Doctor University of Massachusetts - Baystate Health, United States
Introduction: Stiff Person Syndrome (SPS) is a rare autoimmune neurological disorder characterized by progressive muscle stiffness and rigidity. It has rarely been described in association with secondary adrenal insufficiency. We present a patient with known SPS later presenting with adrenal crisis.
Description: 47-year-old female with a known diagnosis of SPS with positive anti-glutamic acid decarboxylase antibodies presented with painful spasms leading to a fall, after she missed her intravenous immunoglobulin session the week prior. She was noted to be hypotensive and hypothermic prompting ICU admission for vasoplegia thought to be secondary to escalating doses of sedatives for spasm relief. Of note, the patient had not been exposed to any meaningful doses of steroids prior to admission. The patient had an unrevealing CT head and MRI brain on admission and her diagnostic work up suggested against active infection with absent leukocytosis and normal inflammatory markers. Labs demonstrated a markedly low cortisol level with a normal ACTH, and her hemodynamics showed improvement with stress dose hydrocortisone. Subsequent cortisol levels normalized, and she was safely discharged home days later.
Discussion: As an auto-immune entity, SPS has been associated with other autoimmune disorders including Addison’s disease. There are also reported cases of stiff-person-like syndrome associated with pan-hypopituitarism, and links of SPS with adrenal insufficiency secondary to pituitary adenomas. The overwhelming majority of these very rare cases describe individuals first diagnosed with adrenal insufficiency, followed by the later development of muscle stiffness or flexion contractures suspicious for SPS. Our patient carried a formal diagnosis of SPS from a specialty center for over a year before she presented with adrenal crisis from secondary adrenal insufficiency, not caused by a structural process like a macro-adenoma as previously described in the literature amongst her age group. This case highlights that clinicians should keep a differential diagnosis of adrenal insufficiency in mind when a patient with SPS presents with vasodilatory shock.
