Primary hypothalamic lymphoma with panhypopituitarism presenting as stiff-man syndrome.
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 2 days 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.
Division of Endocrinology and Metabolism, Department of Medicine, Taipei Medical University Affiliated Wan-Fang Hospital, Taipei, Taiwan.
May-Tze
MT
Lee
Ting-I
TI
Lee
Justin Ging-Shing
JG
Won
Wing-Keung
WK
Chau
Hong-Jye
HJ
Yang
Jer-Chuan
JC
Li
Hong-Da
HD
Lin
Kam-Tsun
KT
Tang
D000309:Adrenal Insufficiency / Q000175:diagnosis; D001921:Brain / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D007018:Hypopituitarism / Q000175:diagnosis* / Q000473:pathology; D007029:Hypothalamic Neoplasms / Q000175:diagnosis* / Q000473:pathology; D007031:Hypothalamus / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016750:Stiff-Person Syndrome / Q000175:diagnosis*; D013997:Time Factors