Two patients with scleromyxedema who had progressive neurologic impairment are described. One patient died, and one required prolonged mechanical ventilation. A review of the literature has produced 24 other cases of scleromyxedema in which neurologic changes were prominent.

The association of potentially lethal neurologic syndromes with scleromyxedema (papular mucinosis)

third and nineteenth day from the beginning of the symptoms, the first one being normal and the second one showing an increase in protein (0-95 g/l) with 11 cells and negative titres to brucella, syphilis, toxoplasma, herpes simplex, varicella-zoster, and a negative anticryptococcal antigen determination. The patient's clinical pattern began to improve two weeks after admission; she was discharged a week later. Follow up a month later revealed only a generalised areflexia and a slight dysmetria on the heel-knee test. Electrophysiological studies (table) were carried out on the sixth and eighteenth day, which revealed absent sensory potentials. Motor NCS showed less abnormality than that of sensory nerves. There was delay in the distal motor latency in all the nerves tested and there was also reduction in motor amplitude in both median nerves, with a 25-3% drop in peak-to-peak amplitude between wrist and elbow, and a 39-2% drop in amplitude between wrist and axilla in the first right median nerve conduction study. Neither temporal dispersion nor motor conduction block in the remaining nerves was noted. EMG studies were normal. A third electrophysiological study performed a month after her discharge showed the recovery of the sensory nerve action potentials in the lower and upper extremities, although the amplitude and sensory conduction velocity of the nerves studied were lower than the normal values. The motor NCS, however, were similar to previous values. The EMG study of the right anterior tibialis and right first dorsal interosseous muscles showed compatible changes with minimum denervation. Until recently the Guillain-Barre syndrome was an entity whose nosological characterisation relied upon a purely descriptive base, with relatively widely accepted diagnostic criteria,' Among these, the presence of muscular weakness is the most noticeable, indicating the main affectation of the motor roots. However, a purely sensory clinical variant of this illness is also believed to be possible and the following features are necessary for it to be accepted:2 rapid onset, distribution widespread and symmetrical, complete or near recovery, high CSF protein content, with few or no cells, and an electrophysiological study compatible with a demyelinative process in the peripheral nervous system. There is still a certain controversy as to whether this clinical variant occurs. In a recent review of 42 patients with acute sensory neuropathy, the authors concluded that this condition is not part of the spectrum of inflammatory demyelinating neuropathies;' nevertheless, it is noticeable that only 2 of the 42 patients had complete remission of symptoms and that the course of their disease was very protracted-from six to nine months. A case of acute polyneuropathy has been published in which the sensory disorders were also prominent and very similar to those of our patient.4 The necropsy carried out after the patient's death from pulmonary embolism revealed inflammatory infiltration with segmental demyelination in the peripheral nerves, posterior roots and spinal ganglia, with a marked preservation of the anterior roots. These findings, except for the preservation of the motor roots, are identical to those which are described in the GuillainBarre syndrome.' Our patient had an acute sensory neuropathy after a presumed viral illness and vaccination with a monophasic course and nearly complete recovery-both clinical and electrophysiological-in a short time; CSF protein content was high with a mild pleocytosis. The evidence suggests that this was probably an acute sensory inflammatory demyelinating polyneuropathy. FRANCEISC MIRALLES JORDI MONTERO RAMON RENE JUAN A MARTINEZ MATOS Department of Neurology, Hospital Princeps d'Espanya Ciutat Sanitaria de Bellvitge, Barcelona, Spain

https://jnnp.bmj.com/content/jnnp/55/5/412.2.full.pdf

Toxic reaction following the combined administration of fluoxetine and phenytoin: two case reports.

https://jnnp.bmj.com/content/jnnp/55/5/411.2.full.pdf

Pure sensory Guillain-Barré syndrome.

https://jnnp.bmj.com/content/jnnp/55/5/412.1.full.pdf

Cerebral venous thrombosis in paroxysmal nocturnal haemoglobinuria.

Escleromixedema y síndrome dermato-neuro: buena respuesta al tratamiento con glucocorticoides e inmunoglobulinas endovenosas

[Recurrent coma, papular mucinosis and benign dysglobulinemia].

[Benign intracranial hypertension and Marchiafava-Micheli disease].

Papular mucinosis recurrent comas benign dysglobulinemia

The course of acute leukaemias, particularly of the lymphoblastic type, is very frequently complicated by the development of neuromeningeal localisations of the disease. They may, more rarely, be presenting initial lesions, their origin being confirmed clinically and/or biologically, but it is exceptional that they remain a strictly isolated expression of the affection. A case is reported of a 16-year-old adolescent with leukaemic meningitis, in whom haematological tests (blood counts, bone marrow smear, iliac medullary biopsy, lymph node biopsy) gave negative results over a period of at least two months. Based on the findings in this case, the significance of early neuromeningeal localisations of acute leukaemias is discussed.

[Primary neuromeningeal manifestation, isolated for a long period, of acute lymphoblastic leukemia].

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[Recurrent coma, papular mucinosis and benign dysglobulinemia].

[Benign intracranial hypertension and Marchiafava-Micheli disease].

Papular mucinosis recurrent comas benign dysglobulinemia

[Primary neuromeningeal manifestation, isolated for a long period, of acute lymphoblastic leukemia].