The 9 neurological manifestations of anti MOG antibody disorder

Autoimmune disorders are probably the most proliferative field of neurology. It seems like there is a blazing headline every week announcing a new antibody disease. Many of these antibodies are esoteric, but some shake the foundations of medical practice. Anti-MOG antibody is one of those which requires you to stop and pay attention, and it has significantly affected neurological practice in a very big way.

By Simon Caulton – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=20522656

Perhaps the most important thing about anti-MOG antibody disease is that, like the chameleon, it presents in many guises. For the neurologist therefore, the first thing is to recognise these varied manifestations. Here then is a quick list of the 9 manifestations of anti MOG antibody disorder.

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1. Optic neuritis (ON)

2. Neuromyelitis optica spectrum disorders (NMOSD)

3. Multiple sclerosis (MS)

4. Acute disseminated encephalomyelitis (ADEM)

5. Multiphasic disseminated encephalomyelitis (MDEM)

6. Isolated transverse myelitis (TM)

7. Leukodystrophy-like phenotype

8. Cerebral cortical encephalitis

9. Combined central and peripheral demyelinating syndrome (CCPD)

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Optic Nerve Side View. Francisco Bengoa on Flikr. https://www.flickr.com/photos/frecuenciamedicafb/7404373800

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You can explore anti MOG antibody disorder further in Neurochecklists under the following titles:

Image from page 400 of “Diseases of the nervous system” (1910). Internet Archive Book Images on Flickr. https://www.flickr.com/photos/internetarchivebookimages/14586405720/

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For a detailed review and guidance, check this paper in Journal of Neuroinflammation:

 MOG encephalomyelitis: international recommendations on diagnosis and antibody testing.

By PecatumOwn work, CC BY-SA 4.0, Link

7 ominous signs that suggest you need to see a neurologist

Neurologists spend most of their time diagnosing benign conditions which are curable or treatable, or at least people learn to live with. Every now and then we see people with startling symptoms such as coma, convulsions, neck stiffness, or paralysis. These are obviously concerning to patients and their families who have a foreboding of diseases such as meningitis, epilepsy, and stroke. Serious as these disorders are, they at least announce themselves and show their hands. Many other neurological symptoms unfortunately give no hint of the serious diseases that follow in their trail. That is when things get a bit tricky.

Ominous. Ankakay on Flikr. https://www.flickr.com/photos/ankakay/4101391453
Ominous. Ankakay on Flikr. https://www.flickr.com/photos/ankakay/4101391453

What are these seemingly benign symptoms which jolt neurologists out of their blissful complacency? What are these red flag symptoms that pretend they are grey? Here are my 7 deceptively ominous neurological signs everyone should know about.

7. A numb chin

By Henry Vandyke Carter - Henry Gray (1918) Anatomy of the Human Body (See "Book" section below)Bartleby.com: Gray's Anatomy, Plate 784, Public Domain, https://commons.wikimedia.org/w/index.php?curid=531758
By Henry Vandyke CarterHenry Gray (1918) Anatomy of the Human Body (See “Book” section below)Bartleby.com: Gray’s Anatomy, Plate 784, Public Domain, https://commons.wikimedia.org/w/index.php?curid=531758

This must be the most deceptive sinister symptom in neurology. Not many people will rush to their doctors to complain about a numb chin, but it is a symptom that makes neurologists very nervous. This is because the chin gets its sensory supply from the mandibular branch of the fifth cranial nerve, also called the trigeminal nerve because it has three branches. And neurologists know that, for some bizarre reason, cancers from other parts of the body occasionally send deposits to this nerve. The numb chin syndrome is therefore not to be treated lightly.

6. Muscle twitching

OK, don’t panic yet. We have all experienced this; a flickering of an overused and tired muscle; a twitching of the odd finger; the quivering of the calf muscles in older people. Neurologists call these fasciculations, and they are only a concern if they are persistent, progressive, and widespread. And also usually only if the affected muscles are weak. In such cases neurologists worry that fasciculations are the harbingers of sinister diseases, particularly motor neurone disease (MND), better known in America as amyotrophic lateral sclerosis (ALS) or Lou Gehrig disease. Many people with muscle twitching will however have nothing seriously wrong with them, and many will be shooed out of the consulting room with the label of benign fasciculations syndrome (we love our syndromes, especially when they are benign). There are many other causes of fasciculations, but MND is clearly the most sinister of them all.

5. Transient visual loss

Scott Maxwell on freestockphotos. http://www.freestockphotos.biz/stockphoto/9747
Scott Maxwell on freestockphotos. http://www.freestockphotos.biz/stockphoto/9747

Neurologists often ask people with headache if their vision blurs or disappears for brief periods of time. These visual obscurations are not as dramatic as the visual loss that accompanies minor strokes or transient ischaemic attacks (TIAs). Visual obscurations affect both eyes and last only a few seconds. They are the result of sudden but brief increases in an already elevated pressure in the head. This may occur with relatively benign conditions such as idiopathic intracranial hypertension (IIH), but it may also portend a serious disorder such as a brain tumour.

4. Sudden loss of bowel or bladder control

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Loss of control down there would surely concern many people, but often not with the urgency it deserves. There are many non-neurological causes of bowel or bladder incontinence, but a sudden onset suggests that it is arising from the nervous system. The worrying diagnoses here are spinal cord compression and spinal cord inflammation (transverse myelitis). These disorders are often associated with other symptoms such as leg stiffness and weakness, but I really wouldn’t wait until these set in before I ask to see a neurologist.

3. Saddle anaesthesia

bicycle-saddle-791704_1920

Whilst we are on the topic of things down there, a related sinister symptom is loss of sensation around the genitals and buttocks, something your doctor will prudently call saddle anaesthesia. This arises when the nerves coming off the lower end of the spinal cord, collectively called the cauda equina, are compressed. The unpalatable condition, cauda equina syndrome (CES), worries neurologists because the compression may be due to a tumour in the spinal canal.

PS: The bicycle saddle is an apt analogy, but if you prefer horse riding, below is an alternative image to soothe your hurt feelings.

 

By BLW - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=1956552
By BLW – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=1956552

2. A painful droopy eyelid

A droopy eyelid is a deceptively benign symptom which worries neurologists. This symptom, which neurologist prefer to call ptosis, is particularly concerning if it is accompanied by double vision. One worrying disorder which causes ptosis is myasthenia gravis (MG), and this presents with ptosis on both sides. More sinister is ptosis which is present only on one side, particularly if it is painful. This may be caused by brain aneurysms, especially those arising from a weakness of the posterior communicating artery (PCOM) artery. As the aneurysm grows, it presses on the third cranial or oculomotor nerve, one of three nerves that controls the eyeballs and keeps the eyelids open. An aneurysm is literally a time-bomb in the brain as they wield the threat of bursting and causing a catastrophic bleeding around the brain. This makes ptosis an ominous, but also a helpful, neurological symptom.

By Cumulus z niderlandzkiej Wikipedii, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=3167579
By Cumulus z niderlandzkiej Wikipedii, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=3167579

There are many other causes of ptosis including Horner’s syndrome, so don’t panic yet but get that eyelid checked out if it refuses to straighten out.

 

1. Thunderclap headache

By © Marie-Lan Nguyen / Wikimedia Commons, CC BY 2.5, https://commons.wikimedia.org/w/index.php?curid=24189896
By © Marie-Lan Nguyen / Wikimedia Commons, CC BY 2.5, https://commons.wikimedia.org/w/index.php?curid=24189896

thunderclap headache is a symptom that means exactly what it says on the label! Neurologists will ask if the onset felt as if one was hit by a cricket bat. Even though most people have never been so assaulted, almost everyone with thunderclap headache readily agree this is what it feels like. It is such a distressing symptom that it doesn’t strike the afflicted person (pun intended) that their doctors are more concerned about investigating them, then they are in curing their headache. They patient is rushed to the CT scanner, and then subjected to a lumbar puncture. The doctors then heave a huge sigh of relief when the spinal fluid shows no blood or blood products, reassured that the patient has not suffered a subarachnoid haemorrhage (SAH) from a ruptured a brain aneurysm. The patient, who now has just another headache, is left to get to grips with their now, suddenly, very uninteresting symptom. There are many other causes of a thunderclap headache, but a ruptured aneurysm is the most sinister. If you develop a thunderclap headache, don’t wait to see a neurologist…just get to the nearest hospital!

PS: Don’t feel aggrieved if you are across the Pacific; it is also a thunderclap headache if it felt like being hit by a baseball bat!

Baseball bat in sun. Peter Chen on Flikr https://www.flickr.com/photos/34858596@N02/3239696542
Baseball bat in sun. Peter Chen on Flikr https://www.flickr.com/photos/34858596@N02/3239696542

 

Want to check out more ominous signs? Check out Smart handles and red flags in neurological diagnosis by the neurologist Chris Hawkes in Hospital Medicine.

 

Why is chronic Lyme disease so frustrating to neurology?

Lyme disease is a well-known infection. It takes its name from Lyme, Connecticut, where it was first recognised as a distinct disease in 1975. The disease is caused by the infamous Borrelia species which get into humans through tick bites. The transmission typically occurs when the victim is taking a gingerly walk in deer-inhabited forests, usually in the pleasant months of May or June. The tick may leave a signature skin rash, erythema migrans. In the ideal situation, the clear history and a positive Lyme serology test make the diagnosis. A short treatment course with an antibiotic such as doxycycline or ceftriaxone and, hey presto, Lyme disease is cured, totally and permanently. And doctor and patient live happily ever after….

By USDA photo by Scott Bauer - Image Number: K5437-3.http://www.ars.usda.gov/is/graphics/photos/may01/k5437-3.htm, Public Domain, https://commons.wikimedia.org/w/index.php?curid=245466
By USDA photo by Scott Bauer – Image Number: K5437-3.http://www.ars.usda.gov/is/graphics/photos/may01/k5437-3.htm, Public Domain, https://commons.wikimedia.org/w/index.php?curid=245466

The above scenario, unfortunately, only plays out on planet Utopia. On planet earth, things are rarely that straightforward. In reality, the story is often vague and devoid of ticks, deer, and forests. The Lyme blood test is often ambiguous and frequently misleading. And in many cases, the antibiotic only partially improves the symptoms. The end result is a frustrated patient and a baffled doctor. Several Google searches after and the patient is convinced they have chronic Lyme disease, and demanding extended courses of antibiotic treatment. Several PubMed searches later, the doctor finds no scientific evidence to support prolonged antibiotic use, and refuses to acquiesce (apologies to Captain Barbossa). A vicious pantomime then follows.

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Neurologists tango with Lyme disease in the form of neuroborelliosis. This form of Lyme disease is a nightmare for neurologists because of the varied ways it may present. This was highlighted by a recent excellent review in the American Journal of Neuroradiology titled Lyme Neuroborreliosis: Manifestations of a Rapidly Emerging Zoonosis. These manifestations include a painful lymphocytic meningoradiculitis, cranial nerve palsies, meningoencephalitis, encephalomyelitis, and transverse myelitis. Then there is the nebulous concept of chronic Lyme neuroborreliosis, something the authors say is ‘a focus of ongoing conjecture and controversy‘. They, however, jumped into this minefield and proposed a set of diagnostic criteria which include characteristic symptoms, specific serum antibodies, spinal fluid inflammation, and spinal fluid antibody production. On Utopia, you might add.

By Centers for Disease Control and Prevention (NIH) - Centers for Disease Control and Prevention (NIH), Public Domain, https://commons.wikimedia.org/w/index.php?curid=29608423
By Centers for Disease Control and Prevention (NIH) – Centers for Disease Control and Prevention (NIH), Public Domain, https://commons.wikimedia.org/w/index.php?curid=29608423

To resolve the tricky question, a group of researchers carried out a systematic review of 44 clinical trials that had reported on chronic Lyme neuroborreliosis. They published their findings in the Journal of Neurology under the title Prevalence and spectrum of residual symptoms in Lyme neuroborreliosis after pharmacological treatment: a systematic review. The authors found that, in those studies that were rigorously carried out, there was very little evidence of chronic Lyme disease. They concluded that chronic Lyme disease may just be ‘an artifact of unspecific case definitions in single studies‘.

By Photo Credit:Content Providers(s): CDC - This media comes from the Centers for Disease Control and Prevention's Public Health Image Library (PHIL), with identification number #6631.Note: Not all PHIL images are public domain; be sure to check copyright status and credit authors and content providers.English | Slovenščina | +/−Cropped and uploaded originally to (http://en.wikipedia.org/wiki/Image:Borrelia_image.jpg), Public Domain, https://commons.wikimedia.org/w/index.php?curid=4393667
By Photo Credit:Content Providers(s): CDC – This media comes from the Centers for Disease Control and Prevention‘s Public Health Image Library (PHIL), with identification number #6631.Note: Not all PHIL images are public domain; be sure to check copyright status and credit authors and content providers.English | Slovenščina | +/−Cropped and uploaded originally to (http://en.wikipedia.org/wiki/Image:Borrelia_image.jpg), Public Domain, https://commons.wikimedia.org/w/index.php?curid=4393667

This conclusion is supported by another study in the same journal titled Quality of life, fatigue, depression and cognitive impairment in Lyme neuroborreliosis. This study discovered that patients who were adequately treated for Lyme neuroborreliosis hardly ever developed persisting symptoms.

By Childe Hassam - http://www.the-athenaeum.org/art/full.php?ID=19897, Public Domain, https://commons.wikimedia.org/w/index.php?curid=10199778
By Childe Hassam – http://www.the-athenaeum.org/art/full.php?ID=19897, Public Domain, https://commons.wikimedia.org/w/index.php?curid=10199778

I guess this will not be the end of the story with chronic Lyme disease, but research is shedding light on a very controversial subject. Nirvana soon?

Whilst on the subject, you may want to check out these articles that open up another potential can of worms, intracranial hypertension in Lyme neuroborreliosis.