Congenital neuroblastoma with liver metastasis presenting with Hashimoto Pritzker disease.

A two-month-old female child presented with discrete skin-colored papules on the forehead and scalp. She was earlier diagnosed to have neuroblastoma of the adrenal gland which had metastasized to the liver. Histological sections of the skin lesion showed a nodule composed of sheets of cells with abundant eosinophilic cytoplasm and an occasional grooving of the nucleus. These cells were CD 68 positive. Eosinophilic abscesses were seen in between the neoplastic cells.

Induction of resistance in host against the infection of leaf blight pathogen (Alternaria palandui) in onion (Allium cepa var aggregatum).

The Pseudomonas fluorescens isolate Pfl was found to inhibit the growth of pathogen Alternaria palandui, in vitro. In the present study, foliar application of a talc-based formulation of Pfl significantly reduced the incidence of leaf blight of onion, caused by A. palandui. Induction of defense-related proteins viz., chitinase, beta-1,3 glucanase, peroxidase (PO) and polyphenol oxidase (PPO) by application of Pfl, was studied against A. palandui infection in resistant (IHR 56) and susceptible (MDUI) onion cultivars. Chitinase in both cultivars, with or without challenge-inoculation of A. palandui revealed changes in the isoform pattern. The Native-PAGE of PO showed induction of PO2 isoform in both the cultivars, in response to inoculation of pathogen. Isoform analysis of PPO also exhibited induction in the Pfl-treated plants challenged with pathogen. Similarly, the activity of beta-1,3-glucanase was greatly induced in Pfl-treated plants, challenged with pathogen as compared to controls. Thus, the P. fluorescens-treated plants showed significant increase in the levels of the defense enzymes, in comparison to the plants challenged with the pathogen.

Familial infantile myaesthenia.

We report a family of two brothers with familial infantile myaesthenia which is an autosomal recessive congenital myaesthenic syndrome. It is a presynaptic neuro muscular junction disorder, responsive to treatment and has got good prognosis.

Crossed aphasia leading to pure word deafness.

We report a case of crossed aphasia following a vascular insult of the right hemisphere in a middle aged dextral man with subsequent evolution to pure word deafness. Development of pure word deafness during recovery from crossed Wernickes aphasia is an extremely rare clinical entity. Probably this is the first Indian report of this entity.

Neurological manifestations of homocystinuria.

Six cases of homocystinuria with different clinical manifestations are described. Two of these patients presented with vascular problems, two had proximal muscle weakness, one each had cerebellar ataxia and psychiatric manifestations. Biochemical defects and the pathological mechanisms in different types of homocystinurias are reviewed.