Raeder's Syndrome

Dear Doctors, My 7 year old daughter has just been diagnosed with Raeder's Syndrome. She has had an MRI scan in Bristol UK with no abnormalities detected. We have follow up appointments. She has recently been prescribed glasses and has tracking problems. She is awaiting a hearing test as she cannot hear below 25 decibels. My question is how can I best treat her headaches which are recurring and her ptosis as this is now causing her to miss school.

I would also welcome imformation on the long term progress of this condition as other literature has indicated it happens predominantly in male adults.

Many thanks, Carolyne (Mum)

[CCF Neuro[P] MD, RPS]

Dear Carolyne:

Raeder's is a type of cluster headache.  You are correct in thinking that it is primarily male dominant, but 15-20% occur in females.  The usual age range is 20-50 but we have seen it in children.  As a type of cluster headache, there seems to be a clustering and repetitive nature to them (hence the cluster definition).  In some patients with this disorder, it becomes chronic (meaning years and years) and the syndrome of chronic paroxysmal hemicrania develops.  Most patients develop a pattern that is almost clockwork in appearance to time of day and days of the month.  Unfortunately, the ptosis or eye lid problems may become permanent in a small percentage.  Recent evidence suggests that there is a lesion in the lateral hypothalmus on the side of the headache symptoms.  We have found benefit with using indomethicin and calcium channel blockers, and occasionally amitriptyline has a benefit.  We will sometimes use oxygen at the beginning of a headache and many patients respond to this.  There are reports in the literature with NSAIDs working in some patients.

I hope this helps.

Sincerely,

CCF Neuro MD

[Carolyne]

Thank you for your excellent response which was very helpful. Her headaches are being alleviated with paracetomal at the moment and  her ptosis has improved. We have noticed a pattern emerging of when the headaches "come on" which seems to be approx. every 3 weeks.

I am interested to know if there are any known genetic links as my husband has had two family members suffer fatal blood clots to the brain and we are concerned that if this syndrome has been passed down (even though not formally diagnosed before) the same could occur to our daughter. Secondly we have noticed that she becomes very tempramental before her headaches - we had wondered if hormones might also have been playing a part - is there any evidence to suggest this could be possible?

Many thanks in anticipation

Carolyne

[CCF Neuro[P] MD, RPS]

Dear Carolyne:

The likely behavioral change that is preceding her headache is likely the hypothalamic change inducing the cluster (my theory).  It might be a good way to treat the headache, when to initiate treatment.  The clots you indicate that may run in the family are likely not genetic in the true thought of inherited process.  True there are hypercoagulable states that may lead to stroke, or even mitochondrial diseases that may lead to strokes, but most are due to high cholesterol, bad hearts, overweight or obesity, diabetes, etc (environmental stresses on the cardiovascular system that we place on it).

Sincerely,

CCF Neuro MD