What is the diagnosis and management for a patient with sudden onset unilateral headaches, significant visual changes, and skin sensitivity, presenting with ptosis, blurred vision, and intermittent eye twitching?

Evaluation and Management of Unilateral Headache with Visual Changes and Ptosis

The patient's presentation strongly suggests a diagnosis of Tolosa-Hunt syndrome or a parasellar lesion requiring urgent MRI with contrast and MR venography to rule out serious pathology. 1

Clinical Assessment of Concerning Features

This patient presents with several concerning features that require immediate attention:

1. New unilateral headache pattern - Sudden onset of right-sided headaches that are not typical for the patient
2. Progressive neurological deficits:
   • Worsening ptosis of the right eyelid over 2 years with recent acceleration
   • Rapid deterioration of vision in the right eye over 4 months
   • Blurriness without peripheral field loss
   • Intermittent right eye twitching
3. Unusual sensory symptoms - Severe scalp and facial skin sensitivity

These features represent multiple red flags that indicate a potential secondary headache disorder requiring neuroimaging 1.

Differential Diagnosis

Most Likely Diagnoses:

1. Tolosa-Hunt syndrome - Painful ophthalmoplegia with cranial nerve involvement
2. Parasellar lesion (tumor, aneurysm, or inflammatory process)
3. Cavernous sinus pathology - Thrombosis or inflammation
4. Ophthalmoplegic migraine - Though less likely given the progression

Less Likely but Must Consider:

• Carotid artery dissection
• Cluster headache with secondary complications
• Orbital inflammatory disease

Diagnostic Workup

Immediate Imaging:

• Brain MRI with contrast and special attention to orbital/cavernous sinus - To evaluate for mass lesions, inflammation, or vascular abnormalities 2, 1
• MR venography (MRV) - To evaluate for venous sinus thrombosis 2

Additional Testing:

1. Complete neuro-ophthalmologic examination including:

   • Visual acuity testing
   • Visual field testing
   • Fundoscopic examination
   • Extraocular movement assessment
   • Pupillary responses 2

2. Laboratory studies:

   • ESR and CRP (to evaluate for inflammatory conditions)
   • Complete blood count
   • Basic metabolic panel

Management Approach

Initial Management:

1. Urgent neuroimaging as outlined above
2. Neurology consultation for specialized assessment
3. Symptomatic pain relief with acetaminophen or NSAIDs while awaiting diagnosis 1

Specific Management Based on Findings:

If Tolosa-Hunt syndrome:

• High-dose corticosteroids (prednisone 60-100 mg daily with taper)
• Response to steroids within 24-72 hours is characteristic and diagnostic

If Parasellar mass:

• Neurosurgical consultation
• Management depends on the specific pathology identified

If Cavernous sinus thrombosis:

• Anticoagulation therapy
• Antibiotics if infectious etiology suspected

Follow-up and Monitoring

1. Close monitoring of visual symptoms - Any worsening warrants emergency evaluation
2. Serial neurological examinations to track progression or improvement
3. Follow-up imaging based on initial findings and clinical course

Important Considerations and Pitfalls

• Do not attribute to migraine - The progressive neurological deficits and ptosis make a primary headache disorder unlikely 1
• Avoid diagnostic delay - The combination of headache with progressive visual changes and ptosis requires urgent evaluation
• Do not miss carotid dissection - Consider vascular imaging if MRI/MRV is negative but symptoms persist
• Beware of medication overuse - While treating symptoms, avoid frequent use of analgesics that could complicate the clinical picture 1

This patient's presentation with unilateral headache, progressive ptosis, visual changes, and skin sensitivity represents a neurological emergency requiring prompt imaging and specialist consultation to prevent potential permanent vision loss or other neurological sequelae.