Treatment of Hashimoto's Encephalopathy
High-dose corticosteroids are the first-line treatment for Hashimoto's encephalopathy, with most patients showing significant improvement in neurological symptoms after initiation of therapy. 1
Initial Management
First-Line Treatment
- Intravenous methylprednisolone (1 g/day for 3-5 days) followed by oral prednisone (1-2 mg/kg/day) for acute severe presentations with significant neurological symptoms 1
- Oral prednisone (1-2 mg/kg/day) can be used for less severe presentations with a gradual taper over 6-12 months based on clinical response 1
- Monitor for steroid side effects including weight gain, hyperglycemia, hypertension, and mood changes 2
Hospitalization Criteria
- Consider hospitalization for patients with severe symptoms affecting daily activities or altered mental status for close monitoring and intravenous corticosteroid administration 2
- Patients with seizures, stroke-like episodes, or severe cognitive decline require inpatient management and neurological monitoring 1
Second-Line and Adjunctive Therapies
For Steroid-Resistant Cases
- Intravenous immunoglobulin (IVIG) can be used in patients who fail to respond to corticosteroids 3
- Plasmapheresis is effective for steroid-resistant cases, particularly in children with status epilepticus or severe neurological symptoms 3, 4
- Rituximab (anti-CD20 monoclonal antibody) may be considered in steroid-refractory adolescents, though careful monitoring for side effects is warranted 5
Maintenance Therapy
- Azathioprine (1-2 mg/kg/day) can be considered as a steroid-sparing agent for long-term management 2, 6
- Slow tapering of corticosteroids is recommended to prevent relapse, with some patients requiring low-dose maintenance therapy 1
Management of Concurrent Thyroid Dysfunction
Hyperthyroid Phase
- Beta-blockers (e.g., atenolol or propranolol) for symptomatic relief of adrenergic symptoms such as palpitations, tremors, and fever 2
- Monitor thyroid function every 2-3 weeks during the initial phase to detect potential transition to hypothyroidism 2
Hypothyroid Phase
- Levothyroxine replacement therapy should be initiated in patients with concurrent hypothyroidism 3
- Regular monitoring of thyroid function is necessary, as thyroid status may fluctuate during treatment 1
Monitoring and Follow-up
- Regular neurological assessment to evaluate treatment response 1
- Monitor anti-thyroid antibody titers (particularly anti-thyroid peroxidase antibodies) as they may correlate with treatment response 1
- Brain imaging (MRI) may be useful to exclude other causes of encephalopathy but is often not diagnostic for Hashimoto's encephalopathy 1
- Electroencephalography (EEG) to monitor patients with seizures or altered mental status 3
Common Pitfalls and Considerations
- Hashimoto's encephalopathy should be considered in any patient presenting with unexplained encephalopathy and seizures, even with normal thyroid function 3
- Symptoms that appear psychogenic in origin (such as give-way weakness and anatomically unexplainable pain) may actually be manifestations of Hashimoto's encephalopathy 6
- Avoid diagnosing somatoform disorders without first excluding autoimmune encephalopathy 6
- Abrupt cessation of corticosteroid therapy may lead to relapse; therefore, gradual tapering is essential 1
- Budesonide should be avoided in patients with cirrhosis or peri-hepatic shunting due to high risk of side effects from impaired first-pass metabolism 2
Special Populations
Pediatric Patients
- Children and adolescents may present with seizures or altered mental status as predominant symptoms 3
- Steroid-resistant cases in children may respond well to plasmapheresis or IVIG 3, 4
- Consider rituximab for steroid-refractory cases in adolescents, with careful monitoring for immunoglobulin levels 5