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Addison's disease presenting with cerebral edema

C Geenen1, I Tein, R M Ehrlich

  • 1Division of Neurology, Hospital for Sick Children, Toronto, Ontario, Canada.

The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques
|May 1, 1996
PubMed
Summary

Addison's disease, a rare cause of increased intracranial pressure, was diagnosed in a 16-year-old female with cerebral edema. Prompt treatment with corticosteroids led to a full recovery, highlighting the importance of considering this condition in similar cases.

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Area of Science:

  • Endocrinology
  • Neurology

Background:

  • Increased intracranial pressure (ICP) with encephalopathy is a rare manifestation of Addison's disease.
  • Addison's disease, or primary adrenal insufficiency, results from the adrenal glands producing insufficient steroid hormones.

Observation:

  • A 16-year-old female presented with cerebral edema of unknown origin, exhibiting symptoms like headaches, fatigue, diarrhea, and cognitive decline.
  • Initial investigations revealed hyponatremia, hyperkalemia, low serum osmolality, high urine osmolality, and postural hypotension.
  • A definitive diagnosis of Addison's disease was confirmed via an ACTH stimulation test showing a blunted cortisol response.

Findings:

  • The patient demonstrated significant electrolyte imbalances and signs of adrenal insufficiency.
  • Cerebral edema resolved, and neurological symptoms improved following treatment with intravenous fluids and corticosteroids (Solu-Cortef).

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  • Long-term management with hydrocortisone and fludrocortisone (Florinef) resulted in sustained recovery without neurological sequelae for 18 months.

    • Implications:

    • Addison's disease should be included in the differential diagnosis for patients presenting with symptomatic cerebral edema and idiopathic intracranial hypertension.
    • Early recognition and treatment of Addison's disease can prevent severe neurological complications and lead to complete recovery.