[Neurological manifestations of Paget's disease: presentation of one case and review of the literature]. / Manifestaciones neurológicas en la enfermedad de Paget: presentación de un caso y revisión de la literatura.

INTRODUCTION: Paget's disease is a frequent metabolic disease. It is usually diagnosed as a accidental finding. Initial symptoms usually consist of generalized pain in bones. When it affects the Central Nervous System, symptoms usually appear in advanced cases and are due mainly to compression of the spinal cord. Neurological problems as the first manifestation of the disease are exceptionally found. CLINICAL CASE: We report the case of a woman affected by Paget's disease who presented as a triventricular obstructive hydrocephalus. The patient was 72 years old and consulted first to the emergency ward due to headache, gait disorders and cognitive abnormalities. COMMENTS: The most remarkable data about this patient are the indolent course of the disease which caused its debut as hydrocephalus associated to basilar impression, its favourable evolution after ventricular drainage and the almost absolute absence of previous similar cases reported in literature all over the last years.

[Stiff-man syndrome. Presentation of a new case]. / Síndrome de la persona rígida. Presentación de un nuevo caso.

INTRODUCTION: The stiff-man syndrome (SPR) is a rare neurological condition characterized by the presence of marked, involuntary rigidity of the axial muscles and limbs, together with intense painful muscle spasms which characteristically occur following external stimuli such as sudden noises, brusque movements or emotional stimuli. Symptoms are markedly improved by sleep and by diazepam. The aetiology is unknown, although it is associated with certain auto-immune disorders, particularly diabetes mellitus (DM) and others such as thyroid disease, vitiligo, pernicious anaemia, adrenal insufficiency, etc. The presence of high titres of glutamate-decarboxylase (GAD) antibodies seems to indicate an auto-immune aetiology. CLINICAL CASE: We describe a 59 year old woman who complained of an illness starting seven years previously with progressive pain and contractions of the dorso-lumbar muscles, which later spread to the cervical and abdominal muscles and to the upper limbs. From the time of onset, there were exacerbations of the condition following external stimuli. Also there was generalized vitiligo and whilst this progressed, DM developed (which finally required insulin to control it). On EMG there was continuous muscular activity and absence of muscle relaxation. The presence of high titres of anti-nuclear antibodies, gastric parietal cell antibodies and anti-GAD was very marked. There was a spectacular response to treatment with diazepam, with progressive improvement although ever increasing dosage was required. CONCLUSIONS: A new case of the 'stiff-man' syndrome is presented and aspects of aetiopathology and treatment are reviewed.