THE FACES AND MEN
BEHIND THE SYNDROMES

Andy Dzenitis, M.D., FACS

10 March 1998

 
This presentation is devoted to the men whom we have come to accept in our medical lexicon: Rombergs sign, Erbs palsy, Downs syndrome Huntingtons & Sydenham's chorea, Horners sign, Gulliane-Barre disease, eponyms we readily recognize.

Eponyms are useful not only for brevity, but also in recognizing the original contributor.  In other words, identifying disease by name rather than describing an array of signs and symptoms.

It was a challenge to decide whom, and in what order to present these names of people who, through their curiosity, analysis and study, have left us with a lasting legacy.

 Also you will note that this paper is confined to the central nervous system, a subject near and dear to myself.

The CNS, expressly the brain, is the crown jewel of creation in evolution.  It weighs 3 pounds, a mere 2% of our body weight, yet it requires 20% of the blood that flows from the heart to nourish it.

Contrary to the sentiments of song and literature, the seat of humanity is not the heart but the brain.  It controls empires, develops works of artistic beauty, directs a complex ebb and flow of hormones, recalls memories, exercises imagination, understands language, sometimes directs unspeakable cruelties yet at other times shows compassion and love.

JOSEPH FRANCOIS FELIX BABINSKI

A thorough and methodical clinician, he was Parisian, born of Polish parents.  In 1896, in a series of short publications, he described one of the most significant signs familiar to all clinicians and students of neurology, the pathological cutaneous plantar reflex (fanning of the toes and upgoing big toe), indicating dysfunction in the main descending or motor pathway in the spinal cord, known as the Babinski sign.

Remak, a German clinician had mentioned this finding 3 years earlier (1893), but did not correlate its’ significance or relationship to the pyramidal tract or the upper neuron dysfunction.   Numerous other pathological responses characterized by upgoing toes have been described by Chaddock, Oppenheim, and Gordon, and are generally referred to as Babinski's confirmatory signs.

JOHN HUGHLINGS JACKSON

Considered by many as the father of British Neurology by his interest and observations in focal motor epilepsy (1861). 

     John Hughlings Jackson — a peculiar, aloof man, son of a farmer, was largely self-taught, obviously a keen scholar with intense powers of observation.  He inferred that muscle movement is represented by specific areas of the brain surface (well before Fritch-Hitzig) and reasoned, correctly, that seizure occurred from sudden discharges of abnormal or unstable grey matter (cortex) in the vicinity of the brain supplied by the middle cerebral artery.

As fate would have it, his wife was afflicted with the form of epilepsy that until this day bears Jackson’s name.

Other observers, Richard Bright and Bentley Todd, looked at these episodes as spino-medullary phenomena.

CHARLES BELL

Edinborough educated surgeon, anatomist, and gifted artist, early on, was fascinated by movements of face and changes in expression (1806).  He was a prolific writer and illustrator in textbooks and atlases in surgery and anatomy.  He also served the Crown as a military surgeon and was in the field at the battle of Waterloo.

Charles Bell is mostly known for his description of peripheral facial paralysis, Bell's Palsy, which also facilitated his observation of upward movement of the pupil upon closing the eyelid that one is unable to do with facial palsy, Bell's Phenomenon.  The long thoracic nerve is also named the Nerve of Bell - which he referred to as external respiratory nerve.

Like other scientist of the day, he recognized nine pairs of cranial nerves, but separated the seventh from the eighth cranial nerve.  Charles Bell believed that spinal nerves served a double function - both motor and sensory.

CHARLES EDWARD BROWN-SEQUARD

The syndrome of Brown-Sequard, represents a major turning point in neuroanatomy.

Through his studies in 1846, he presented evidence of sensory pathway decussation or crossing in the spinal cord, then upsetting Bell’s doctrine that proposed that sensory impulses ascend on the ipsilateral side of the cord via the dorsal columns.

     Brown-Sequard experimented on animals in which he cut half of the cord - pain sensation was lost on the opposite side below the cut.  The motor paralysis, on the same side, was also observed but more difficult to observe because of spastic reflex gait in quadripeds.

     Brown-Sequard progeny of a French woman and an American sea captain did not receive recognition for his work until 1855, when Broca threw in with him.

Parenthetically "Gowers tract" is named for the English neurologist responsible for proposing that different sensory impulses ascend in a different part of the cord—i.e. posterior columns for position and touch vs. antero-lateral for pain and temperature.

PETER PAUL BROCA

In the Edwin Smith Surgical Papyrus, dating excavations to 17th century B.C., an association was noted between temporal skull trauma and loss of speech.

However, before the observations and study of P. P. Broca, the brain was thought to function as a whole instead of a focal or topical (though integrated) organ.

In 1861, he presented two cases of expressive aphasia in which lesions of 2nd & 3rd frontal gyri were present.

Celebrated case of M. Labonte.  The patient seemed to understand, but his expression was confined to single monosyllable "tan".

A Renaissance man, Broca was also interested in the science of anthropology and considered kidney and pancreas as hormone secreting endocrine organs.

CARL WERNEKE

     A neurophysiatrist, 10 years later took the concept of comprehension and communication further.  (Note - "communication" rather than "speech" - a mute can suffer such a loss in communication with a dominant hemisphere dysfunction).

     In a monograph 1874, using diagrams, he described five clinical syndromes of speech impairment ranging from perception to context of expression – hence - Werneke's dysphasia: "I don't know. Yes, the Bick.  I would say that the mick daysis nosis or chipickters.  Course, I have also missed on the carfter teck."  (I.E. word salad).  Typically the lesion is supposed to be in the posterior superior temporal region, projecting to other areas of the brain.  He did not stress or elaborate left or right brain.

     Although incomplete by today’s standards — both Broca & Werneke built the framework for our present knowledge of aphasia. 

FRACOIS MAGENDIE

However, it was Francois Magendie who clarified, by his experiments on a litter of puppies, that posterior (dorsal) roots carried sensory impulses, and anterior (ventral) roots carried motor function (1821).

Bell had refused to perform painful experiments on animals —perhaps after his experiences as a battle surgeon at Waterloo.

Magendie's name, of course, is also associated with a foramen in the fourth ventricle in the posterior fossa.

JAMES PARKINSON

The Essay of Shaking Palsy by James Parkinson, condition which since has been named as Parkinson's Disease (published in 1817) was actually coined by Charcot, father of French Neurology, who practiced at Hospital Salpetiere.

Parkinson inherited his practice from his father who ran an apothecary and practiced surgery.  His essay was based on observation of only six cases, of whom only 3 were under his care.  The remaining 2 were only observed on the street.  He never got to study any of their brains, i.e. there was no anatomical correlation.

He wrote eloquently though:

“…The disease, respecting which the present inquiry is made, is of a nature highly afflictive.  Notwithstanding which, it has not yet obtained a place in the classification of nosologists; some have regarded its characteristic symptoms as distinct and different diseases, and others have given its name to disease differing essentially from it; whilst the unhappy sufferer has considered it as an evil, from the domination of which he had no prospect of escape."

Parkinson was also a contributor in palentology, geology, and a prominent voice in political reform under the name of Old Hubert.

It was Meinert, a German neuroscientist, who pointed towards the basilar ganglia as a source of dysfunction in Parkinson's Disease (1871).

Of course it has been in our generation that dopamine, a neurotransmitter, is a factor in Parkinson’s Disease.

FRITCH & HITZIG

Although their names are not attached to specific anatomical structures or syndromes, Gustav Teodor Fritch and Eduard Hitzig's contributions in topical, cortical mapping stand out.

We are all familiar with the picture of the grotesque homonculus superimposed on the brain surface.

During the Prussian-Danish war, 1864, Herr Doctor Fritch observed that dressing head wounds of injured soldiers produced twitching in the opposite side of the body when the cortex was touched.  He shared this with Hitzig, a practitioner in Berlin, and together they carried out electrical stimulation of cortex in dogs.  Since they had no lab facility, the studies were often carried out on Frau Hitzig's dressing table.

Despite the crudity of the procedure the results agreed with Fritch’s original observation: Electrical stimulation of the cortical surface of dogs produced contractions in muscles of the opposite side of the body.  Cortical mapping was born.

ALOIS ALSHEIMER

In 1907, a German neuropathologist by his lifelong studies of dementia showed the value of clinical-pathological correlation.  The man was Alois Alsheimer.

In these studies he showed the relationship of intellectual deterioration in patients with specific changes in the brain architecture, such as: cerebral atrophy, senile plaques, and neurofibrillary tangles which look like skeletons of brain cells.

Various theories of causation have been put forth, none have been proven, same goes for treatment - certain drugs have seemed to slow the progression of the disease, and there is some justification for cautious optimism that Alzheimers' Disease may someday be treated or even prevented.