, both positive and negative, may be helpful to other investigators interested in defining more accurately the clinical features and the pathogenesis of these diseases. [ABSTRACT FROM AUTHOR]

Neurological findings are described in 200 consecutive cases of suicidal ingestion of organophosphorous insecticides. Miosis is almost universal. We found impairment of consciousness in 10%, fasciculations in 27%, convulsions in 1%, toxic delirium in 50%, and paralysis in 26%. Toxic delirium was attributed to treatment with atropine. Paralytic signs were divided into type 1 signs (present on admission) and type 2 signs (appearing later while on atropine treatment). Type 1 signs, chiefly impaired consciousness and bilateral pyramidal tract signs, respond to atropine. The most common type 2 signs are proximal limb weakness, areflexia, and cranial nerve palsies. EMG studies during type 2 paralysis show a myasthenic response in some cases. Of 36 cases with type 2 signs 15 died from respiratory paralysis after a variable period of artificial respiration. Twenty-one recovered and no residual neurological deficit has been noted. Atropine did not influence type 2 paralysis. It is claimed that type 2 signs differ significantly from those described before as `delayed neurotoxicity' and may represent an alternative mode of human toxicity with organophosphorous compounds. [ABSTRACT FROM AUTHOR]

In the past, the classification of progressive neurological diseases in childhood has been based on either clinical or histological criteria. More recently biochemical aspects have been included and the resulting terminology has become very complex. As the electrical activity of the brain is a sensitive indicator of altered cerebral metabolism, the EEG has added further parameters in the study of neurometabolic disorders. In the present study 30 children were selected on the basis of an unusual combination of EEG features: excess of irregular slow activity, spikes, often polyphasic, and very large amplitude discharges in response to low rates of photic stimulation. All these children suffered from a disease which after an insidious onset progressed with a fairly uniform clinical course leading to death in a few years. The histological diagnosis was that of `Batten's disease', but other patients with the same histological diagnosis did not show either the same EEG features or a similar clinical picture. It is suggested that the EEG features make it possible to identify at an early stage, a disease entity within the wider range of conditions sometimes called `Batten's disease'. [ABSTRACT FROM AUTHOR]

The clinical features of idiopathic polyneuritis in 22 patients admitted to the University of Kentucky Medical Center between July 1965 and June 1970 are described. Serial studies of peripheral nerve function in 12 patients followed to clinical recovery showed changes in nerve conduction velocity, distal motor latency, and/or muscle action potential amplitude. Six children and one adult, showing a change in all three parameters, exhibited a strong correlation between the degree of slowing and the shortness of their illness. Assessment of immune status, by quantitative measurement of cerebrospinal fluid immunoglobulin G(11 patients) and by the degree of lymphocyte transformation on exposure to specific brain protein and/or a homogenate of sciatic nerve (10 patients), bore no consistent relation to the severity or course of the illness. A double-blind trial of short-term, high dose adrenocorticotrophic hormone has yielded no valid evidence to date for or against the effectiveness of such therapy in this illness. [ABSTRACT FROM AUTHOR]

Dementia paralytica may present diagnostic difficulties when routine serological test using a non-treponemal antigen is non-reactive. We present an illustrative demented patient who initially had negative VDRL test both in his serum and cerebrospinal fluid. However, the brain biopsy specimen showed active meningoencephalitis. By special staining technique, a spiral organism was found in the brain exhibiting morphology perfectly compatible with treponema pallidum. Later in the course, the VDRL became reactive in the blood but remained non-reactive in the cerebrospinal fluid. On the basis of the experience of other workers in the field and ours with this patient, we advise the use of FTA-ABS test as a screening procedure in patients with neurological problems of possible syphilitic origin. We urge further research in this field. [ABSTRACT FROM AUTHOR]

Intercostal nerve conduction study has proved to be an accurate technique in diagnosis of thoracic radiculopathy in 161 patients, 80 of whom had subsequent posterior rhizotomy with relief of pain in 81% of those undergoing surgery. The only significant complication of intercostal nerve conduction study is an 8·8% incidence of pneumothorax. [ABSTRACT FROM AUTHOR]