Some cases of raised intracranial pressure, previously diagnosed as idiopathic intracranial hypertension (IIH), are now found to have intracranial venous sinus thrombosis which is linked to a prothrombotic state. This study looked at the prevalence of familial thrombophilia in cases of IIH with normal cerebral sinus imaging. Thirty patients with IIH, fulfilling the modified Dandy criteria, were prospectively enrolled to form the study group. All but two patients were female. All had a full risk-factor history taken and underwent investigation of rheological factors and thrombophilia, including tests for the factor V Leiden mutation, prothrombin G20210A allele, and hyperhomocysteinaemia. Ninety people made up the control group. Twenty-five patients had a normal thrombophilia screen. Two patients were heterozygous for the factor V mutation, two had mild raised levels of antiphospholipid antibodies, one of whom was transient, and one had reduced levels of free protein S. Both males and 28 females (90%) were obese (body mass index >25). Other lifestyle risk factors for thrombosis, such as smoking and the oral contraceptive pill, were not uncommon, and a positive or 1(st)-degree relative family history of deep venous thrombosis was found in three patients. No patient had the prothrombin G20210A variant or raised levels of homocysteine. Despite previous reports of a possible aetiological link between thrombophilia and IIH, the present study does not support this hypothesis.

Many patients attending an Accident and Emergency (A&E) department with seizures never come into contact with a neurological service. This survey was designed to find out how many patients with epileptological emergencies come to A&E and how they are managed. Cases were identified using the computerized A&E database. The A&E records of all adult patients attending the casualty department at St James's University Hospital with emergencies related to epilepsy between 1 April and 30 September 1998 were reviewed retrospectively. Out of a total of 36 024 adults attending A&E, 190 were related to epileptological emergencies. A problem relating to a previously recognized seizure disorder was the commonest reason for attendance. Patient management was highly variable and often suboptimal. Descriptions of seizure semiology and examination findings were frequently deficient. Up to 37.5 mg of diazepam, in up to five boluses, was given. Twenty per cent of patients with a diagnosis of status epilepticus were discharged home after diazepam treatment. Neurologists only became involved in 24.2% of cases. Epileptological emergencies only make up a small proportion of cases seen in adult A&E departments. Treatment and referral guidelines should be agreed between A&E staff and neurologists. The communication between general, specialist and acute services needs to be improved.

Postoperative epileptic seizures are recognised but rare. Psychogenic seizures and pseudostatus epilepticus are relatively common, particularly in the peri‐operative period. Our series of five cases of postoperative pseudostatus epilepticus demonstrates that the failure to recognise the psychogenic nature of this condition may cause anaesthetists to give inappropriate and potentially harmful treatment. Psychogenic ‘status’ is easy to diagnose once it has been considered. Convulsive episodes lasting longer than 90 s, closed eyes during a ‘tonic–clonic’ attack, retained pupillary response and resistance to eye opening are useful signs. Often there is a history of multiple admissions with ‘status epilepticus’ and of previous postoperative ‘status’.

The purpose of this study was to examine the possible association between "classic" motor neuron disease (cMND) and frontotemporal dementia (FTD), using neuropsychological evaluation and single photon emission computed tomography (SPECT). Psychological tests assessing language, perceptuospatial, memory, and "frontal lobe" functions were given to patients with cMND and test scores were compared with those of normal control subjects. 99mTc-HMPAO SPECT was performed on patients with cMND, FTD and motor neuron disease (FTD/MND), FTD alone, and normal control subjects. Regional cerebral blood flow indices (rCBFi) were determined in 36 cortical regions, and differences between grouped rCBFi data were investigated by canonical discriminant analysis. There were significant group differences in the scores of picture sequencing and token tests in patients with cMND compared with normal controls. Regional CBFi data showed frontal and anterior temporal reductions in patients with cMND compared with normal controls. A similar pattern of SPECT abnormality was seen in patients with FTD/MND and FTD alone, but to a more pronounced degree than in patients with cMND. Neuropsychological and SPECT findings in cMND, FTD/MND, and FTD showed a common pattern of cerebral involvement, most pronounced in the second two conditions. It is suggested that cMND, FTD/MND, and FTD represent a clinical range of a pathological continuum.

Talbot, P. R.; Goulding, P. J.; Lloyd, J. J.; Neary, D.; Testa, H. J. Author Information

Sixteen patients with progressive language disorder have been studied longitudinally. Anomia was a prominent presenting characteristic and mutism ultimately occurred. Patients, however, were clinically heterogeneous. Some exhibited nonfluent, agrammatic features, whereas others demonstrated a fluent aphasia, with profound loss of word meaning. Although language disorder remained the sole symptom in a minority of patients, in others an associative agnosia or personality and behavioral changes, or both, emerged. Findings on computed tomography and single photon emission tomography mirrored the areas of dysfunction suggested by the neuropsychological profiles and demonstrated abnormalities restricted to the left hemisphere or involving bilateral frontotemporal cortices. Brains of 3 patients, with distinctive clinical pictures, have been examined at autopsy. Each revealed a focal distribution of atrophy, gliosis and spongiform change, and an absence of senile plaques and neurofibrillary tangles. There was clinical and pathological overlap with frontal lobe dementia. We argue that progressive language disorder is clinically heterogeneous and forms part of a spectrum of clinical presentations of non-Alzheimer lobar atrophy.

Four patients are described, in whom a profound and rapidly progressive dementia occurred in association with clinical features of motor neuron disease. The pattern of dementia indicated impaired frontal lobe function, confirmed by reduced tracer uptake in the frontal lobes on single photon emission computed tomography (SPECT). Pathological examination of the brains of two patients revealed frontal-lobe atrophy, with mild gliosis and spongiform change. The spinal cord changes were consistent with motor neuron disease. The clinical picture and pathological findings resembled those of dementia of frontal-lobe type and were distinct from those of Alzheimer's disease. The findings have implications for the understanding of the spectrum of non-Alzheimer forms of primary degenerative dementia.

Presents case reports of a 67‐yr‐old man and 2 women (aged 60 and 66 yrs) with primary cerebral atrophy in whom progressive breakdown in language and visual perception are attributed to loss of semantic information. This form of dementia is distinct from that of Alzheimer′s disease and is assumed to represent a form of circumscribed cerebral atrophy with emphasis of pathology in temporal rather than frontal regions of the brain.

128 nor structural cause was found.The presence of biological markers of lead intoxication and the favourable response to chelation therapy allowed the diagnosis of acute lead encephalopathy.2The search for recent occupational and environmental exposure was negative.The normal blood lead in the patient's wife rules out some other environ- mental source which might be unnoticed.Therefore, the only apparent lead exposure was his past work.The previously reported cases of lead encephalopathy occurred shor- tly after recent lead exposure2 except for patients with retained bullets."On the other hand, it is known that lead can be stored in bone for decades6 and mobilised by an intercurrent stress with increased bone turn- over."5The gastroenteritic process and bed rest in this patient could have been the conditions which caused lead mobilisation.To our knowledge, this is the first case reported of acute encephalopathy as a likely delayed presentation of occupational lead exposure.This observation emphasises the possibility of severe late toxicity as the first manifestation of a remote lead exposure.