We investigated the prevalence of mental depression (MD) in 34 patients with Parkinson's disease (PD) of recent onset, not receiving dopaminergic drugs, and in 23 healthy individuals of comparable age and sex. In the patients, dysthymic disorder and major depression were more common than in controls (p = 0.017). The severity of MD and PD were unrelated. In 15 patients, MD began before the symptoms of PD (mean, 5 years). These patients were younger, less impaired, and with a higher positive family history of PD. MD-associated PD may be a specific subgroup of PD.

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A 39-year-old man was referred to our centre with hepatosplenomegaly that had been present since childhood. He was asymptomatic and his full blood count showed only mild thrombocytopenia (platelet count 123 9 10 9 /l). A blood film was normal.A bone marrow aspirate revealed hyperplasia of foamy and, especially, sea-blue histiocytes (images), with haemophagocytosis. The activity of acid sphingomyelinase from dried blood was below the normal range (54 pmol/ spot*20 h, normal range 200-3500) in leucocytes, leading to the diagnosis of type B Niemann-Pick disease (NPD). Acid lipase activity was within the normal range (0Á56 nmol/ spot*3 h, normal range 0Á10-2). Molecular analysis showed compound heterozygosity for two mutations in SMPD1 (p.Tyr469Ser/p.Arg610del). No mutation was detected in NPC1 or NPC2.NPD is a sphingolipidosis that includes at least three different disease types (A, B, C), all inherited by autosomal recessive transmission. Complete and partial deficiency of the lysosomal enzyme acid sphingomyelinase (ASM) is responsible for NPD types A and B, respectively. Type A is the most common form in which symptoms begin shortly after birth,

An 80-year-old woman presented with asthenia of several weeks duration. She had been diagnosed with Gaucher disease 34 years earlier (N370S/N370S genotype, acid beta-glucosidase activity 18%, serum chitotriosidase levels: 1557 nmol/ml/h). She had been asymptomatic since then, with only mild splenomegaly, and no treatment had been required. A full blood count showed macrocytic anaemia (haemoglobin concentration (Hb) 109 g/l, MCV 122 fl) and a platelet count of 122 9 10 9 /l. Serum vitamin B 12 was reduced (112 pg/ml) and vitamin B 12 therapy was therefore initiated. In spite of the normalization of platelet count and vitamin B 12 levels, her Hb decreased and several red blood cell transfusions were needed.A bone marrow aspiration and trephine biopsy were therefore performed. A May-Gr€ unwald-Giemsa stain revealed numerous Gaucher cells and megakaryocytes with hypolobated nuclei (images). No other dysplastic features were found. Karyotype showed deletion of 5q in one out of 21 metaphases, and fluorescent in situ hybridization (FISH) for 5q

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