Primary Hypothalamic Lymphoma with Panhypopituitarism Presenting as Stiff-Man Syndrome

• Published in: The American journal of the medical sciences 2004-08, Vol.328 (2), p.124-128
• Main Authors: Lee, May-Tze, Lee, Ting-I, Won, Justin Ging-Shing, Chau, Wing-Keung, Li, Jer-Chuan, Lin, Hong-Da, Tang, Kam-Tsun, Yang, Hong-Jye
• Format: Article
• Language: English
• Subjects: Adrenal insufficiency; Adrenal Insufficiency - diagnosis; Adrenals. Adrenal axis. Renin-angiotensin system (diseases); Biological and medical sciences; Brain - pathology; Diabetes insipidus; Diagnosis, Differential; Diseases of striated muscles. Neuromuscular diseases; Endocrinopathies; General aspects; Hematologic and hematopoietic diseases; Humans; Hypopituitarism - diagnosis; Hypopituitarism - pathology; Hypothalamic lymphoma; Hypothalamic Neoplasms - diagnosis; Hypothalamic Neoplasms - pathology; Hypothalamus - pathology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, B-Cell - diagnosis; Lymphoma, B-Cell - pathology; Magnetic Resonance Imaging; Male; Medical sciences; Middle Aged; Neurology; Non tumoral diseases. Target tissue resistance. Benign neoplasms; Panhypopituitarism; Stiff-man syndrome; Stiff-Person Syndrome - diagnosis; Time Factors
• ISSN: 0002-9629; 1538-2990
• DOI: 10.1097/00000441-200408000-00010

We report an unusual case of primary hypothalamic lymphoma with hypopituitarism presenting as Stiff-man syndrome (SMS). A 64-year-old man was hospitalized due to a 3-week history of general weakness, anorexia, vomiting, weight loss, and muscle pain and spasms precipitated by motion and tactile stimuli resulting in muscle stiffness and difficulty in mobility. Physical examination revealed normal sensorimotor function and reflexes, except for bitemporal visual field defect. Routine laboratory and gastrointestinal examinations provided no remarkable clues. Endocrine assessment revealed low levels of morning cortisol, thyroxine, and anterior pituitary hormones but an increase in prolactin level. The patient’s muscle pain and stiffness improved dramatically within 2days after hydrocortisone therapy and thyroxine replacement. Magnetic resonance imaging (MRI) of the brain confirmed an 18-mm enhancing hypothalamic tumor with optic chiasm involvement, which proved to be a B-cell lymphoma. The results of the extensive studies for systemic lymphoma were negative, suggesting a primary hypothalamic lymphoma. The tumor regressed completely and was invisible on MRI scan after adjuvant radiotherapy. The patient’s condition was satisfactory and there was no recurrence of SMS during the 2-year follow-up period. This case demonstrated that primary hypothalamic lymphoma complicated with adrenal insufficiency may manifest as SMS. Early diagnosis and prompt intervention can lead to a favorable outcome and reduce morbidity.