A Case of Isolated ACTH Deficiency with Suspected Origin from Cervical Spondylotic Myelopathy

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The patient was a 46-year-old man who presented with fatigue for 6 months. His activities of daily living (ADL) gradually worsened and he was transported to our hospital’s emergency room because of difficulty in walking due to muscle weakness in the extremities. His weight had decreased by 20 kg in 6 months, and he was almost bedridden. Upon arrival, he presented with emaciation, hypotension, and muscle weakness predominantly in the proximal portions. However, he showed no clumsiness in the hands, pain, sensory disorders, or bladder-rectal disturbances. Only Hoffman’s reflex was positive. Deep tendon reflexes were almost normal in his upper limbs but flaccid in his lower limbs. The Japan Orthopaedic Association (JOA) score was 14/17. Images of the cervical spine showed a developmental narrow canal, cervical spondylotic changes at C4-7 levels, and cervical spinal cord compression with myelomalacia at C4-6 levels. These findings suggested cervical spondylotic myelopathy (CSM). However, laboratory data showed mild hyponatremia (sodium 135 mmol/L) and hypothyroidism (free thyroxine [T4] 1.00 ng/ml, thyroid-stimulating hormone [TSH] 6.62 µIU/ml). Based on radiology, endocrinology, and neurology consultations, the patient was diagnosed with an isolated adrenocorticotropic hormone (ACTH) deficiency. Hydrocortisone (20 mg/day) administration dramatically improved his symptoms and ADL. The JOA score improved to 17/17 over a few months, and the patient returned to his daily life. He has been followed-up without surgery until now.

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