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Saturday, April 21, 2012

WHAT?!? (Update in Neurology)

In the year 1986 I loved neurology so much that I arranged to spend 3 months in England watching the most godlike neurologists on earth practice their art at the National Hospital for Neurological Diseases in Queen's Square, London. People came from all over the world to be diagnosed by grand old men (there may have been women but I didn't see them) of nearly magical prowess who also taught medical students. They knew what people had, mostly by talking to them and watching them, doing neurological examinations, and less importantly by doing blood tests and imaging procedures. I was a sponge and would have gone into Neurology as a specialty except that the rest of medicine was also really interesting.

Now I am reading the MKSAP booklet on neurology as I continue to study for my internal medicine board exam, 90 pages of digested and compacted information about the brain in health and disease, treatments, recommendations for evaluation of symptoms, and although I have been practicing medicine and treating all kinds of neurological diseases, or so I thought, the whole field looks nearly entirely unrecognizable. Wow.

Dementia, for instance. I have been treating dementia nearly constantly for the over 20 years I have practiced. I mentally categorized dementia into Alzheimer's disease which comprised most of the elderly and forgetful patients, and strokes, also called multi-infarct dementia, and then "other weird things" which had names like frontotemporal dementia, Huntington's disease, normal pressure hydrocephalus and things I might have to look up, though they definitely weren't treatable. It turns out they still aren't treatable, at least not very effectively, unless the person is forgetful due to some deficiency or something, but now you definitely DO want to get a CT scan or MRI (dogma used to be that imaging was only for cases that looked atypical) and that you don't want to check for neurosyphilis (which we used to do all the time though the tests were never positive, maybe because of where I practiced.)  And the classification is different.  The weird things are still weird, but there is a classification called frontotemporal lobar disease (FTLD) that includes progressive non-fluent aphasia and semantic dementia, both of which involve primarily word finding issues, as well as frontotemporal dementia which involves prominent personality changes and odd behaviors.

Now for headaches. I have treated headaches forever, kept reading about treatments, had them mentally categorized as migraines, cluster headaches, muscle tension headaches and other weird stuff, with the largest category being muscle tension headaches. Wrong, apparently. 90% of significant headaches are considered to be migraine type headaches and the pain can be in the sinuses, the neck, forehead, or one side of the face, which was the default location according to my ancient and now apparently defunct wisdom. They are usually associated with sensitivity to light and sound, can have nausea and have some sort of aura or neurological symptom that precedes the headache (I already knew that.) Cluster headaches are just one of 3 types of headaches that are categorized as Trigeminal Autonomic Cephalgias, which also include Paroxysmal Hemicrania (sharp stabbing brief head pain, 15 times a day or more) and Short Lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing (SUNCT.) I thought that cluster headaches were treated with migraine medicines and prevention with verapimil and sometimes responded to oxygen. That's true, apparently, but they also respond to a course of oral corticosteroids in conjunction with an occipital injection of topical anesthetic and cortisone, which I have only used for what I thought of as occipital neuralgia, which was supposed to be an inflamed nerve in the back of the head, but is disturbingly missing from the syllabus! The weird stuff is reassuringly about the same as it used to be, things like spinal headaches, pseudotumor cerebri, analgesic associated headaches and thunderclap headaches which sometimes presage subarachnoid hemorrhage and need to be aggressively evaluated.

The treatments for migraine have changed a little bit, though I have been following this pretty well because of having to use these medications on my patients in primary care. There are a few surprises, though. Prevention with medications for seizure disorders, especially lamotrigine and topiramate, are familiar to me and is the use of various blood pressure medication. I had heard that the herbal medication feverfew was effective, but the syllabus only mentions butterbur (Petasites Hybridus) in the herbal category, but does so with none of the usual lack of enthusiasm that most herbals get.

Much of this syllabus is no different than what I thought I already knew, but here is a list of the things that I wouldn't have believed as recently as yesterday:
1. Hypothyroidism and celiac disease can cause ataxia. Celiac disease can also cause neuropathy.
2. 10% of patients with frontotemporal dementia will have ALS or the other way around. I always thought patients with ALS had no cognitive problems.
3. CT scan is recommended in anyone with a head injury whose headache lasts longer than 72 hours.
4. Botulinum toxin injections are used for treating benign essential tremor of the head.
5. The vocal cords can spasm causing a person to be unable to speak in certain circumstances and the treatment is botulinum toxin injection into the muscles controlling vocal cord contraction.
6. The most common cause of Guillain-Barre syndrome is an intestinal infection with Campylobacter Jejuni and patients can get dysautonomia including cardiac rhythm disturbances and constipation.

How much of this will I remember, I wonder, and how much of it will be true in another 10 years?

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