Biomedical subjects
Publications and source records attributed to W B Matthews.
A patient with profound panhypogammaglobulinaemia due to common variable immunodeficiency developed two discrete intra-cranial space-occupying lesions, 10 years apart, requiring craniotomy. Histological examination revealed the intra-cranial masses to be granulomata of unknown aetiology on both occasions.
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A concurrent change in evoked potential measurements and quantitation of circulating T-suppressor (CD8) lymphocyte subpopulations might indicate increased subclinical disease activity. Eight untreated patients with clinically definite multiple sclerosis were monitored monthly for changes in the numbers of cells positive for CD8 markers, and hence in the ratio of CD4: CD8 positive cells. Such changes were found not to be associated with changes in evoked potentials or clinical status.
Two hundred and sixty seven patients with a definite or probable diagnosis of Creutzfeldt-Jakob disease (CJD) were identified in an attempt to ascertain all cases of the disease in England and Wales in the period 1970-84. No evidence of space-time clustering of dates and places of onset was found for these cases. Two age- and sex-matched controls were selected for 72 of the 122 cases diagnosed in the period 1980-84 and life histories of places of residence were obtained for these cases and the controls. No evidence was found that cases had lived closer to each other than had controls at any time in their lives, except that there was an excess of cases born in London. The study does not provide supportive evidence for case-to-case transmission of CJD being an important component in the aetiology of the disease.
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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.
The laboratory transmission to animals of an apparently degenerative disease of the nervous system, Creutzfeldt-Jakob disease (CJD), is now well established. Important questions arising from this observation are the possibility of natural transmission or infectivity and the existence of other similarly transmissible diseases. Epidemiological studies have revealed some possible clusters of CJD and also an association with previous craniotomy, but there is no definite evidence of natural infection. A few instances have been reported of experimental CJD in animals following inoculation with material from Alzheimer's disease, but apart from this there is so far no evidence of transmission of any other form of degenerative nervous disease.
The pattern reversal evoked visual potential (VEP) was recorded in 37 normal subjects and in 186 patients in whom a diagnosis of multiple sclerosis (MS) was established or suspected. Taking the upper limit of normal as the mean +2.5 SD (111 ms), prolonged latency was found in 75% of definite cases of MS, 58% of probable cases, and 38% of possible cases. A smaller number of patients without prolonged latency had abnormal asymmetry of latency or low amplitude potentials. In patients with a single acute episode of neurological disease resembling MS the incidence of abnormal VEP was very low. In patients examined within three months of an episode of retrobulbar neuritis (RBN), latency was prolonged in 81% of affected eyes, a similar proportion being found in patients with a more remote history of RBN. The importance of establishing the normal for every laboratory engaged on this investigation is emphasised. Prolonged latency of the VEP is common in established MS but has not yet been shown to be a sensitive diagnostic test of the early case.