Abstract


Background: Patients with Cushing’s disease (CD) often experience disabling symptoms but the diagnosis may be delayed by failure to recognize the constellation of gradually developing symptoms. Some of these symptoms such as severe fatigue and weakness can also be seen with other conditions such as multiple sclerosis (MS), an autoimmune demyelinating disease affecting the brain and spinal cord. MS may be a steroid responsive disease. Failure to recognize MS in the setting of CD may lead to inappropriate treatment and worse prognosis. view more

Background: Patients with Cushing’s disease (CD) often experience disabling symptoms but the diagnosis may be delayed by failure to recognize the constellation of gradually developing symptoms. Some of these symptoms such as severe fatigue and weakness can also be seen with other conditions such as multiple sclerosis (MS), an autoimmune demyelinating disease affecting the brain and spinal cord. MS may be a steroid responsive disease. Failure to recognize MS in the setting of CD may lead to inappropriate treatment and worse prognosis. We report, for the first time, an unusual case of CD which masked MS, leading to severe flare-up of MS once CD was treated and cortisol level decreased.
Clinical Case: A 45-year-old woman presented to the Endocrinology clinic for follow up of CD. Ten years ago, she had a 50 lb weight gain following pregnancy. She was diagnosed with Polycystic Ovarian Disease (PCOD) and asked to pursue lifestyle changes for weight loss. She had progressive symptoms and developed hypertension, borderline diabetes, depression, and severe fatigue. Evaluation led to the diagnosis of CD: 24 hour urine cortisol of 116.3 mcg/24hr (Ref 4-50 mcg), cortisol level following 1 mg overnight dexamethasone administration was 18.2 mcg/dl (normal <1.8 mcg/dl). She was found to have a pituitary microadenoma measuring 5 X 7 mm for which she underwent trans-sphenoidal resection. Surgical pathology showed tumor cells diffusely immune reactive for chromogranin and ACTH and focally positive for prolactin. Post-operative cortisol levels were indeterminate for cure at 21.5 mcg/dl and 8 mcg/dl. She was placed on steroids which were weaned off. Patient developed progressive gait abnormalities over 3 months, with difficulty climbing stairs to eventually being unable to complete activities of daily living. Evaluation by Neurology with MRI brain revealed new demyelinating disease, compatible with a diagnosis of MS. Patient was placed on intermittent IV steroid pulses for multiple MS, with relapsing and remitting disease. She then developed recurrence of her Cushing symptoms including weight gain, diabetes, HTN and fatigue. She was found to have recurrence of her CD and underwent a second trans-nasal trans-sphenoidal resection of pituitary tumor. Pathology showed tumor was strongly immunoreactive to chromogranin and ACTH. Patient continues to be symptomatic, and the co-existence of MS and CD present a management challenge.
Conclusion: We report a rare case of MS co-existing with CD. MS was likely masked by CD and flared up when CD was treated. Periods of remission of MS with high dose steroids attested to the steroid responsiveness of the condition. We seek to heighten awareness about co-existing co-morbid conditions such as MS with CD so as to facilitate early diagnosis and improve clinical outcomes. To our knowledge, this is the first case in the literature reporting MS in the setting of CD.

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