Chief Complaint. History. History of Similar Episodes. A 10 Year-Old Boy With Headache

Transcription

1 A 10 Year-Old Boy With Headache Chief Complaint Recent Advances in Neurology year-old boy presented with his fifth lifetime bout of left-sided head pain followed by diplopia. Amy A. Gelfand, MD UCSF Headache Center UCSF Child Neurology Relevant Disclosures: None History Headache Location: left retro-orbital Headache Quality: constant and sharp, not pounding Associated symptoms: +nausea, vomiting Pertinent negatives: photophobia, phonophobia Sequence: Head pain X 2 days, then awoke 3 rd day with complete left ptosis and diplopia. Head pain remitted after day 3; ptosis/diplopia ongoing. ROS: No recent fevers or illnesses History of Similar Episodes 1 st episode: Age 3. Lasted 3 days. 2nd episode: Age 5. Lasted 7 days. 3rd episode: Age 8. Lasted 3 weeks. 4 th episode: Age 9. Six weeks to improve; residual mild left ptosis and anisocoria. 1

2 PMH and Family Hx Otherwise healthy; born at term Physical Examination (Day 7) -Normal general examination Normal developmental milestones No headaches between episodes No family history of headaches -Neurologic examination findings: Left pupil 6 mm and reacts sluggishly to 4 mm Right pupil 4 mm and briskly reactive to light Complete left ptosis Extraocular movements full (+subjective diplopia) Previous Studies MRI brain at 1st episode (age 3): Focal enhancement and thickening of cisternal portion of the 3 rd nerve MRI at time of 2 nd episode (age 5): Same MRI when asymptomatic (age 7): persistent nerve root thickening but no enhancement MRI at time of 3 rd episode (age 8): Enhances CSF at 4 th episode (age 9): 2 WBC, normal protein and glucose Current Investigations: CSF: OP normal. 2 WBC, normal glucose and protein. Normal IgG index and no oligoclonal bands. MRI: thickening and enhancement of cisternal left 3 rd nerve 2

3 MRI: 1 st episode, Age 3 A MRI findings current episode B T1 post-gadolinium T1 post-gadolinium Audience Response Question #1 Audience Response Question #2 What would you do next? What is your working diagnosis? A. Start a migraine preventive medication B. No intervention C. Refer to Neurosurgery for biopsy of enhancing nerve D. Treat with corticosteroids E. CT chest/abdomen/pelvis F. Send CSF for viral PCRs and treat with IV acyclovir 10% 19% 7% 34% 15% 15% A. Migraine with aura B. Status migrainosus C. Ophthalmoplegic migraine D. Oculomotor schwannoma E. Neurosarcoidosis F. Lymphoma M i g r a i n e w i t h... 0% 1% S t a t u s m i g r a i n... O p h t h a l m o p l e g i... 43% O c u l o m o t o r s c h... 29% N e u r o s a r c o i d o s... 21% L y m p h o m a 7% 3

4 Ophthalmoplegic Migraine Rare disorder: annual incidence 0.7 per million Classified in the International Classification of Headache Disorders, Second Edition, as a cranial neuralgia Hansen et al, Acta Neurol Scand, 1990 ICHD-II, Cephalalgia 2004 Ophthalmoplegic Migraine Diagnostic criteria: 1. At least two attacks 2. Migraine-like headache accompanied or followed within 4 days of its onset by paresis of one or more of the third, fourth, and/or sixth cranial nerves 3. Parasellar, orbital fissure, and posterior fossa lesions ruled out by appropriate investigations ICHD-II: in some cases MRI shows gadolinium uptake in the cisternal part of the affected cranial nerve ICHD-II, Cephalalgia 2004 Ophthalmoplegic Migraine : What do we know about it? Is it migraine? Systematic review of all cases of ophthalmoplegic migraine published in English in the MRI era ( ) Ophthalmoplegic migraine : Age at onset n=84 (80 in literature, 4 from our own experience) Median age at onset: 8 years; Range: 7 months to 50 years 4

5 Ophthalmoplegic migraine : Attacks Persist Into Adulthood Clinical Phenotype of Ophthalmoplegic Migraine Slight female predominance (63%) Third nerve most commonly affected (77%) Almost always unilateral and side-locked (97%) Lag from headache onset to ophthalmoplegia onset typically a couple days (range 0-14 days) Ophthalmoplegia tends to last longer with subsequent episodes Recovery from later episodes may be incomplete Head Pain of Ophthalmoplegic Migraine is not necessarily migraine-like Ophthalmoplegic Migraine : Clues to etiology? Headache location (n=53) Headache quality (n=38) 30 (57%) peri/retro-orbital 17 (32%) ipsilateral 6 (11%) other 26 (68%) migrainous 12 (32%) not migrainous Photophobia (n=40) 26 (65%) Phonophobia (n=25) 14 (56%) Nausea (n=38) 25 (66%) Vomiting (n=35) 24 (69%) Persistent neurologic findings after a bout (n=35) 19 (54%) Cerebrospinal fluid findings (n=32) 30 normal (94%) one case had IgG index 0.87 one had a single oligoclonal band (only two cases involving 4 th nerve) Vascular imaging (n=42) 39 normal (93%) one had a venous angioma two cases of nerve-vessel contact CBC (n=14) and ESR (n=21) Normal when tested (as were virologic studies) Gelfand et al, J Child Neurol

6 Ophthalmoplegic Migraine : MRI findings Audience Response Question #3 Should ophthalmoplegic migraine be renamed? Findings on contrast MRI during an acute attack (n=52): Nerve root thickening (n=50) Nerve root enhancement (n=52) 38 (76%) 39 (75%) A. Yes, use of the term migraine is confusing and inaccurate B. No, changing the name will create confusion C. No, I m still not convinced it isn t a migraine variant 50% 31% 19% When imaged between attacks, focal thickening persists but no longer enhances Is this a recurrent focal demyelinating disorder? Y e s, u s e o f t h... N o, c h a n g i n g t... N o, I m s t i l l... Renaming Ophthalmoplegic Migraine Use of the term Migraine is confusing and leads to inappropriate therapies Suggested new terminology: Recurrent ophthalmoplegic cranial neuropathy Treatment of Recurrent Ophthalmoplegic Cranial Neuropathy No controlled trials Acute therapy: Several published cases suggest corticosteroids may be of benefit For persistent deficits (eye misalignment): Strabismus surgery or botulinum toxin No role for migraine-specific therapies Gelfand et al, J Child Neurol

7 Case Conclusion A 10 Year-Old Boy With Headache Treatment: Prednisone 1mg/kg daily X 5 days Outcome: Near complete resolution of ptosis and diplopia in 48 hours Recent Advances in Neurology 2013 Two-year follow-up: 6 th episode age 12. Treated with Prednisone and recovered back to baseline within 12 days. Relevant Disclosures: None Amy A. Gelfand, MD GelfandA@neuropeds.ucsf.edu UCSF Headache Center UCSF Child Neurology Oculomotor Schwannoma Migraine with visual aura Oculomotor Schwannoma Ophthalmoplegic migraine Clinical Presentation MRI findings Persistent or slowly progressive 3 rd nerve palsy Persistent enhancement of the 3 rd nerve Episodic 3 rd nerve palsy with spontaneous remissions Enhances during bouts, not during remissions New York Times, Migraine Art : opinion/ _migraine_slideshow_index-9.html 7