13.9 Paratrigeminal oculosympathetic (Raeder’s) syndrome

Identification: Male patient, 56 years old, with a history of orbital pain and unilateral ptosis for 2 months.

History of Present Illness:
Ocular Symptoms:
Pain:

Location: Right hemi-fronto-orbital

Characteristic:

Constant, with deep pressure (6-8/10 VAS)
Exacerbations with stabbing pain (9/10) without specific triggers
No response to common analgesics

Autonomic Signs:

Partial ptosis (Müller's muscle)
Ipsilateral anhidrosis restricted to the forehead (does not affect face or body)
Mild miosis (2mm pupillary difference)

Associated Symptoms:
Paresthesias in right V1-V2
History of cluster headache (5 years ago)

Relevant History:
Smoking (30 pack-years)
Untreated dyslipidemia

Physical/Neurological Examination:
Pupillary Assessment:

Partial Horner's syndrome (miosis + ptosis, without generalized anhidrosis)
4% cocaine test positive (contralateral pupillary dilation)

Trigeminal Sensory:

Hypoesthesia in right V1-V2
Decreased corneal reflex

Vascular Examination:

Absent carotid bruits
Symmetrical temporal pulses

Complementary Exams:
3T MR Angiography of Cerebral Arteries:

Segmental dissection of the right extradural internal carotid artery
Compression of the trigeminal ganglion by parietal hematoma

Positron Emission Tomography (PET-CT):

Absence of inflammatory/neoplastic processes

Carotid Doppler Ultrasound:

Turbulent flow in the C1-C2 segment