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book reviews
neurology case studiesreviewed by T. Nelson |
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book reviews
neurology case studiesreviewed by T. Nelson |
Reviewed by T. Nelson
I was once shocked when a clinical colleague, supposedly a neurologist, said it was impossible to distinguish vascular dementia from Alzheimer's disease. I told my collaborator the colleague was wrong and added the diagnostic criteria to our grant. But she listened to the clinical person instead. Big mistake: the grant got bashed and the agency told her to add what I had added—almost word for word.
They lost the grant anyway for other reasons. But it confirmed my belief that having an ego bigger than one's knowledge base is an occupational hazard among MDs. Hence the need for yet another case book. This one is from hospital cases in Northern Ireland, where the NHS is combined with local councils into something called the HSC, or Health and Social Care.
The book has three great features: (1) It explains the cause of the disease in molecular terms when possible. (2) The author is honest about those times when the hospital makes mistakes (or sort-of honest; you have to read between the lines). And (3) the patients (or their surviving relatives) get a chance to give feedback. And boy, do they give it.
For instance, in Case #53, a person training to become a nurse got a ‘bizzing’ sensation in her head and lost consciousness. They gave her lamotrigine, an anti-seizure medication. Then she had a cautery procedure for dry eyes and ended up with orbital myositis. They gave her steroids. Then she got red cell aplasia. Eventually this poor lady was taking thirteen different medications and died from sepsis. The author dryly says better coordination of medical care is needed. The patient's mother, also a nurse, let them have it, calling them a ‘disaster’:
B had 15 consultants but there was no correspondence between them and each one was only interested in his/her piece of the jigsaw. . . It encouraged my daughters to be knowledgeable about their condition and not to tolerate arrogance from medical professionals . . . .
Say that with an Irish accent.
Many of the cases were some form of spinocerebellar ataxia (SCA), a hereditary disorder causing loss of coordination. Others had cerebellar problems including cerebellar cognitive affective syndrome, which we used to believe was not possible. But it turns out that the cerebellum is not just for motor coordination; some cognition happens there.
An encouraging sign in this book is the increasing recognition that over-aggressive treatment usually does more harm than good. For instance, in Case #48, a person had a vascular malformation called a hemangioma in the brain, but they left it untreated because surgery often makes it worse. In Case #19, a patient who “drank excessive amounts of beer” and had hep C from drug use was found to have superficial siderosis, which means iron on the outer surface of the brain. In earlier times, they would have given him deferiprone, an iron chelator. But 60% of the time deferiprone has no effect and 25% of the time it makes the condition worse, so (quite reasonably) the patient declined treatment. So they gave him a crutch instead. In Case #50, a patient had surgery to remove a clot, after which CT showed a big infarction that was not there before. In Case #24, the poor patient came in with a tingling right toe and went out with diabetes, a side effect of treatment.
A big one is progressive multifocal leucoencephalopathy (PML), an opportunistic infection of the brain caused by JC virus, which is normally harmless. Anything that suppresses the immune system can trigger it, including the antibodies Natalizumab (which is now withdrawn) and Rituximab. Another antibody called Alemtuzumab is now restricted because it causes Grave's disease in 30% of patients and a rare fatal cardiovascular disorder in some patients. The author says the adenovirus Covid vax ChAdOx1 nCov-19 (the ‘clot shot’) screws up platelet factor 4, causing blood clotting in cerebral venous sinuses. This had a 40% mortality.
For some, the problem was the bureaucracy: in Case #22 the patient could not be treated because the system wouldn't pay for 3,4-diaminopyridine to treat his Lambert-Eaton syndrome. This easily-manufactured drug is unavailable because government attempts to enhance development of drugs for rare diseases allowed drug companies to price it so high that it's now unavailable for any disease.
A lot of progress is being made. Researchers are content to let the media brand people as conspiracy theorists, but among their peers most doctors will admit that many kinds of medical treatment cause as many problems as they solve. Sometimes they experience it personally. I wouldn't trust any doctor who tried to deny it.
Good quality MRI and CT images; has an index.
aug 27, 2024