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Introduction: Ulnar neuropathy at the elbow (UNE) is the second most common entrapment neuropathy. Our goal was to create and analyze a grading system for UNE electrodiagnostic severity. Methods: We retrospectively analyzed EMG reports with UNE. We then classified 112 limbs as having mild, moderate, or severe grade UNE based on electrodiagnostic findings. The association between presenting symptoms and signs, EMG findings, treatment type, and electrodiagnostic grade was statistically analyzed. Results: Seventeen limbs (15.2%) had mild, 80 (71.4%) had moderate, and 15 (13.4%) had severe UNE. Symptoms (P =.016), exam findings (P <.001), and treatment type (P =.043) were significantly associated with electrodiagnostic grade. Discussion Our UNE grading system was significantly related to symptoms, physical exam, and treatment selection and may be useful to measure electrodiagnostic severity. [ABSTRACT FROM AUTHOR]

At least 9 neuroscientists immigrated from Nazi Europe to Illinois to escape tyranny and attempt to re-establish their careers. Some work has been published in print on eponymous neuroscientist Adolf Wallenberg, as well as 2 others but not on Ernst Haase, Frederick Hiller, Erich Liebert, Bruno Volk, Heinz (Henry) von Witzleben, or Gerhard Pisk. Before leaving Germany or Austria, these downtrodden specialists were dismissed from long-held posts sometimes for trumped-up charges, stripped of their financial security, and forced to leave relatives behind. At least 1 left only for personal and political, but not because of racial, reasons. Illinois, in exemplary fashion, welcomed these unfortunate survivors more than many other states because of limited licensing requirements, numerous opportunities at state hospitals, and special internship programs. Some of them successfully continued their research agendas and published, taught neurology students and trainees, and added to the expansion of neurologic care in Illinois or elsewhere, but most of them took years to reacquire the academic rank they lost and never regained their career momentum. These refugees survived and passed on some of their extensive training and expertise to a new generation of neuroscientists in America, but not without significant cost. [ABSTRACT FROM AUTHOR]

Introduction: Causes of small-fiber peripheral neuropathies (SFN) are often undefined. In this study we investigated associations of serum autoantibodies, immunoglobulin G (IgG) vs fibroblast growth factor receptor-3 (FGFR-3), and immunoglobulin M (IgM) vs trisulfated heparan disaccharide (TS-HDS) in cryptogenic SFN.Methods: One hundred fifty-five patients with biopsy-proven SFN and no identified cause for their neuropathy were blindly tested for serum IgM vs TS-HDS and IgG vs FGFR-3.Results: Forty-eight percent of SFN patients had serum antibodies, 37% with IgM vs TS-HDS and 15% with IgG vs FGFR-3. TS-HDS antibodies were more frequent in SFN patients than in controls (P = .0012). Both antibodies were more common in females, and with non-length-dependent nerve pathology. Nintey-two percent of patients with acute-onset SFN had serum IgM vs TS-HDS.Discussion: Autoantibodies directed against TS-HDS and FGFR-3 suggest an immune disorder in otherwise idiopathic SFN. Serum IgM vs TS-HDS may be a marker for SFN with an acute onset. [ABSTRACT FROM AUTHOR]

A response by Lawrence A. Zeidman and colleagues to a letter to the editor about the article regarding Hans Berger's ties to the Third Reich and the actions of physicians during the Nazi era published in the "Journal of Child Neurology" is presented.

Approximately 9,000 physicians were uprooted for so-called “racial” or “political” reasons by the Nazi regime and 6,000 fled Germany. These refugees are often seen as survivors who contributed to a “brain drain” from Germany. About 432 doctors (all specialties, private and academic) were dismissed from the major German city of Hamburg. Of these, 16 were Hamburg University faculty members dismissed from their government-supported positions for “racial” reasons, and, of these, five were neuroscientists. In a critical analysis, not comprehensively done previously, we will demonstrate that the brain drain did not equal a “brain gain.” The annihilation of these five neuroscientists’ careers under different but similar auspices, their shameful harassment and incarceration, financial expropriation by Nazi ransom techniques, forced migration, and roadblocks once reaching destination countries stalled and set back any hopes of research and quickly continuing once-promising careers. A major continuing challenge is finding ways to repair an open wound and obvious vacuum in the German neuroscience community created by the largely collective persecution of colleagues 80 years ago. [ABSTRACT FROM PUBLISHER]

Austrian neuroscientist Josef Gerstmann, well known for describing Gerstmann syndrome and for pioneering works on tactile agnosia, also co-described the familial prion disorder later known as Gerstmann-Sträussler-Scheinker disease. In 1938, Nazi Germany annexed Austria (the “Anschluss”) and the three-time decorated war veteran Gerstmann was dismissed from his professorship in Vienna because of his “race.” In 1942, he unknowingly had his doctorate stripped, only to have it returned in 1955. The Gerstmann properties were seized in Vienna, resulting in a bitter postwar reclamation battle. Gerstmann immigrated to the United States quickly after the annexation and had some success in exile but never again directed a hospital. He maintained a private practice throughout his exile and, in the 1940s, had some research and consulting positions in New York. More than 75 years after theAnschluss, many questions remain unanswered about Gerstmann’s forced exile and the impact of becoming a refugee on his life and career. [ABSTRACT FROM PUBLISHER]

Dutch neuroscientist Cornelius Ubbo Ariëns Kappers is famous for pioneering neuroembryological work and for establishing the Amsterdam Central Institute for Brain Research. Less well known is his anthropological work, which ultimately played a role in saving Dutch Jews from deportation to their deaths during the Holocaust. Ariëns Kappers extensively campaigned against anti-Semitism and Nazi persecution during the 1930s. During World War II, he utilized his credentials to help create anthropological reports “proving” full-Jews were “actually” partial- or non-Jews to evade Nazi criteria, and at least 300 Jews were thus saved by Ariëns Kappers and colleagues. His earlier work demonstrating differences between Ashkenazic and Sephardic Jewish skull indices became the focus of an attempt to save hundreds of Dutch Portuguese Jews collectively from deportation. Ariëns Kappers and colleagues brilliantly understood how anthropology and neuroscience could be utilized to make a difference and to save lives during a tragic era. [ABSTRACT FROM PUBLISHER]

Hans Berger was a German neuropsychiatrist and head of the neurology department at the University of Jena, who discovered the human electroencephalogram (EEG). Many sources state that Berger was forced into retirement and suicide by the Nazis because he was at odds with the regime. In fact, Berger helped select his Nazi successor Berthold Kihn (complicit in “euthanasia” murders), financially supported the Nazi Schutzstaffel (SS), and was a willing participant on Nazi genetic health higher courts that reviewed appeals for forced sterilizations of neuropsychiatric patients. His motivations could be related to avoiding Nazi harassment, indoctrination by Nazi ideology, or less likely, career opportunism. His actions stand in contrast to colleagues who partially resisted the Nazis, and hopefully will serve as an example to future generations of neurologists regarding the danger of allowing one’s professional standing to be used as a tool to support the policies of tyranny and oppression. [ABSTRACT FROM AUTHOR]

Walther Birkmayer, an Austrian neurologist, codiscovered the efficacy of levodopa therapy for Parkinsonism in 1961. However, little has been published regarding Birkmayer’s ties to National Socialism. Through documentary review, we have determined that he was an early illegal member of the SS and the Nazi party, taking part in the “de-Jewification” of the Vienna University Clinic of Psychiatry and Neurology. He also was a leader in the Nazi racial policy office and was praised for his dedication and fanaticism despite being forced to later resign from the SS. He sought support from leading Viennese Nazis, and was able to maintain his professional status for the war’s remainder. Postwar, he succeeded at reintegration personally and professionally into Austrian society, all but erasing any obvious ties to his Nazi past. His story reflects ethical transgressions regarding professional and personal behavior in response to a tyrannical regime and provides lessons for today’s neuroscientists. [ABSTRACT FROM PUBLISHER]

Peter Becker was a German neurologist who helped classify the muscular dystrophies, and described Becker muscular dystrophy and Becker myotonia. His involvement in National Socialism began in 1933, when he was compelled by his peers to join the SA (brown shirts). He later joined the Nazi party, the Nazi Doctors Association, and the Nazi Lecturers’ Association. He renewed his SA membership to maintain his position at a genetics institute. Colleagues stated postwar that he was not an active Nazi, and he was de-Nazified in 1947, able to continue his career. Later, Becker admitted to most, but not all, of his Nazi memberships in his autobiography, and wrote 2 books exploring the origins of Nazism and racial hygiene. The “neurologic court of opinion” must weigh in on how we should best remember Becker, and at the very least, we as neurologists must learn the dangers of career opportunism at any cost. [ABSTRACT FROM PUBLISHER]

There has been a movement to rename Hallervorden–Spatz disease to pantothenate kinase-associated neurodegeneration given Hallervorden and Spatz’s complicity in murderous Nazi programs. Similar controversy surrounds Reiter syndrome, and 2 studies demonstrated decreased unqualified use of that eponym in the literature, but not in textbooks. There have been no similar studies regarding Hallervorden–Spatz disease. The authors performed a MEDLINE search (1990-2010) looking for unqualified use of Hallervorden–Spatz disease and performed statistical analysis. They defined “unqualified” as having no reference to the eponym’s disfavored use. They then looked in 6 neurology textbooks. The authors identified 156 of 278 articles (56.1%) containing unqualified use of Hallervorden–Spatz disease. But there was a declining trend (P = .000), with 70/80 (87.5%) of articles from 1990 to 1999 and 86/198 (43.4%) from 2000 to 2010. There was also decreased unqualified use of the eponyms in textbooks, with all recent editions using pantothenate kinase-associated neurodegeneration instead. The significant decrease in unqualified use of Hallervorden–Spatz disease is reassuring. [ABSTRACT FROM PUBLISHER]

ABSTRACT: Johannes Pompe is famous for describing type II glycogenosis, Pompe disease. However, Pompe's participation in the Dutch resistance during World War II has not been well described in the neurology literature. Pompe saved many Jews by hiding them as patients, saved a Jewish boy who was a neighbor, hid many young resistance fighters in his laboratory, resisted the Nazi call for all Dutch doctors to submit to their puppet physician's chamber, and hid a radio transmitter in the animal room of his laboratory. He was executed by firing squad in a German reprisal shortly before the end of the war. Pompe's patriotism and religious and humanitarian values seem to have been the basis for his actions. His heroic and tragic story should not be forgotten and should serve as an example to all during such dark times. Muscle Nerve 46: 134-138, 2012 [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome, or acute inflammatory demyelinating polyradiculoneuropathy, and immune thrombocytopenic purpura are both autoimmune disorders thought to result from molecular mimicry in response to an antecedent introduction of foreign antigen. Guillain-Barré syndrome is an ascending motor paralysis that can lead to respiratory compromise. Immune thrombocytopenic purpura is an isolated disorder of platelet destruction leading to mucocutaneous bleeding. Guillain-Barré does not typically occur with other autoimmune disorders, and concurrent Guillain-Barré and immune thrombocytopenic purpura has only rarely been reported. We present a patient with both conditions who experienced prompt resolution of neurologic and hematologic sequelae after intravenous immunoglobulin therapy was initiated within 12 hours of presentation. The case provides further evidence that Guillain-Barré syndrome and immune thrombocytopenic purpura can occur simultaneously, possibly caused by a similar pathogenic mechanism, as well as suggesting that the prompt initiation of intravenous immunoglobulin is an effective monotherapy leading to prompt resolution of both conditions and prevention of further sequelae. [Copyright &y& Elsevier]

Small fiber neuropathy (SFN) is a prevalent neurologic syndrome. Testing methods have emerged in recent years to better diagnose it, including autonomic tests and skin punch biopsy. SFN can present in a non-length-dependent fashion and can be mistaken for syndromes such as fibromyalgia and complex regional pain syndrome. SFN is caused by a variety of metabolic, infectious, genetic, and inflammatory diseases. Recently treatments have emerged for TTR amyloid neuropathy and Fabry disease, and novel biomarkers have been found both in genetic and inflammatory SFN syndromes. Ongoing trials attempt to establish the efficacy of intravenous immunoglobulin in inflammatory SFN syndromes., Competing Interests: Disclosure Dr L.A. Zeidman has participated on a scientific advisory board for Akcea Therapeutics., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Russian-born, Vienna-trained neurologist and neuropathologist Ilya Mark Scheinker collaborated with Josef Gerstmann and Ernst Sträussler in 1936 to describe the familial prion disorder now known as Gerstmann-Sträussler-Scheinker disease. Because of Nazi persecution following the annexation of Austria by Nazi Germany, Scheinker fled from Vienna to Paris, then after the German invasion of France, to New York. With the help of neurologist Tracy Putnam, Scheinker ended up at the University of Cincinnati, although his position was never guaranteed. He more than doubled his prior publications in America, and authored three landmark neuropathology textbooks. Despite his publications, he was denied tenure and had difficulty professionally in the Midwest because of prejudice against his European mannerisms. He moved back to New York for personal reasons in 1952, dying prematurely just 2 years later. Scheinker was twice uprooted, but persevered and eventually found some success as a refugee.

Adolf Wallenberg became the "anatomical conscience" to at least one famed neurologist, and was known worldwide by top neurologists. His comprehensive clinical-pathological descriptions of what became known as Wallenberg Syndrome had a large impact on neurology and launched his career. He did not let a skull base injury from an accident, or his service in the German army in World War I, impede his progress. Despite his accomplishments, because he was Jewish he was stripped of his research laboratory and forced to stop working when the Nazis took over his native Danzig. He barely escaped just before World War II began and immigrated to England, then to the United States. Because of his impact on neurology and his unusual strife, his story is one that neuroscientists should not forget.

Although Scandinavian neuroscience has a proud history, its status during the Nazi era has been overlooked. In fact, prominent neuroscientists in German-occupied Denmark and Norway, as well as in neutral Sweden, were directly affected. Mogens Fog, Poul Thygesen (Denmark) and Haakon Sæthre (Norway) were resistance fighters, tortured by the Gestapo: Thygesen was imprisoned in concentration camps and Sæthre executed. Jan Jansen (Norway), another neuroscientist resistor, escaped to Sweden, returning under disguise to continue fighting. Fritz Buchthal (Denmark) was one of almost 8000 Jews escaping deportation by fleeing from Copenhagen to Sweden. In contrast, Carl Værnet (Denmark) became a collaborator, conducting inhuman experiments in Buchenwald concentration camp, and Herman Lundborg (Sweden) and Thorleif Østrem (Norway) advanced racial hygiene in order to maintain the "superior genetic pool of the Nordic race." Compared to other Nazi-occupied countries, there was a high ratio of resistance fighters to collaborators and victims among the neuroscientists in Scandinavia.

Dr. Haakon Sæthre was a leader of Norwegian neurology and psychiatry. He was resourceful, compassionate and had immense pride in his independent homeland. He described Sæthre-Chotzen syndrome (acrocephalosyndactyly type III). When Nazi Germany occupied Norway during World War II, Sæthre fearlessly and actively resisted, from revoking his medical association membership, to hiding persecuted Jews as patients in his psychiatric ward and aiding in their escape to Sweden, to managing the largest "illegal" food warehouse in Oslo with Danish humanitarian aid. As a prominent and noticeable citizen, he was arrested and executed by the Nazis in reprisal for the resistance's assassination of a hated Norwegian Nazi. His legacy lives on in Norway, where he was honored by a scholarship fund, a portrait and multiple plaques at Ullevål Hospital, and a street and memorial statue in his hometown. He was a hero and should be remembered by all who practice neurology.

In Part I, neuroscience collaborators with the Nazis were discussed, and in Part II, neuroscience resistors were discussed. In Part III, we discuss the tragedy regarding european neuroscientists who became victims of the Nazi onslaught on “non-Aryan” doctors. Some of these unfortunate neuroscientists survived Nazi concentration camps, but most were murdered. We discuss the circumstances and environment which stripped these neuroscientists of their profession, then of their personal rights and freedom, and then of their lives. We include a background analysis of anti-Semitism and Nazism in their various countries, then discuss in depth seven exemplary neuroscientist Holocaust victims; including Germans Ludwig Pick, Arthur Simons, and Raphael Weichbrodt, Austrians Alexander Spitzer and Viktor Frankl, and Poles Lucja Frey and Wladyslaw Sterling. by recognizing and remembering these victims of neuroscience, we pay homage and do not allow humanity to forget, lest this dark period in history ever repeat itself.

Previously, I mentioned that not all neuroscientists collaborated with the Nazis, who from 1933 to 1945 tried to eliminate neurologic and psychiatric disease from the gene pool. Oskar and Cécile Vogt openly resisted and courageously protested against the Nazi regime and its policies, and have been discussed previously in the neurology literature. Here I discuss Alexander Mitscherlich, Haakon Saethre, Walther Spielmeyer, Jules Tinel, and Johannes Pompe. Other neuroscientists had ambivalent roles, including Hans Creutzfeldt, who has been discussed previously. Here, I discuss Max Nonne, Karl Bonhoeffer, and Oswald Bumke. The neuroscientists who resisted had different backgrounds and motivations that likely influenced their behavior, but this group undoubtedly saved lives of colleagues, friends, and patients, or at least prevented forced sterilizations. By recognizing and understanding the actions of these heroes of neuroscience, we pay homage and realize how ethics and morals do not need to be compromised even in dark times.

The Nazi regime in Germany from 1933 to 1945 waged a veritable war throughout Europe to eliminate neurologic disease from the gene pool. Fueled by eugenic policies on racial hygiene, the Nazis first undertook a sterilization campaign against "mental defectives," which included neurologic patients with epilepsy and other disorders, as well as psychiatric patients. From 1939-41 the Nazis instead resorted to "euthanasia" of many of the same patients. Some neuroscientists were collaborators in this program, using patients for research, or using extracted brains following their murder. Other reviews have focused on Hallervorden, Spatz, Schaltenbrand, Scherer, and Gross, but in this review the focus is on neuroscientists not well described in the neurology literature, including Scholz, Ostertag, Schneider, Nachtsheim, and von Weizsäcker. Only by understanding the actions of neuroscientists during this dark period can we learn from the slippery slope down which they traveled, and prevent history from repeating itself.