“…However, of the 17 families reported (Kuo and Bassett, 1962;van Buchem et al, 1966;Critchley et al, 1968;Mars et al, 1969;Richet et al, 1969;Mawatari et al, 1972;Aggerbeck et al, 1974;Cottrill et al, 1974;Biemer and McCammon, 1975;Glueck et al, 1976;Sigurdsson et al, 1977), five have one or two members with neurological disease and three of these families have a member with additional features of the Bassen-Kornzweig syndrome: one had ataxia, acanthocytosis, and fat-loading of jejunal enterocytes (Mars et al, 1969); one had neuropathy, acanthocytosis, steatorrhoea, and retinitis (Kuo and Bassett, 1962); and one had ataxia and acanthocytosis (Critchley et al, 1968). Since both hypo-and a-betalipoproteinaemia have been described within three families (Salt et al, 1960;Cottrill, et al, 1974;Biemer and McCammon, 1975), hypobetalipoproteinaemia may 'Present address: Lincoln County Hospital, Lincoln LN2 SQY.…”