Clinical Neuroimmunology: Multiple Sclerosis and Related Disorders 2e

by S.A. Rizvi, J.F. Cahill, P.K. Coyle, eds.
Humana Press,Imprint Academic, 2020, 351 pages
reviewed by T. Nelson

he best way to learn a medical topic is to study one disease at a time. You'll invariably find that one becomes your favorite disease, and you can relate everything else to how similar it is to it.

This is a great collection of articles on neurological demyelinating autoimmune disorders, mainly MS. The first 42 pages of background on the biochemistry and immunology are somewhat disorganized, but the remainder is well organized, non-repetitive, and well written. MS is far more complicated than a simple autoimmune response to myelin basic protein as we were once taught. It is now considered 30–50% genetic, with over 230 susceptibility genes, the most important being HLA-DRB1*1501, a human leukocyte antigen protein involved in cell recognition. There are also suspicions about viruses such as Epstein-Barr virus (infectious mononucleosis) and HHV6. Even the intestines, which people don't realize are one of the most important organs of the immune system, are also suspected in MS.

MS is dependent on geography, and deficiency in vitamin D, which regulates the immune system, is a big risk factor. Spending the first ten years of one's life in an area of good sunshine reduces your lifetime risk. Vitamin D levels decrease after onset of MS, but it's not yet clear how beneficial dietary supplements are.

An MRI scan is essential for diagnosis. New quantitative MRI technologies such as syMRI and the oddly named QRAPMASTER (Quantification of Relaxation times And Protein density by Multi-echo Acquisition of Saturation recovery using Turbo spin-Echo Readout), will soon allow automatic analysis of demyelination in patients.

Some of the drugs used for MS, including Fingolimod and Glatiramer, may sound like characters from Lord of the Rings, but the trend is toward monoclonal antibodies. Indeed, Daclizumab (an anti-CD25 monoclonal, now withdrawn due to adverse events) was the first humanized monoclonal antibody ever approved by the US FDA for clinical use. But any drug that messes with the immune system is risky; many are teratogenic and had a Category D or X rating. (Although the book doesn't mention this, the FDA dropped the A, B, C, D, and X pregnancy categories in 2015.)

The most interesting part of this book is the section on related diseases, such as acute hemorrhagic leuko­encepha­litis, which is a severe form of ADEM, an autoimmune reaction to childhood viruses such as measles (and, very rarely, vaccination) that can permanently affect the brain.

Paraneoplastic disorders are a recently discovered type of autoimmune disorder that manifest as psychiatric symptoms. These are not mental disorders at all, but physical diseases in the brain that are caused by an immune response to a tumor elsewhere in the body, and they often appear before the tumor is manifested. An example is paraneoplastic cerebellar degeneration, which can produce uncoordination and negative affective symptoms. Another is NMDA receptor encephalitis, which is classically a sign of a teratoma somewhere outside the nervous system.

Other interesting disorders include stiff person syndrome and area postrema syndrome, which is manifested as intractable hiccups, nausea and vomiting. Many of these diseases have cuddly-sounding names, like POEMS, CLIPPERS, and PANDAS, but in reality they are hardly cuddly at all.

jan 01, 2021