Emmanuel Ellie

Thymoma-Associated Neuromyotonia with Antibodies against Voltage-Gated Potassium Channels Presenting as Chronic Intestinal Pseudo-Obstruction

Chronic intestinal pseudo-obstruction can occur as a paraneoplastic disorder, and several cases have been reported in association with thymoma or small-cell lung cancer. Autoantibodies against voltage-gated potassium channels (VGKCs) are found in acquiredneuromyotonia (Isaac’s syndrome), and have been reported in one case of slow transit constipation without apparent neurological disease. We describe a patient with VGKC antibodies, acquired neuromyotonia and thymoma, who first presented with a severe slow-transit constipation and in whom the gastrointestinal symptoms responded well to plasmapheresis. We suggest that VGKC antibodies might be helpful in patients with possible paraneoplastic chronic intestinal pseudo-obstruction, and a positive result should stimulate the search for a thymoma or other tumour and raise the possibility of immunotherapy.

Extensive brain calcifications in systemic sclerosis: two cases

Systemic sclerosis (scleroderma) is a multisystem connective tissue disease of unknown aetiology, characterised by progressive fibrosis of the skin and internal organs including the lungs and gastrointestinal tract.1 Pathological calcification of soft tissues (known as calcinosis) is a common feature in the CREST syndrome of scleroderma (calcinosis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia). By contrast, internal organ calcification is rare, and isolated cases of spinal calcinosis and calcific constrictive pericarditis have been reported.2 3 We report here the cases of two patients with systemic sclerosis whose CT examination disclosed extensive brain calcifications. Case 1, a 48 year old man was referred to hospital because of polyarthralgia involving the wrists and ankles, Raynaud’s phenomenon, and tightness of hand skin. The patient’s wife had noticed that during the previous months, he had a slowed mentation and a depressive mood. Physical examination disclosed a sclerodactyly but no telangiectasias. Routine haematological tests were normal. Antinuclear antibodies were positive at a 1/2000 dilution with nucleolar fluorescence. Rheumatoid factors, antidouble stranded DNA and antiphospholipid antibodies were negative. There was no cryoglobulinaemia. Complement was normal. Lung function tests showed a restrictive syndrome (forced vital capacity=75% predicted). Chest radiography was normal, as were oesophageal manometry and cardiac ultrasonographic examination. A diagnosis of systemic sclerosis …

Dermatopolymyosite révélatrice d'un myélome à IgM

We report the association of polymyositis with multiple myeloma secreting IgM lambda monoclonal paraprotein. Few observations describe coexisting polymyositis and benign monoclonal gammopathy but so far a similar association has never been mentioned.