SYNOPSISThe clinical, pathological, and chemical findings in a fatal case of generalized HandSchiiller-Christian disease in a man of 33 years are presented in case 1. The necropsy examination revealed granulomatous infiltration of the infra-orbital fat, the pituitary stalk, and the posterior lobe of the pituitary and also of the skull bones and the thyroid cartilage. The spinal dura mater contained multiple xanthomatous deposits which had produced degenerative changes in the cord substance by epidural compression. Interesting features were the tumour-like enlargement of the right heart, massive pancreatic and renal infiltration, also heavy constricting xanthomatous mantles which had produced compression of both kidneys and adrenal glands.Case 2 is an example of xanthomatous granuloma, confined to the lungs, in a man of 57 years, and not associated with diabetes insipidus. The histological appearances were those of long-standing chronic xanthoma with marked cystic changes in the lungs, dilated bronchioles, and thick-walled stenosed pulmonary arteries.The pathogenesis of Hand-Schiiller-Christian disease is briefly discussed.
About five months later she knocked her back at a local fairground but did not complain afterwards. Eleven days later she felt weakness in both legs, was unable to walk, and fell to the floor.On admission to hospital there was flaccid paraplegia of both legs, with loss of sensation below the sixth thoracic level, followed by urine retention and neck stiffness. A myelogram on the third day did not show evidence of spinal block. The patient was transferred to a neurosurgical unit (Mr. G. K. Tutton) on the fourth day: the sensory level had now reached the second thoracic segment; the patient was fully conscious with pain and stiffness in the back of the neck and 'pins and needles' in both arms. There was flaccid paraplegia of both legs with absence of tendon and plantar reflexes. Abdominal reflexes were absent and there was urinary Received for publication 21 April 1967. incontinence. Speech was not slurred; cranial nerve functions were normal; the optic discs were blurred. Weakness of the arms was not demonstrated. Temperature and respiration rate were normal; the pulse rate was 100 per minute and the blood pressure 210/150 mm.
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