Case report Stiff-person like syndrome in a patient with multiple pituitary hormone deficiencies Chroni E, Papadimitriou A, Avramidis T, Terentiou AE, Tzioras C, Divari R. Stiff-person like syndrome in a patient with multiple pituitary hormone deficiencies. Acta Neurol Scand 2000: 102: 403–405. # Munksgaard 2000. A patient with long-standing, occult pituitary insufficiency, who developed painful muscle stiffness and superimposed spasms, closely resembling stiff-person syndrome, was described. Complete resolution of neuromuscular symptoms with hormone replacement in this case, as well as in a previously reported one, led to the suggestion that a syndrome like stiff-person could represent a rare consequence of multiple pituitary hormone deficiencies. E. Chroni 1 , A. Papadimitriou 1 , T. Avramidis 1 , A. E. Terentiou 1 , C. Tzioras 2 , R. Divari 1 From the Departments of 1 Neurology and 2 Endocrinology, Red Cross Hospital, Athens, Greece Key words: stiff-person syndrome; pituitary hormone deficiencies E. Chroni, Department of Neurology, University Hospital of Patras, P.O. Box 1045, Rion 26500 Tel./Fax: 01 03 061 993949 Accepted for publication July 5, 2000 Stiff-person syndrome (SPS) is an unusual neuro- logical disorder characterized by progressive rigidity of axial and limb muscles with super- imposed painful, often stimulus induced, spasms. Although SPS has often been associated with various, mainly autoimmune, disorders (such as diabetes mellitus type I) (1, 2), its treatment is basically unrelated to any coexisting disease and occasionally symptomatic (1, 3). There was a single report of a case of pituitary insufficiency and SPS, which was resolved with hormone replacement (4). We present a similar case. Case report A 51-year-old woman was referred for investigation of painful muscle stiffness in lower spine and both legs. Sixteen months prior to admission, the patient began to experience intermittent cramping pains of thigh muscles, which gradually became more frequent and finally permanent, involving also abdominal and lower back muscles. Her past history was unremarkable, until the age of 30, when the normal delivery of her third child was followed by severe postpartum haemorrhage. From then menstruation, which was normal from the age of 12, became very infrequent and ceased at 48 years of age. The patient reported that within the next 2 years following the obstetric incident, she presented intolerance to cold, eyebrow hair loss and photosensitive, pruritic face rash. Physical examination showed a well developed female of normal intellect with dry, cold skin, characteristic ‘‘waxy’’ or alabaster pale complexion of the face and generalized eyebrow, axillary and pubic hair loss. Her temperature was 36.5 uC, pulse 90 and blood pressure 110/65 mmHg. The thyroid gland was not palpable. On motor examination, there was normal muscle bulk and strength. Muscle tone was markedly increased in the proximal parts of the lower limbs (especially adductors and flexors), moderately increased in the abdominal and lower paraspinal area and normal elsewhere. Passive movements of hip or knee joints showed marked, stable resistance throughout the entire range of motion and triggered painful flexor spasms. Walking presented great difficulty, since in the upright position her knees were permanently flexed to 90–100 degrees and hip up to 40–60 degrees. In the supine position, the examiner’s arm could freely pass under the tunnel formed between the patient’s waist and bed mattress, due to fixed lumbar hyperlordosis. Acta Neurol Scand 2000: 102: 403–405 Printed in UK. All rights reserved Copyright # Munksgaard 2000 ACTA NEUROLOGICA SCANDINAVICA ISSN 0001-6314 403