Christmas Neurology

christmasmind_oval_ornament
Christmas Ornament from Cafe-Press

The Christmas Tree Sign
Keyoumars Ashkan and Adrian Casey
J Neurol Neurosurg Psychiatry 1999;67:824
xmas tree
Back pain and sciatica in patients with neurofibromatosis may result from a multitude of pathological processes which include scoliosis, degenerative diseases of the spine, and spinal tumours. Neurofibroma, schwannoma, meningioma, and glioma can all occur. When specific tumours are responsible for the symptoms, surgical excision can offer effective treatment. Many patients, however, have widespread disease. We recently treated a patient with multiple neurofibromas which involved several nerve roots and the cauda equine (figure). The extensive distribution of the tumours produced an MRI picture resembling a Christmas tree. In patients with “the Christmas tree sign” the challenge is in the correct diagnosis of those tumours which produce the symptoms if surgery is to remain a realistic management option.

A Christmas Tree Cataract
E Obi and C Weir
BMJ 2010; 341, (Published 8 December 2010)
cataratc
A 73 year old woman referred for cataract surgery had on examination a left Christmas tree cataract (fig 1A). Highly reflective, iridescent, polychromatic crystals were noted within the lens. Their colours varied according to the angle of the incident light, (fig 1B, C). We postulate that Christmas tree cataracts result from the accelerated breakdown of membrane associated proteins. The peptides and amino acids accumulate in the lumen of the reticular meshwork, and cystine is concentrated beyond the level of crystallisation, giving rise to growing crystals.

A Christmas Carol: A memorable patient
Stephanie Davies
BMJ. 1998 December 19; 317(7174):1732.
It was a few weeks before Christmas on a dull, chill November morning when Jason and his parents came to see me. As I listened to the, alas, too familiar tale of a young boy, difficult and unreasonable at home who was misbehaving at school, my heart sank and I began to feel as depressed and hopeless as everyone in the room. Jason sat quietly, certainly not demonstrating the hyperactivity suggested by his teacher. Assessment and examination showed that he was a normal and intelligent boy, so was this attention deficit hyperactivity disorder and would the magic pill help?

We discussed strategies to help based mainly on the premise of rewarding good behaviour and ignoring the bad coupled with praise and appreciation. Unfortunately, at this time, there did not seem to be any good behaviour to reward or any achievements to praise.

The review took place some time shortly before Christmas. I sat with prescription pad at the ready, prepared to offer methylphenidate if there was no other way. Jason came in like spring sunshine and his parents were glowing with pride and happiness. What could have wrought this transformation? It seemed unlikely that our previous consultation could have had such a dramatic effect.

I was correct. The magic had been woven by the muse of drama and Charles Dickens. Jason had starred as Scrooge in the school production of A Christmas Carol and was a smash hit. He had even saved the day by playing the part when a less intrepid thespian was incapacitated by stage fright.

Eight months passed before I saw Jason and his father again. With bated breath I inquired about progress. With delight I heard that it had been maintained, culminating in an excellent school report at the end of the academic year.

How important it is for us all to have some measure of success and how I wish all my patients could be a star for a day. I was certainly grateful that Jason and his parents allowed me to share his success. It has been one of my most memorable Christmas presents.

The Christmas “Clacker”
David Strachan
BMJ. 1994 December 24; 309(6970): 1747.
clacker
A 44 year old occasional drinker presented to the accident and emergency department at 5 30 am on New Year’s Day. A sensation at the back of his throat was causing a slight discomfort on breathing. On examination his uvula was found to be swollen. He had suffered the same problem on Christmas Day morning the previous year, the common factor being alcohol consumption during the previous evening. It is well known that alcohol consumption triggers or worsens snoring. Overindulgence causes muscle hypotonia and depression of the arousal mechanisms, leading to many people sleeping flat on their backs rather than on their sides. This narrows the airway and leads to a tendency to obstruction. If a kick in the back from a disgruntled partner does not rectify the situation and the “comatose” state persists then forced inspiration through a narrowed airway causes repeated trauma of the uvula and swelling.

Convulsions at Christmas
S Kirker
BMJ. 1992 December 19; 305(6868): 1580.
butcher
A healthy 26 year old butcher described convulsions without warning or focal onset between 11 am and noon on Christmas Eve in 1987, 1988, 1989, 1990, and 1991. He had no other convulsions or episodic symptoms that could be epileptic in origin and he had no relevant medical history. His usual working day was from 7 am to 6 pm but for the 14 days before Christmas he worked until at least 10 pm and as late as 1 am, seven days a week. He worked all through the night of 23 December. He did not miss any meals, and ate through the night as well. His usual alcohol consumption was 35 units a week but he drank less during these weeks. Results of examination and computed tomography were normal. Electroencephalograms in 1989 and 1992, each three months after the period of sleep deprivation, showed paroxysmal generalized spike and wave activity at 8 Hz, with a normal background rhythm. The patient was not prepared to change his working hours during the busiest time of year so we prescribed phenytoin over the second half of December. His electroencephalogram suggested he had an underlying tendency to have seizures, which only became apparent when his “seizure threshold” was lowered by sleep deprivation.

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