What is the recommended treatment for sarcoidosis patients with 6th (sixth) nerve palsy?

Treatment of Sarcoidosis with 6th Nerve Palsy

For sarcoidosis patients with 6th nerve palsy, corticosteroids should be used as first-line treatment, with consideration of second-line agents such as methotrexate or anti-TNF inhibitors for refractory cases.

First-Line Treatment

Corticosteroids

• Initial treatment should consist of high-dose corticosteroids:
  • Methylprednisolone 1g daily for 3-5 days intravenously for severe cases 1
  • Followed by oral prednisone 60mg daily with a slow taper over 2 months 2
• This approach is supported by case reports showing improvement in cranial nerve palsies with corticosteroid therapy 2

Treatment Algorithm for Neurosarcoidosis with 6th Nerve Palsy

1. Assess severity and confirm diagnosis:

   • MRI brain with and without contrast to evaluate for dural thickening, enhancement, or mass effect 2
   • Look for other manifestations of sarcoidosis (pulmonary, ocular, cutaneous)
   • Consider CSF analysis to rule out other causes

2. Initial treatment:

   • Start high-dose corticosteroids
   • Monitor for clinical response within 2-4 weeks

3. If inadequate response to corticosteroids (occurs in ~70% of cases) 3:

   • Add second-line agent:
     • Methotrexate (61% response rate in neurosarcoidosis) 3
     • Or cyclophosphamide for more severe cases (90% response rate) 3

4. For refractory cases:

   • Consider anti-TNF therapy (infliximab or adalimumab) 4
   • Intravenous immunoglobulin (IVIg) may be beneficial in select cases 5

Evidence Quality and Considerations

• The European Respiratory Society (ERS) guidelines note that while there are no specific recommendations for treatment of neurological manifestations of sarcoidosis, current practice includes corticosteroids as first-line therapy 4
• Evidence suggests that agents other than corticosteroids may have increased efficacy with lower morbidity for neurosarcoidosis 3
• The response rate to corticosteroids alone for neurosarcoidosis (excluding isolated facial nerve palsy) is only about 29%, highlighting the need for additional therapies in many cases 3

Monitoring and Follow-up

• Regular ophthalmological examinations to assess improvement in 6th nerve function
• Monitor for side effects of immunosuppressive therapy
• Consider neuro-ophthalmology consultation for persistent diplopia 1
• For persistent symptoms, nonsurgical options such as temporary prisms may be considered 1

Important Caveats

• Unlike 7th nerve palsy (which often resolves spontaneously), other cranial nerve manifestations of neurosarcoidosis typically require more aggressive and prolonged treatment 6
• Delayed diagnosis and treatment can lead to permanent neurological deficits
• Treatment duration is typically prolonged for neurosarcoidosis compared to other manifestations 6
• Avoid tapering corticosteroids too quickly as recurrence of disease is common 4