Introduction
Advances in non-invasive neurological diagnostic techniques have enabled the authors to study 3 comparatively young patients in whom a particularly severe cutaneous vascular disturbance was associated with neurological abnormalities.
Case 1
A 29-year-old right-handed road patrol man developed extensive livedo reticularis which was complicated by a chronic painful ulcer on the plantar aspect of the right great toe 6 years later. He was constitutionally obese and mildly hypertensive. At the age of 35 years he suddenly developed expressive dysphasia and a right facial weakness which recovered completely within 2 weeks. One year later he experienced a left hemiparesis and hemianaesthesia which partly resolved leaving residual difficulty with intricate finger movements. He had noticed memory impairment and problems of sight in his right visual field.
Case 1
Following a minor road traffic accident without head injury (age 37 years) he was referred to hospital where neurological examination revealed a right homonymous hemianopia and left-sided pyramidal signs including mild weakness (MRC Grade 4) hyperreflexia and an extensor plantar response. Florid livedo reticularis covered his trunk and limbs.
Case 1
Detailed haematological, biochemical and serological investigations were normal. Computerized axial tomography (CAT) showed low density areas in both parieto-occipital areas which did not enhance following contrast administration consistent with multiple small cerebral infarctions (Fig. 1) .
Case 1
Over the next 6 months he experienced several sudden dysphasic episodes, lasting up to 10 min during which time he reported an improvement in the hemianopia. He complained of progressive difficulty in dressing and often lost his way both outside and within the confines of his own home. At the age of 38 years he experienced a deep nagging ache in both thighs with wasting of both quadriceps and Grade 4 weakness of left hip flexion (MRC, 1976) . Knee (Feldaker, Hines, and Kierland, 1956; Kliiken and Gerl, 1972) . After careful exclusion of the secondary causes described by Champion (1965) they can all be considered to have idiopathic livedo reticularis (Copeman, 1975) .
Case 1
The neurological features were characterized by bilateral cortical abnormalities. In case 1 they were shown to be caused by multifocal cerebral infarction involving the watershed areas supplied by both anterior and posterior cerebral arteries. The inverse relationship between several of the transient dysphasic and hemianopic attacks may indicate intracerebral vascular 'stealing'. The dressing apraxia and loss of directional sense, both point to visuospatial problems which were confirmed on detailed neuro-psychological testing and are consistent with non-dominant hemisphere dysfunction.
Case 1
In case 2, the 'step-wise' decline suggests multiple infarctions which initially affected the parietal regions of both hemispheres. The features of Gerstmann's syndrome indicate a lesion in the dominant parietal lobe (H6caen and Albert, 1978), whereas major visuo-spatial problems, including dressing apraxia, point to non-dominant hemisphere dysfunction as do his left-sided symptoms and signs. The expressive dysphasia suggests that the dominant hemisphere lesion had extended more anteriorly to include the region supplied by the middle cerebral artery.
Case 1
In case 3 the history of linguistic difficulty and visuospatial abnormalities on psychological testing provided evidence of bilateral hemisphere disease involving particularly the parieto-occipital junctional areas similar to case 1. The perceptual abnormality of visuo-motor ataxia is characterized by misjudging the position of the objects in his right field. This phenomenon was described by Rondot, De Recondo and Ribadeau Dumas (1977) and is associated with bilateral parieto-occipital disease.
Case 1
Case 1 was further complicated by a clinical picture suggesting a femoral nerve infarction (Asbury, 1977) and a cardiac abnormality consistent with cardiomyopathy due to a generalized small blood vessel disorder (Oakley, 1972) .
Case 1
A detailed search of the world literature reveals sporadic reports of very similar cases which previously have never been collected together (Barker, Hines and Craig, 1941; Champion and Rook, 1960; Church, 1962; Sneddon, 1965; Strzalko, Rymczonek and Zajgner, 1966; Trzebicki and Buksowicz, 1969; Buksowicz, Niedzielska and Trzebicki, 1970; Gralewski, 1972; Piniol Aguade et al., 1975; Pellat et al., 1976; Rumpl and Rumpl, 1979 The limited amount of pathological data available (Pellat et al., 1976) casts little light on the pathophysiology of this clinical association and detailed post-mortem studies are required. However, these will not be forthcoming unless the occurrence of this condition is more widely acknowledged. Speculations on the aetiology have included cerebral thromboangiitis obliterans (Miller-Fisher, 1957) and a 'vasculopathy' of obscure aetiology (Sneddon, 1965) . Arteritis has usually been fully excluded (Fauci, Haynes and Katz, 1978) .
Case 1
If a young to middle-aged person presenting with transient ischaemic attacks is found to have widespread livedo reticularis, then evidence of unsuspected diffuse abnormalities should be sought by detailed neuropsychological testing (McFie, 1979 

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