Adolf von Strümpell

Ernst Adolf Gustav Gottfried von Strümpell (1853 - 1925)

Ernst Adolf Gustav Gottfried von Strümpell (1853 – 1925) was a German neurologist, researcher and teacher.

A pioneer of his time, Strümpell described several complex diseases that contributed to the development of the field of neurology, and was anointed a knighthood for his work. He is regarded as one of the co-founders of neuropathology as a scientific subject.

As a teacher and scientist, Strümpell influenced a whole generation of students and doctors. He co-founded the journal Deutsche Zeitschrift für Nervenheilkunde (the predecessor to the Journal of Neurology) alongside Friedrich Schultze, Wilhelm Erb, and Ludwig Lichtheim, which was critical to the emancipation of neurology as an independent subject. Strümpell’s textbook of internal medicine, Lehrbuch der speziellen Pathologie und Therapie der inneren Krankheiten, is regarded to be one of the best of his time and was utilized for decades.

Strümpell is eponymous with Strümpell signs, Strümpell-Lorrain disease, Marie-Strümpell disease and Westphal-Strümpell pseudosclerosis.


Biography
  • Born June 29, 1853 in Neu-Autz, Courland
  • 1875 – Graduated Doctor of Medicine from the University of Leipzig
  • 1876-1882 – Clinical assistant to Carl Wunderlich and Ernst Wagner at Leipzig St. Jacob’s University Hospital
  • 1878 – Privatdozent for internal medicine at the University of Leipzig
  • 1883-1884 – Associate professor and director of the medical clinic at the University of Leipzig
  • 1884-1886 – Director of the medical clinic in Heidelberg, with Paul Möbius as his assistant
  • 1883 – Published Lehrbuch der speziellen Pathologie und Therapie der inneren Krankheiten
  • 1886-1903 – Professor and director of the medical clinic at the University of Erlangen
  • 1891 – Founded the Deutsche Zeitschrift für Nervenheilkunde with Friedrich Schultze, Wilhelm Erb and Ludwig Lichtheim
  • 1893 – Awarded the Knight’s Cross of the Order of Merit of the Kingdom of Bavaria for his scientific merits
  • 1903-1909 – Professor and director of the medical clinic at the University of Breslau
  • 1909 – Head of the 3rd Medical Clinic in Vienna
  • 1910-1925 – Professor for Special Pathology and Therapy and director of the medical clinic at the University of Leipzig
  • Died January 10, 1925 in Leipzig, secondary to pneumonia.

Medical Eponyms
Strümpell Signs (1899)

A collection of clinical signs found in patients with pyramidal tract lesions. Detailed in Strümpell’s publication, Zur Kenntnis der Haut- und Sehnenreflexe bei Nervenkranken.

The most renowned sign refers to an extensor plantar reflex that occurs with flexion at the hip against resistance.

So kann man durch leises vorsichtiges durch leises vorsichtiges Streichen am medialen Fussrande nicht selten eine isolirte schwache Zuckung im M. extensor
hallicus longus (Dorsalflexion der grossen Zehe) bewirken. Bei stärkerer Reizung tritt eine Dorsalflexion des Fusses hinzu und dann meist bald der gesammte Beugereflex (Verkürzungsreflex) der unteren Extremität. Derselbe besteht dann in einer anscheinend gleichzeitig eintretenden Dorsalfiexion des Fusses und der Zehen, in einer Beugung des Unterschenkels und Beugung des Oberschenkels. Manchmal vereinigt sich hiermit auch noch eine Adduction und Einwärtsrollung oder auch eine Auswärtsrollung des Oberschenkels.

Strümpell 1899; Zur Kenntnis der Haut- und Sehnenreflexe bei Nervenkranken, p. 260.

Thus, by gently stroking the medial edge of the foot, it is not uncommon to find an isolated, weak twitch in the extensor muscle hallicus longus (dorsiflexion of the big toe). If the irritation is more severe, dorsiflexion of the foot occurs and then usually soon the entire flexion reflex (shortening reflex) of the lower extremity. The same thing then apparently exists where dorsiflexion of the foot and toes occur, with flexion of the lower leg and flexion of the thigh. Sometimes this is also combined with an adduction and an inward roll or an outward roll of the thigh.

Strümpell; Zur Kenntnis der Haut- und Sehnenreflexe bei Nervenkranken, 1889: 260.

Other signs include:

  • Forced (involuntary) dorsal flexion of the hand with volar flexion of the finger occurring in spastic syndrome.
  • Forced (involuntary) pronation of the hand when the lower arm is flexed. 

Strumpell-Lorrain Disease (1880)

Also known as Hereditary spastic paraplegia.

A group of inherited diseases characterized by progressive spasticity and weakness secondary to length-dependent axonal degeneration of the corticospinal tract upper motor neurons. The lower extremities are more commonly affected due to the associated nerve fibres being longer.

Although Otto Adolph Seeligmüller is commonly credited for being the first-ever to describe this disease in the early 1870s, a closer analysis reveals that what he described was not spastic paraplegia but rather muscular atrophy and bulbar palsy. Wilhelm Heinrich Erb’s description in 1875 is therefore considered to be the earliest description of hereditary spastic paraplegia.

Strümpell published what is considered to be the first comprehensive description of Strümpell-Lorrain disease in 1880, where he reported a family in which two brothers were affected by spastic paraplegia. The father was said to be “a little paralyzed”, suggesting autosomal dominant transmission. Strümpell, as well as Maurice Lorrain, then added similar cases to the literature in subsequent years.

Gaum I., Brunnenbauer, 58 Jahr alt. Ueber die Eltern niehts Genaues zu erfragen. DerVater soll auch ,,ein weniggelähmt” gewesen sein. Pat. selbst, abgesehen von mehreren acuten Krankheiten bis 1876 gesund. Seitdem Sohwere in den Beinen beim Gehen und zuweilen Zittern in denselben. Niemals Schmerzen in denselben. Keine Blasen- und Stuhlbesohwerden. Patient ist verheirathet, hat 29 Kinder!… Er kann längere Strecken ohne zu ermüden gehen, muss aber immer rasch gehen, weil langsames Gehen ihn welt mehr ermüdet… Kräftig gebauter, muskulöser Mann. Schädelbildung normal, Kopfumfang 56.5 Ctm. Intelligenz nicht besonders entwickelt. Sprache leicht seandirend, aber vollständig verständlich. Im Gesicht der breite vortretende Unterkiefer auffallend. Der rechto untere Facialis wird gewöhnlich ein wenig sehwächer innervirt, als der linke…Zuweilen deutlicher leichter Tremor des Kopfes. Bei starkem Oeffnen des Mundes Tremor des Unterkiefers. In den oberon Extremitäten keine Spur von Lähmung. Rohe Kraft beiderseits sehr bodeutend.

Bei allen activen Bewegungen der Arme aber ein leichter Tremor in denselben zu bemerken. Sehnen-und Periostreflexe an beiden Armen sehr erhöht. Im Biceps, Triceps, am Vorderarm etc. sind eine grosse Zahl ziemlich seltener Reflexe auszulösen. Die Sensibilität ganz intact. Beide untere Extremitäten muskulös, fühlon sich rigide an. Rohe Kraft in allen Muskeln bedeutend. Passive Bewegungen durch den sofort eintretenden reflectorischen Muskelwiderstand erschwert. Der Gang ist in hohem Grade auffallend.

Patient geht stets mit ziemlieh raschen, grossen Schritten. Dabei kleben die Fussspitzen am Boden. Bet jedem Schritt schnellt fast der ganze Körper durch die reflectorische Contraction des Gastrocnemius ein wenig in die Höhe. Die Kniee werden beim Gehen nut sehr wenig gebeugt. Langsames Gehen macht dem Patienten viel mehr Mühe, als rasches Gehen, weil er dutch die reflectorische Hebung des einen Beines stets genöthigt ist, das andere Bein möglichst rasch vorzuschieben. Sensibilität vollkommen intact. Harn ohne Besehwerden entleert und von normaler Beschaffenheit.

Strümpell; Beiträge zur Pathologie des Rückenmarks, 1880: 711.

Gaum I., well builder, 58 years old. Little detail regarding parents. The father is also said to have been “a little paralyzed”. The patient himself, apart from several acute illnesses, was healthy until 1876. Since then, his legs have become so heavy when walking and sometimes have tremors in them. Nil pain. Nil bladder or stool problems. The patient is married, and has 29 children!… He can walk longer distances without getting tired, but always has to walk quickly because walking slowly tires him more… Strongly built, muscular man, normal skull formation, head circumference 56.5 cm. Intelligence is not particularly developed. Speech is slightly distracting but completely understandable. The broad, protruding lower jaw is conspicuous on the face. The lower right facial nerve is usually a little less visually innervated than the left … Sometimes a distinct, slight tremor of the head is present. Strong opening of the mouth, tremor of the lower jaw, no trace of paralysis in the upper extremities, raw strength on both sides very groundbreaking.

In all active movements of the arms, however, a slight tremor can be noticed in them. Tendon and periosteal reflexes on both arms are increased. In the biceps, triceps, forearm, etc., a large number of rather rare reflexes can be triggered. The sensitivity is quite intact. Both lower extremities are muscular and rigid to the touch. Significant raw strength in all muscles. Passive movements made more difficult by the immediately occurring reflex muscle resistance. The gear is highly striking.

The patient always walks with fairly quick, large steps. The tips of his feet stick to the ground. With every step, almost the whole body shoots up a little due to the reflex contraction of the gastrocnemius. The knees are only slightly bent when walking. Walking slowly causes much more trouble for the patient than walking quickly, because the reflex lifting of one leg always forces him to push the other leg forward as quickly as possible. Sensitivity is completely intact. Urine has evacuated without discomfort and is normal.

Strümpell; Beiträge zur Pathologie des Rückenmarks, 1880: 711.

Gaum II., Bruder des vorigen, gleichfalls Brunnenbauer, 56 Jahr alt. Ist in der Jugend ganz gesund gewesen, bis auf alle ¼ – ½ Jahr eintretende epileptiforme Krämfe, welche auch in den letzten Jahren noch mehrmals wiedergekehrt sind. Im Jahre 1859 ist er beim Arbeiten in einen Brunnen gestürzt, ohne abet irgend eine schwerere Verletzung davonzutragen. Er hat auch gleich nach dem Sturze weiter gearbeitet. Kurze Zeit darauf abet soll sein Gang sich verändert haben, worauf ihn zuerst seine Frau aufmerksam gemacht hat. Seitdem, also seit 19 Jahren, ist der Gang absolut derselbe geblieben. Patient hat seine Arbeit dabei stets verrichtet. In der letzten Zeit erst hat er öfter über rheumatische Schmerzen in den Beinen zu klagen. Ansserdem zuweilen Magenbeschwerden. Sonst aber ganz wohl und
kräftig. Hat noch vor kurzer Zeit einen Weg von 4 Stunden machen können.

Wohlaussehender Mann. Schädel etwas scheitelsteil, sonst symmetrisch gebaut. Im Gesicht keine Lähmungen. Obere Extremitäten kräftig und normal beweglich, zeigen aber eine deutliche Erhöhung der Sehnenreflexe. In den untern Extremitäten alle Bewegungen mit Kraft ausführbar, nur steif und zuweilen etwas verlangsamt durch eintretende Muskelcontractionen. Ebenso bei passiven Bewegungen Muskelwiderstand zu überwinden. Sehnenreflexe an beiden Beinen äusserst hochgradig. Sensibilität vollständig normal, ebenso die Harn- und Stuhlentleerung. Potenz erhalten. Der Gang ist dem seines Bruders durchaus ähnlich, exquisit spastisch, auf den Spitzen, dabei die einzelnen Schritte ziemlich gross und rasch auf einander folgend. Der ganze Körper dabei etwas vornüber fallend.

Strümpell; Beiträge zur Pathologie des Rückenmarks, 1880: 711.

Gaum II, brother of the previous one, also well builder, 56 years old. Was quite healthy in his youth, with epileptiform cramps occurring every ¼ – ½ year, which have returned several times in recent years. In 1859, while working, he fell into a well, without suffering any serious injury. He continued to work immediately after the fall. A short time afterwards, his gait is said to have changed, which his wife first made him aware of. Since then, for 19 years, the course has remained absolutely the same. The patient has always done his job. Recently he has been complaining of rheumatic pains in his legs. In addition, sometimes stomach problems. Otherwise quite well and strong. Was able to walk 4 hours a short time ago.

Well-looking man. Skull slightly apex, otherwise symmetrical. No paralysis in the face. Upper extremities strong and normally mobile, but show a significant increase in tendon reflexes. In the lower extremities all movements can be carried out with force, only stiff and at times somewhat slowed down by muscle contractions. It is also possible to overcome muscle resistance during passive movements. Tendon reflexes on both legs extremely exaggerated. Sensitivity completely normal, as well as urination and defecation. Has potency. The gait is quite similar to that of his brother, exquisitely spastic, on the tips, the individual steps being rather large and quickly following one another. The whole body is falling forward a little.

Strümpell; Beiträge zur Pathologie des Rückenmarks, 1880: 711.

Strümpell credited Jean-Martin Charcot as the first to describe the disease in 1869, however Charcot had actually described a case of amyotrophic lateral sclerosis.


Marie-Strümpell Disease (1884)

Commonly known as Ankylosing Spondylitis. Also known as Bekhterev disease and Bechterew’s disease.

A rheumatoid disease characterized by chronic inflammation of the spine. Formation of syndesmophytes and ossification of the intervertebral discs may also occur, resulting in fusion and the characteristic “bamboo spine”.

Whilst the first comprehensive description of ankylosing spondylitis is commonly deemed to be by Vladimir Bekhterev in 1893, descriptions of the condition date back as early the 2nd century AD by Galen.

Indeed, Strümpell briefly described the disease in his 1884 version of Lehrbuch der speziellen Pathologie und Therapie der inneren Krankheiten, where he observed progressive stiffening and fusion of the spinal joints.

Als ein merkwürdiges und, wie uns scheint, eigenartiges Leiden mag hier beiläufig noch diejenige Erkrankungsform erwähnt werden, bei welcher es ganz allmählich und ohne Schmerzen zu einer vollständigen Ankylose der ganzen Wirbelsäule und der Hüftgelenke kommt, so dass Kopf, Rumpf and Oberschenkel fest mit einander verbunden und vollkommen steif sind, während alle übrigen Gelenke ibre normale Bewegliehkeit behalten. Dass hierdurch ganz eigenthümliche Modificationen der Körperhaltung und des Ganges entstehen müssen, liegt auf der Hand. Wir selbst haben zwei ganz gleiehartige Fälle dieser eigenthümlichen Krankheit gesehen.

Strümpell; Lehrbuch der speziellen Pathologie und Therapie der inneren Krankheiten, 1884; II(2): 152.

As a remarkable and, as it seems to us, peculiar ailment, that form of illness may be mentioned in passing, in which there is a complete ankylosis of the whole spine and the hip joints very gradually and without pain, so that the head, trunk and thighs are firmly together connected and perfectly rigid while all remaining joints retain their normal mobility. It is obvious that this results in very peculiar modifications of posture and gait. We ourselves have seen two very similar cases of this peculiar disease.

Strümpell; Lehrbuch der speziellen Pathologie und Therapie der inneren Krankheiten, 1884; II(2): 152.

After Bekhterev’s publication, Strümpell published a reply in 1897 detailing another case study of a patient with the disease in more detail. Bekhterev, Strümpell, and Pierre Marie’s work on the disease permitted an accurate diagnosis of ankylosing spondylitis prior to its manifestation as a severe spinal deformity.


Westphal-Strümpell Pseudosclerosis (1897)

Refers to the neurological symptoms seen in Wilson’s disease; a genetic disorder characterized by excess copper storage in various body tissues.

Wilson’s disease was initially known as Westphal-Strümpell pseudosclerosis for a number of years before Alexander Kinnier Wilson published the pathoanatomical basis of the disease.

Carl Westphal (1833 – 1890) first described patients in 1883 who showed cerebellar symptoms similar to those seen in multiple sclerosis but lacked the pathological brain changes.

Strümpell presented a larger study in 1897 discussing the findings of Westphal and other authors, alongside cases seen himself. He identified characteristic features of the disease, such as the early occurrence during adolescence, the extrapyramidal syndromes, and the distinctive mental features, however, could not attribute a cause partially due to limiting his investigations to the central nervous system and not looking at the liver.

Fasse ich das Ergebniss aller unserer bisherigen, freilich leider noch äusserst lückenhaften Beobachtungen über die fraglichen Krankheitszustände zusammen, so lautet es etwa folgendermaassen:

Es giebt ein Krankheitsbild, welches in allen seinen Einzelheiten und in seinem chronischen Gesammtverlauf demjenigen der multiplen Sklerose in hohem Grade ähnlich ist, dessen pathologisch anatomische Grundlage aber bisher nicht aufgefunden werden konnte. Vielmehr waren die bisherigen anatomischen Nachforschungen häufig fast ganz ohne Resultat, so dass es den Anschein haben kann, als ob dieser Krankheit überhaupt keine gröbere anatomische Veränderung zu Grunde liegt. Letztere Ansicht ist aber keineswegs sicher erwiesen, da es sich sehr wohl um Veränderungen handeln kann, welche sich aus den oben näher angeführten Gründen bisher nur dem Nachweise entzogen haben. Es empfiehlt sich, dieses von Westphal zuerst, hervorgehobene Krankheitsbild vorläufig als Pseudosklerose zu bezeichnen.

Strumpell 1897; Ueber die Westphal’sche Pseudosklerose und über diffuse Hirnsklerose, insbesondere bei Kindern, p. 149.

If I summarize the results of all of our previous, unfortunately still extremely incomplete, observations on the disease states in question, it goes something like this:

There is a clinical picture which, in all its details and in its overall chronic course, is very similar to that of multiple sclerosis, the pathological anatomical basis of which, however, has not yet been found. Rather, the previous anatomical investigations were often almost completely without result, so that it can appear that this disease is not based on any major anatomical change. The latter view, however, has by no means been proven with certainty, since it may very well be a matter of changes which, for the reasons detailed above, have so far only eluded the evidence. It is advisable to designate this clinical picture, which Westphal first emphasized, provisionally as pseudosclerosis.

Strumpell 1897; Ueber die Westphal’sche Pseudosklerose und über diffuse Hirnsklerose, insbesondere bei Kindern, p. 149.

Interestingly, Kayser-Fleischer rings were not detected at the time, and it was only years after their discovery did Strümpell explain that the rings “can be easily overlooked, if one is not on the lookout for it”.


Key Medical Attributions
Lehrbuch der speziellen Pathologie und Therapie der inneren Krankheiten

Strümpell’s textbook, written almost entirely on the basis of personal observation, is widely regarded to be the definitive textbook of internal medicine in Germany of the time, with over 32 editions and translations into eight languages (including English) occurring until the 1930s.  The sub-volume on neurological diseases was especially renowned due to its comprehensive overview of neurological illnesses and was a guiding manual for the training in neurology for decades.

The field of Neuropsychiatry

Alongside neurology, Strümpell also contributed to the developing field of psychiatry. He worked with Mobius to develop theories and conceptions on psychoneuroses and the understanding of hysteria, and published texts on the perception of time and pain. Strümpell was also an advocate of affiliating neurology with psychiatry, which differed from the opinions of some of his contemporaries.


Controversies

During his time in Vienna, Strümpell was critical of Vienna’s society and culture, as well as the theories of Vienna’s prominent neuropsychiatrist Sigmund Freud. Strümpell’s lengthy negative review of Freud’s and Josef Breuer’s “Studies on Hysteria”, resulted in a strong scientific dispute.  

Freud was similarly critical of Strümpell’s work, quoting his name directly in his work ‘On the Mental Mechanism of Hysteric Phenomena’, where he called out Strümpell’s publications on the topic of hysteria for not being original.

‘‘One can hardly deny the fact that the erotic-sexual factor plays a significant role in the equation of life in Vienna and that certain Far-Eastern influences are manifest. Hence it is little wonder that it was from Vienna that a whole movement in medicine began to spread; one that displayed eloquence and fine spirit yet excessively biased exaggeration in its attempt to introduce sexual aspects in the therapy of nervous diseases. The scientific damage caused by these theories, which at least offered some undoubtedly new stimuli, is much less than the often most pitiable practical devastations the so-called psychoanalytic treatment caused in many of its poor victims.’’

Strümpell’s opinion on Vienna and the theories of Sigmund Freud. Translated from his autobiography Aus Dem Leben Eines Deutschen Klinikers: Erinnerungen und Beobachtungen.

Major Publications

References

Biography

Strümpell-Lorrain disease

Marie-Strümpell disease

Westphal-Strümpell pseudosclerosis


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Lewis is a fourth-year medical student at UWA. He is currently interested in critical care medicine

Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and informatics. Asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | vocortex |

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