Primary hypothalamic lymphoma with panhypopituitarism presenting as Stiff-man syndrome

May Tze Lee, Ting I. Lee, Justin Ging Shing Won, Wing Keung Chau, Hong Jye Yang, Jer Chuan Li, Hong Da Lin, Kam Tsun Tang

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)124-128
Number of pages5
JournalAmerican Journal of the Medical Sciences
Volume328
Issue number2
DOIs
Publication statusPublished - Aug 2004

Fingerprint

Stiff-Person Syndrome
Lymphoma
Myalgia
Thyroxine
Hydrocortisone
Hypothalamic Neoplasms
Magnetic Resonance Imaging
Anterior Pituitary Hormones
Optic Chiasm
Adrenal Insufficiency
Hypopituitarism
Adjuvant Radiotherapy
Touch
Spasm
Anorexia
B-Cell Lymphoma
Visual Fields
Prolactin
Physical Examination
Vomiting

Keywords

  • Adrenal insufficiency
  • Diabetes insipidus
  • Hypothalamic lymphoma
  • Panhypopituitarism
  • Stiff-man syndrome

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Primary hypothalamic lymphoma with panhypopituitarism presenting as Stiff-man syndrome. / Lee, May Tze; Lee, Ting I.; Won, Justin Ging Shing; Chau, Wing Keung; Yang, Hong Jye; Li, Jer Chuan; Lin, Hong Da; Tang, Kam Tsun.

In: American Journal of the Medical Sciences, Vol. 328, No. 2, 08.2004, p. 124-128.

Research output: Contribution to journalArticle

Lee, May Tze ; Lee, Ting I. ; Won, Justin Ging Shing ; Chau, Wing Keung ; Yang, Hong Jye ; Li, Jer Chuan ; Lin, Hong Da ; Tang, Kam Tsun. / Primary hypothalamic lymphoma with panhypopituitarism presenting as Stiff-man syndrome. In: American Journal of the Medical Sciences. 2004 ; Vol. 328, No. 2. pp. 124-128.
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