Medicine is as much defined by diseases as by the people who named them. Neurology particularly has a proud history of eponymous disorders which I discussed in my other neurology blog, Neurochecklists Updates, with the title 45 neurological disorders with unusual EPONYMS in neurochecklists. In many cases, it is a no brainer that Benjamin Duchenne described Duchenne muscular dystrophy, Charle’s Bell is linked to Bell’s palsy, Guido Werdnig and Johann Hoffmann have Werdnig-Hoffmann disease named after them. Similarly, Sergei Korsakoff described Korsakoff’s psychosis, Adolf Wellenberg defined Wellenberg’s syndrome, and it is Augusta Dejerine Klumpke who discerned Klumpke’s paralysis. The same applies to neurological clinical signs, with Moritz Romberg and Romberg’s sign, Henreich Rinne and Rinne’s test, Joseph Babinski and Babinski sign, and Joseph Brudzinski with Brudzinki’s sign.
Yes, it could become rather tiresome. But not when it comes to diseases which, for some reason, never had any names attached to them. Whilst we can celebrate Huntington, Alzheimer, Parkinson, and Friedreich, who defined narcolepsy and delirium tremens? This blog is therefore a chance to celebrate the lesser known history of neurology, and to inject some fairness into the name game. Here then are 25 non-eponymous neurological diseases and the people who discovered, fully described, or named them.
By A. F. Dressler – Festschrift zum 70. Geburtstag Dr. Woldemar Kernig’s: Von Verehrern und Schülern herausgegeben als Festnummer der St. Petersburger medicinischen Wochenschrift St. Petersburger medizinische Wochenschrift, Bd. 35, Nr. 45. (1910), Public Domain, Link
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.
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.
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.
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
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
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 horseriding, below is an alternative image to soothe your hurt feelings.
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.
A 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!
Neurology is a broad specialty covering a staggering variety of diseases. Some neurological disorders are vanishingly rare, but many are household names, or at least vaguely familiar to most people. These are the diseases which define neurology. Here, in alphabetical order, is my list of the top 60 iconic neurological diseases, with links to previous blog posts where available.
The Neurology Lounge has a way to go to address all these diseases, but they are all fully covered in neurochecklists. In a future post, I will look at the rare end of the neurological spectrum and list the 75 strangest and most exotic neurological disorders.
This is a follow-up to my previous blog post, So what is remarkable about neurology anyway? That post reviewed the challenging tasks neurologists face everyday. How do they go about it? How do they evaluate their patients with suspected neurological disorders?
For the uninitiated, the process of the neurological assessment must seem like an outlandish ritual. Unlike cardiologists who approach patients with the familiar stethoscope, neurologists come armed to the hilt with an arsenal of threatening equipment. Patients are often bewildered, and occasionally irritated, with the neurological exam. Admitted, they sometimes, they sometimes emerge from the assessment feeling battered and bruised-all for a good cause of course!
So what are these bizarre deeds that marks the neurological consultation?
1. Neurologists welcome you with an overly firm handshake
The handshake is a valuable neurological tool. It tells the neurologist right from the beginning if there is any weakness or if there is a form of muscle stiffness called myotonia. Therefore avoid the neurologist’s handshake if you suffer with arthritis or other painful hand conditions.
2. Neurologists make you do the catwalk
The way you walk, the gait, may show the neurologist a variety of clues or signs. There are a variety of abnormal gaits that often point to a diagnosis even before the consultation actually begins. Examples include the shuffling gait in Parkinson’s disease, the hemiparetic gait in Stroke, and the waddling gait in diseases that give rise to hip girdle weakness. More embarrassing for some patients is that the neurologist may actually ask them to do a catwalk, all for the sake of making a diagnosis you must understand!
Other bizarre associated tests are walking an imaginary tightrope, standing on one leg, standing on tip toes and then on the heels, and marching in one spot with eyes shut
The face often give the neurologist the clue to many diagnoses. Conditions such as Bell’s palsy and Stroke are evident from the face as are Parkinson’s disease, myotonic dystrophy and facio-scapulo-humeral muscular dystrophy (FSHD). There’s no need to blush therefore when the intent gaze seems to go on endlessly.
4. Neurologists come up very close- to peer into your soul
If the eyes are the windows to the soul, then neurologists are second only to ophthalmologists in recognising this nebulous entity. The back of the eye, or retina, holds a variety of valuable clues for many neurological diseases. The neurologist typically looks for signs of increased pressure in the head and this may occur with brain tumours, meningitis, encephalitis, This may also occur without any obvious cause in a condition called idiopathic intracranial hypertension (IIH). Other eye signssuch as cataracts and pigmented retina seen with disorders for example mitochondrial diseases.
To peer into the soul, the neurologist may come very uncomfortably close, (hoping the aftershave isn’t too strong and that the morning deodorant has lasted till then). Don’t hold your breath however, as this gazing into the soul may take longer than you anticipate.
5. Neurologists ask you to roll your eyes-in all sorts of directions
Abnormal eye movements are key pointers to many neurological disorders. There are six muscles that move each eyeball, and these are under the control of three pairs of cranial nerves-the oculomotor, the trochlear, and the abducens nerves. These nerves in turn are coordinated by complex nerve cell bodies or nuclei in the brain stem.The eyelids and pupils are also muscles under control of nerves.
These cranial nuclei coordinate a symphony of unparalleled and unimaginable complexity. This allows us to focus on moving objects without any hinderance. Things may go wrong with this symphony, and this typically results in double vision (diplopia) and droopy eyelids (ptosis). Diseases that cause these symptoms include brain aneurysms,myasthenia gravis (MG), and brainstem stroke. Some diseases may cause the eyeballs to move in uncontrollable and chaotic ways called nystagmus, oscillopsia, and opsoclonus(neurologists love these names!)
Don’t be shocked therefore when your neurologist asks you to look up, look down, look to the right and left; to follow this or the other hand; to look at this fist then at these fingers…. It’s all a helpful game-honest!
6. Neurologists ask you to pretend to brush your teeth
Your neurologist may request you to brush your teeth or hair with an imaginary brush, or ask you to do victory sign or the thumbs-up sign (never thumbs-down mind you). Almost verging on the comedic, this is a serious test because these simple tasks are impaired in many diseases. The difficulty in performing tasks one has previously been proficient at is called dyspraxia, or apraxia if the ability is completely lost. Without any weakness or numbness, people with dyspraxia are unable to use common tools and equipment, reporting that they have no idea how to manipulate them. This could be seen in some forms of stroke and some dementias. Do decline however if she asks you to mimic the great mime Marcel Marceau.
7. Neurologists ask you to wiggle your tongue and poke it out
The tongue is a very important muscle and holds countless clues for the neurologist. It is innervated by the last of the 12 cranial nerves, the hypoglossal nerve. which may be paralysed by a very localised stroke and this is often in the context of a condition called cervical artery dissection. This is a tear in one of the big arteries in the neck which take blood to the brain. The tear may arise from trivial neck movements and manipulations such as look up for a long time or staying too long on the hairdressers couch. A clot then forms at the site of the tear, and this then migrates to block a smaller blood vessel supplying the brainstem where the hypoglossal nerve sets off from…phew! Anyway, when this kind of stroke occurs, the tongue deviates to the the weaker side when it is poked out.
The more general weakness of the tongue is seen in conditions such as motor neurone disease (MND),in which the tongue also quivers at rest-something neurologists call fasciculations. The cheeky neurologist (pun intended) will ask you to push against her finger through your cheek to test its full strength.
Another problem that may affect the tongue is myotonia, a condition in which he tongue and other muscles are stiff and relax very slowly after they are activated. To test this, your neurologist may actually tap on your tongue, and then watches in fascination as it stiffens and then relaxes very slowly. Strong but slow moving tongues may be seen in Parkinson’s disease (PD). So, when next your neurologist says ‘open up’, he really means business.
OK, she will not literally wrestle you to the ground but it may appear so at times. Pushing against your head, pressing down against your elbows, leaning hard against your leg-she will do everything to show she is stronger than you. Only if she fails will she score your power as grade 5/5-the best you can get. If you do not score full marks however you place the neurologist in a bit of a quagmire; a score between 0-5 is not always easy to allocate, and the obsessive neurologist may get in a bind and may give you marks such as 3+ or 4-. Just for fun let her win, and see her consternation!
9. Neurologists hit you with a hammer-in all sorts of places
The reflex hammer is perhaps the most well-recognised tool of the neurologist. These hammers come in all shapes and sizes, and some are really quite scary. People expect to have their knees tapped and look forward to what they have seen many times on TV-the leg kicking out. Most patients find this amusing. They are however often surprised when the neurologist proceeds to use the hammer on their jaw, elbow, wrist and ankles. The then often bristle at having the soles of their feet stroked by the end of the hammer’s handle, a sharp uncomfortable end it is. All the hammer does is to stretch the tendons of muscles, and this elicits a reflex that causes the muscle to contract or tighten up. This response may be exaggerated (hypereflexia) if there is any problem in the central nervous system. Conversely the reflex response may be diminished (hyporeflexia) with problems of the peripheral nervous system. Stroking the foot is called the Babinski response and gives a similar form of information to the neurologist. But beware the neurologist who then proceeds to stroke the side of your foot or squeeze your shins, all in an effort to get the same information-it is really an unnecessary and uncomfortable duplication of tests.
10. Neurologists prick and prod you with a sharp pin
Now this must take the cake, and quite rightly often comes at the end of the neurological examination. As threatening as this tests appears, this is probably the neurologist at his most acute. Using a sterile pin, the neurologist asks you to respond ‘yes’ if the sensation you perceive is sharp, and ‘no’ if it is dull. He then carefully proceeds to map out areas of reduced sensation or feeling, frowning as he struggles to keep track of your responses in his mind. He tries to establish if you have a glove and stocking pattern of sensory loss seen in peripheral neuropathy (nerve end damage). It may also be a dermatomal pattern seen with radiculopathy (trapped nerve in the spine). Unfortunately for the neurologist however many patients do not understand the rules of the game and give all sorts of unimaginable responses; not surprising when one is under the threat of a sharp pointy object!
These are but a few of the bizarre doings of neurologists. Seeing a neurologist soon? Be prepared-you have been warned!
PS. Images used in this blog post are for illustration purposes only and do not necessary depict the actual equipment used by neurologists. The examination steps described are however a good reflection of actual neurological practice.
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