Biomedical subjects
Publications and source records attributed to W B Matthews.
Three cases of probable iatrogenic transmission of Creutzfeldt-Jakob disease by neurosurgery are detailed together with a cluster of three cases in Eastern England possibly connected by dental procedures, and the development of Creutzfeldt-Jakob disease in a patient who had been in social contact with a familial case.
Visual, somatosensory and brain stem auditory evoked potentials were recorded in 84 patients in whom the diagnosis of multiple sclerosis was under consideration. The patients were followed up for a maximum of 38 months or until a definite diagnosis of multiple sclerosis or of some other condition was established. Seven patients were found not to have multiple sclerosis. Abnormal evoked potentials indicating clinically silent lesions were found at the initial investigation in fourteen of the twenty-eight patients in whom a diagnosis of clinically definite multiple sclerosis was accepted during the period of follow up. In the thirty-seven patients in whom no change in clinical category occurred during the period such abnormalities were found in five. The visual evoked potential was found to be of greatest predictive value.
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Eleven patients with static or slowly progressive multiple sclerosis were treated with continuous spinal cord stimulation via epidural electrodes for a minimum of two weeks. An extensive battery of objective tests was used to assess results. Significant improvement in urinary bladder function was seen in 2 of the patients with a lesser degree of improvement in 4 others and reduction of lower limb spasticity in 6 patients. Five additional patients selected for these disabilities were then treated with further evidence of improvement in bladder function in 4 and in lower limb spasticity in 2. It is suggested that benefit results from inhibition of excessive spinal reflex activity.
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Pattern reversal visual evoked potentials (VEP) and short-latency somatosensory evoked potentials (SEP) were recorded at frequent intevals in 7 patients with multiple sclerosis (MS). A persistent increase in latency of VEP was found to accompany evanescent visual symptoms. SEP, whether normal or abnormal, remained stable in patients without relapse. In 3 patients with active disease the cervical SEP was labile but this could not be related to specific clinical changes. In 51 patients VEP were recorded on two or more occasions at varying intervals. There was a reasonable correlation between alteration in visual acuity and in VEP. The return of abnormal latency to normal values was seen on 9 occasions. In 27 patients in whom SEP were similarly recorded the potentials became more abnormal in the majority of those with relapse involving the spinal cord but SEP did not return to normal with remission. It was concluded that the use of EP for diagnostic purposes in MS depended on persistence of abnormalities during quiescent phases of the disease and that this property was incompatible with the use of these techniques as an effective means of monitoring the course of the disease.
A patient with mild multiple sclerosis died from a ruptured intracranial aneurysm. It was possible to relate abnormalities of somatosensory evoked potentials recorded some months earlier to a plaque involving the root entry zone in the cervical spinal cord which had not resulted in clinical sensory abnormalities.
The effects of raising body temperature on the visual (VEP) and somatosensory (SEP) evoked potentials were observed in normal subjects and in patients with multiple sclerosis. The amplitude of the VEP was significantly reduced to the same degree after heating in normal subjects and in patients with multiple sclerosis but there was no effect on the latency of the potential. Changes in amplitude could not be related to reduction in acuity. In contrast, the cervical SEP was greatly disorganised after heating in many patients with multiple sclerosis while the only effect in normal subjects was to reduce the latency by increasing peripheral conduction velocity. These results suggest that heat caused conduction block in demyelinated axons in the sensory pathways of the cervical spinal cord.
Two patients are described in whom acute symptoms of apparently primary psychiatric disease could be diagnosed in retrospect as due to multiple sclerosis. In both patients the initial symptoms recovered completely. In a third patient, also presenting with mental symptoms, this diagnosis would not have been suspected on clinical grounds but is suggested by the results of modern diagnostic techniques.
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Abnormalities of the potential evoked by stimulation of the median nerve and recorded over the cervical spine were found in 59% of patients with multiple sclerosis (MS) this proportion increasing to 69% of those in the definite diagnosis category and to 100% in the severely disabled. Abnormalities were often found in the absence of relevant clinical signs and the method appears to be capable of revealing clinically silent plaques. In patients with a single episode of neurological disease, including retrobulbar neuritis, and at least compatible with the onset of MS, the proportion of abnormalities did not rise above 18%. Only prolonged follow-up will permit assessment of the value of this and other evoked potential techniques in the detection of the early case of the disease.
A family is described in which a mother and three of her five children showed myoclonic epilepsy. The mother and one son were also ataxic; one other son had additional features of Friedreich's ataxia, and a daughter had peroneal muscular atrophy as well as myoclonic epilepsy and ataxia. Although some of these disorders have been associated in previously reported families, the occurrence of all three disorders in members of one family seems to be unique. It is concluded that this family shows the manifestations of one, probably dominant, gene. The differences in age of onset and manifestations may be explained by the action of one or more subsidiary genes.
Three patients with peripheral neuropathy and an associated benign IgG paraproteinaemia are described. No direct immunological evidence for an aetiological role of the paraprotein was found, and the implications of this are discussed.
The prognosis in 30 patients with spasmodic torticollis proved to be unexpectedly bad, only one patient making a full and sustained recovery. Investigation did not confirm previously reported reflex influences on the degree of spasm. Detailed otological investigation did not suggest that spasmodic torticollis originates in vestibular dysfunction. Psychological assessment of the patients showed no deviation from normal in premorbid personality but confirmed the severe adverse effects of the condition.