Curious Cases of Painful Ophthalmoplegia
Keshav Shah1
1Internal Medicine, Anand Medical Nursing Home, Vadodara
Objective:
Importance of various investigations for diagnoses by exclusion and importance of starting treatment, which might be common for specific pathologies irrespective of different etiologies. (in a resource-limited environment)
Background:
There were two cases of adult women (Patient G and Patient V) residing in the same neighborhood. They presented with similar complaints of right-sided orbital pain, headache, and sixth cranial nerve palsy. 
Design/Methods:

On individual evaluation, Patient G had prominent right-sided dull orbital pain for 3 weeks with right-sided headache and diplopia for 1 week. Examination showed ipsilateral sixth nerve paresis. Investigations like Complete Blood Count, Complete Metabolic Panel, Chest X-ray, ANA, CSF Analysis (including Adenosine deaminase), ESR, and CRP were negative. MRI showed a single hypointense extra-axial lesion in right anteromedial temporal region, anterolateral to ipsilateral cavernous sinus. The lesion appeared to partially encase right sixth cranial nerve and approaching right superior orbital fissure. 

Patient V had similar complaints of right-sided headache for 4 weeks and diplopia for 1 week. Examination showed complete paralysis of ipsilateral sixth nerve palsy. Similarly, investigations were negative (including serum ACE levels). MRI revealed a single extra-axial hypointense lesion along right medial temporal region along with partial invasion of ipsilateral cavernous sinus. Mild asymmetric thickening of right fifth and sixth cranial nerves was seen.


Results:
Investigations were necessary to rule out differential diagnoses like tuberculous meningoencephalitis, thyroid ophthalmopathy, and other infectious, autoimmune, or neoplastic etiologies. By diagnosis of exclusion, patient G was diagnosed with Tolosa-Hunt syndrome. Though biopsy results were unavailable for patient V, she was provisionally diagnosed with Neurosarcoidosis. Both were started on Corticosteroids. On respective follow-up visits, both patients reported significant improvement in their symptoms. This further solidified the respective diagnoses in these patients.

Conclusions:
In a resource-limited environment, response to treatment is as important as other investigations to diagnose rare diseases by exclusion.
10.1212/WNL.0000000000201916