Question:
Are there any particular neurological issues regarding eye and head pain (touch sensitivity), worsening at the end of the day, common in Down syndrome?
This has been occurring for over 6 months but less than a year, I think. He has been to the ophthalmologist several times, the ED once because he told me he couldn’t see out of his left eye. It was resolved by the time we got there though. He had 2 CT scans, with and without contrast and has had MRI of brain and neck. Also CT scan of ear area since he had a mastoidectomy on his left side. No one has seen anything. Saw a neurologist who suggests it could be IIH. Ophthalmologist doesn’t see any clinical signs of that.
He doesn’t have it in the morning, but by dinnertime I can see he is uncomfortable and by 8 pm his eye hurts and the top of his head hurts. He has been getting drops / ointment to treat blepharitis, but the touch of a drop or ointment on his eyeball sends him into wailing and grabbing me for the pain. It somehow connects to a pain in his head (says not his brain, but the scalp) that is also super sensitive to any touch. Have been using a warm, wet washcloth on his eyes before drops/ointment, and the touch of that on his eyeball makes him gasp. One night I was putting his BIPAP mask on him and the strap touching the top of his head caused a painful outburst.
It occurs almost exclusively on the left side, but occasionally on the right.
I’ve emailed the neurologist asking what can be done next. Topamax was increased to 100mg twice a day for assumed neuralgia but hasn’t helped. I’ve had no response from the neurologist, and it’s been over 2 weeks since I asked. The pain is real, it’s acute and it isn’t resolving. I just can’t keep subjecting him to the pain of putting ointment in at night if it isn’t helping him. Drops in the morning go in with no problem. (Switched from drops to ointment at night because doc thought that might be less painful. It isn’t)
I know this is long, but he has been going through this for so long, and we’ve been shuttling back and forth to so many doctors and still have no answers. Or treatment. He hasn’t had any recent illness but has a hx of shingles.
Answer:
In our experience and from a review of available literature, the type of eye and head pain and touch sensitivity that you described is not common in people with Down syndrome.
Pain caused by stimuli that are normally not painful (such as light touch) is called allodynia. General information about allodynia can be found at this link.
Other conditions that have already been considered or might be considered include:
- Idiopathic Intracranial Hypertension (IIH) – Pseudotumor Cerebri
- It can be associated with headache and vision loss in some people. General information about IIH can be found at this link.
- A small study of 4 children with Down syndrome and IIH reported that none had headache or vision changes (Esmaili & Bradfield, 2007). We cannot make any definite conclusions about the presentation of IIH in people with Down syndrome from this limited amount of information.
- Keratoconus
- It is more common in people with Down syndrome and can cause eye pain. More information about keratoconus can be found at this link.
- An ophthalmology exam can be done to assess for and rule out keratoconus if it is not present.
- Atlanto-axial instability
- This occurs when the first vertebrae in the neck slips and does not line up correctly with the second vertebrae.
- The broader term, cervical subluxation, can occur when any of the cervical (neck) vertebrae do not line up with the one above or below it. The most common cervical vertebrae to become misaligned are the first about the second.
- It can cause pain, but the pain typically occurs in the back of the neck and/or head.
- It is not usually associated with eye pain.
- In a small study of individuals with Down syndrome with headaches, Siwiec et al. found:
- Headaches were not a common complaint
- Cervical subluxation was a frequent cause of headaches in the small number of people with Down syndrome who had headaches.
- Strokes
- More information on strokes can be found at this link.
- Moyamoya disease is more common in people with Down syndrome.
- Headache is a common symptom of moyamoya.
- t can cause strokes.
- t can cause transient ischemic attacks (TIA) sometimes called “mini strokes.” When an individual has a TIA, the neurological symptoms are temporary and resolve. They may come back but a pattern of recurrence as described in the question seems unlikely to be caused by moyamoya.
- Imaging of the blood vessels in the brain can assess for the changes associated with moyamoya.
- Sinus problems
- They are common in people with Down syndrome.
- They may be due to allergies, sensitivity to environmental irritants (e.g., smoke), and infections.
- Sensitivity to touch of the face is common with sinus problems.
- A CT scan of the sinuses can make the diagnosis.
- Post herpetic neuralgia (PHN)
- This is nerve pain seen after someone has had shingles.
- Interestingly, we have not seen much shingles in our patients with Down syndrome. The cases of shingles we have seen have tended to be in younger individuals (20s and 30s).
- Face pain and sensitivity to touch are common.
- The trigeminal nerve (nerve in the face) is one of the most common nerves affected.
- The pain is described as burning, sharp, or stabbing. It can be constant or intermittent.
- More than 90 percent of patients with PHN also have allodynia.
- There are several medications available to treat this pain.
- The pain may be chronic, so long-term therapy is often required.
- Trigeminal neuralgia
- This is a chronic pain condition that affects the trigeminal nerve, which carries sensation from your face to your brain.
- More information can be found at this link.
- Light touch can trigger the pain.
- Cluster headaches
- These occur in cyclical patterns or cluster periods.
- They are one of the most painful types of headaches.
- They often awaken the person in the middle of the night with intense pain in or around one eye or on one side of the head.
- More information is available at this link.
- Migraine headaches
- These are classically one-sided headaches described as throbbing.
- They are not typically associated with sensitivity to touch.
- More information is available at this link.
The conditions associated with sensitivity to touch in particular might be considered if not done so already.