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Objective Novel antibodies to trisulfated heparin disaccharide (TS-HDS) and fibroblast growth factor receptor 3 (FGFR-3) have been recently described in otherwise cryptogenic small fiber neuropathy (SFN) cases. Our goal was to further describe clinical features in such cases and to analyze treatment responses. Methods In a retrospective analysis, 40 cases of cryptogenic SFN in a university neuropathy clinic were identified. Of these, TS-HDS and FGFR-3 cases were identified, and clinical features and treatment responses were analyzed. Results In this cohort, 95% were women, and 55% had either TS-HDS or FGFR-3 antibodies (77% of these had TS-HDS). Of the seropositive group, 41% had a nonlength dependent epidermal nerve fiber density on skin punch biopsy (OR = 1.80). In the seropositive group, 82% had neuropathic pain as their primary symptom (OR = 1.73). Also 32% of seropositive patients reported widespread pain (OR = 1.63). 63% of seropositive cases presented acutely (OR = 11.0). In the seropositive group, 23% had an initial erroneous diagnosis (OR = 1.47). Eight seropositive patients improved on intravenous immunoglobulin treatment, with a 42% reduction in pain scores (P = 0.02), a 44% reduction in the Utah Neuropathy Score, and improved epidermal nerve fiber density post-treatment. Conclusions TS-HDS and FGFR-3 antibodies may be present in a high proportion of cryptogenic SFN cases with acute onset, nonlength dependent pathology, and primary neuropathic and widespread pain. They are often misdiagnosed as other conditions including fibromyalgia. These cases may be responsive to immune treatment, especially with intravenous immunoglobulin.

Up to 50% of small fiber neuropathy (SFN) cases are idiopathic, but novel antibodies to Trisulfated Heparin Disaccharide (TS-HDS) and fibroblast growth factor receptor 3 (FGFR-3) have been implicated in half of these cases; the role of anti-Plexin D1 is less clear. We aimed to clarify presentation and management of these patients.An 18-month retrospective analysis revealed 54 cases of cryptogenic SFN who had testing for the 3 autoantibodies. Demographics, clinical features, epidermal nerve fiber density, and Quantitative Sudomotor Axon Reflex Test results were analyzed. Intravenous immunoglobulin (IVIG) treatment response was assessed.In total, 44.4% of patients had antibodies (62.5% TS-HDS, 29.2% FGFR-3, and 20.8% Plexin D1). Male patients were more likely to be FGFR-3 positive (P = 0.014). Facial involvement was more common in seropositive patients (P = 0.034), and patients with a higher Utah Early Neuropathy Scale score had a higher TS-HDS titer (P = 0.0469), but other clinical features were not significantly different. Seropositive patients trended toward a higher SFN screening list score (P = 0.16), abnormal Quantitative Sudomotor Axon Reflex Test (P = 0.052), and prior erroneous diagnosis (P = 0.19). In patients who completed IVIG, examinations and questionnaires improved and mean epidermal nerve fiber density increased by 297%.TS-HDS, FGFR-3, and Plexin D1 antibodies are present in a high proportion of cryptogenic SFN cases with more facial involvement, and greater disease severity is associated with higher antibody titers. They are often misdiagnosed but may respond subjectively and objectively to IVIG.

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.

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.

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.

Eighty years ago the greatest mass murder of human beings of all time occurred in Nazi occupied Europe. This began with the mass extermination of patients with neurologic and psychiatric disorders that rendered them “useless eaters” to Hitler’s regime. The neuropsychiatric profession was systematically “cleansed” beginning in 1933, but racism and eugenics had infiltrated the specialty in the decades before that. With the installation of Nazi-principled neuroscientists, mass forced sterilization was enacted, which slowed down by the start of World War II and the advent of patient murder. But the murder of roughly 275,000 patients by the end of the war was not enough. The patients’ brains and neurologic body parts were stored and used in scientific publications both during and long after the war. Also, patients themselves were used in unethical ways for epilepsy and multiple sclerosis experiments. Relatively few neuroscientists resisted the Nazis, with some success in the occupied countries. Most neuroscientists involved in unethical actions continued their careers unscathed after the war. Few answered for their actions in a professional or criminal sense, and few repented. The legacy of such a depraved era in the history of neuroscience and medical ethics is that codes exist by which patients and research subjects are protected from harm. But this protection is possibly subject to political extremes and only by understanding the horrible past can our profession police itself. Individual neuroscientists can protect patients and colleagues if they are aware of the dangers of a utilitarian, unethical, and uncompassionate mindset.

Eugenic principles originated in the nineteenth century, along with the related subject of racial hygiene. Eugenics became popular globally, not just in Germany, and was seen as a solution to society’s problems of poverty, crime, and mental illness. Neuroscientists flocked to eugenics, but long before that they helped popularize scientific racism by espousing ideas about smaller skull sizes in so-called “inferior” races. Even famous neuroscientists of the nineteenth and early twentieth century became staunch eugenicists, including Forel, Möbius, Anton, Lundborg, Lennox, and Foster Kennedy. Thus, it isn’t surprising that leading up to the Nazi era, neuroscientists were some of the biggest proponents of forced sterilization and even euthanasia programs as negative eugenics measures to rid society of unwanted elements, including neurologic and psychiatric patients who were seen as “burdens.” Notably, not all neuroscientists believed in eugenic measures on these patients, with some calling for “exoneration of the feebleminded.”

Some German and Austrian neuroscientists, instead of condemning the patient murder, actually saw a great opportunity in the euthanasia programs. The opportunity was for scientific research on a scale that would never have been possible without the murder programs. The brain parts of these murdered “beautiful defectives” would be used during and long after the war in scientific publications, largely containing mere pathologic descriptions or regarding hereditary causes of neurologic disease. Brain transfer networks existed based on individual scientific interests of neuropathologists at various German institutes; thus, a symbiotic relationship can be seen in the neuroscientists’ desire for scientific material and research funding, which fueled and further intensified the killers’ desire to eliminate patients. The lack of humanity and ethics by Hallervorden, Ostertag, and others regarding this indirect collaboration in killing is striking, as is their lack of repentence or informing future generations about the unethical provenance of the “material.”

The first negative eugenic measure passed by the Hitler regime in July 1933 was the forced sterilization law, which primarily targeted neuropsychiatric patients with “feeblemindedness,” schizophrenia, and epilepsy. Also included were Huntington’s chorea, bipolar disorder, and alcoholism, along with other congenital defects. The law was based on a prior Weimar Germany draft that was never enacted. Although Germany was late to enact a sterilization law, it was more rapidly implemented and executed than in other countries. In 12 years of the Nazi regime, 400,000 people were forcibly sterilized. The hypocritical execution of the law, supposedly foolproof because of the use of “hereditary health courts” and appeals courts, resulted in people of lower socioeconomic background being preferentially affected, and also in non-hereditary forms of diseases (e.g., symptomatic epilepsy) being included. Most in German neuroscience also seemed unconcerned with the 0.5% mortality rate of sterilization procedures, resulting in at least 2000 deaths.

Beginning with even some famous neuroscientists in the nineteenth century anti-Semitism prevented professional promotions and left Jews as outsiders. This included Oppenheim, Flatau, Freud, Liepmann, Weigert, Lewandowsky, Edinger, and others. The specialties of neurology and psychiatry in the first place were fringe areas in which Jews could gravitate and build careers in a field others shunned. But pervasive and insidious discrimination existed in most German and Austrian universities, and Jewish neuroscientists were rarely made department chairs or institute heads. In some instances independent hospitals or clinics in larger cities such as Berlin or Vienna could be headed by Jews, but this became increasingly rare and these dreams were shattered at the onset of the Nazi era. Even as heads of some independent hospitals Jewish neuroscientists faced intolerable degrees of hatred and roadblocks, but persevered and contributed heavily to the growth of neuroscience.

During the war, a more austere method of eliminating “useless eaters” was adopted by the Nazi state: killing patients. The concept of patient murder dated back to Hoche, a neuropsychiatrist, and Binding, a lawyer, and their 1920 book Permission for the Destruction of Life Unworthy of Life. Only in the totalitarian Nazi state, however, could an illegal secret action be authorized leading to the extermination of at least 275,000 neuropsychiatric patients. After about 70,000 patients were killed by gassings in the first phase (Action T4), the program was carried out on a smaller scale. The T4 medical director was a neuroscientist, at least 42.5% of the T4 assessors were neuroscientists, and among the T4 assessors were at least eight neuropsychiatry “Professor Doctors.” Killing methods included starvation, drug overdoses, and killing by painful pneumoencephalograms. It was the Nazi neuroscientists’ “devolution” from small initial ethical transgressions that resulted in patient murder.

With Hitler’s 1933 power seizure, the fate of roughly 9000 political and racial “enemy” neuroscientists was sealed. In the “Gleichschaltung,” or coordination of German neuroscience, step-wise legal and professional sanctions occurred against “non-Aryan” neuroscientists at every university neurology department in Germany. These were either dismissed within the first months of the Nazi takeover or by the passage of the 1935 Nuremberg Laws. Sometimes the dismissed neuroscientists were forcibly removed by colleagues and sometimes they were arrested and imprisoned for trumped-up charges. Even private neurologists were forbidden from seeing insurance panel patients. Half of all German doctors joined the Nazi party, eager to take the positions vacated by their Jewish and communist comrades. The German neurology and psychiatry societies were merged to facilitate Gleichschaltung and Nazi influence on the specialties. And flourishing research and clinical programs were halted and then replaced by the racial hygiene agenda of the Nazi state.

Neuropsychiatric patients were also subjected to various experiments during the euthanasia programs. The unethical experiments ranged from photography using restraints, to inducing hypoxic or hypercarbic states in epileptics to monitor electroencephalographic changes, to exposing children to hypoxia to induce seizures, to inoculating spinal fluid with monkey fluids to transmit a potential multiple sclerosis-causing viral agent, and tuberculosis experiments. These “expendable” patients were loaned from local mental institutions, and some died during the experiments. Results were published in scientific journals and continued to be cited as legitimate experiments long after the war. The individual scientists did not face any sanctions for the unethical experimental designs. Justifications given were that the patients were demented and couldn’t experience pain, or that the experimenter was in the vacuum chamber with the children. But the experiments violated 1931 Weimar guidelines and the Hippocratic Oath, even if formal ethical restrictions were not standardized at the time.

Despite knowledge since the postwar period and the efforts of neurologist Leo Alexander, the neuroscience community has been slow to recognize its involvement in the racial hygiene policies of the Third Reich. Part of this has been denial, but part of it protective of past perpetrators. However, since the popularization of medicine in the Nazi era in the 1980s, the fall of the Berlin Wall making previously unavailable patient data in the 1990s, and some astute articles in the neurology literature, neuroscience in the Nazi era has emerged as a scientific topic. Pioneering works by Shevell and Peiffer highlighted the unethical involvement of even famed German neuroscientists such as Julius Hallervorden. In the 2000s a growing body of literature has begun to show common threads between the exile of persecuted neuroscientists and the rise of increasingly destructive policies toward neurologic patients, and the exploitation of these patients for scientific research.

The first postwar period (1945–50) consisted, importantly, of the Nuremberg Medical Trial and formation of the Nuremberg Code for medical ethics. During the initial period most collaborator neuroscientists, even those involved in patient selection for euthanasia, escaped largely unscathed. The latter faced temporary career setbacks but re-emerged in the next decade professionally. The second period (1950–70) included a seminal event, the Hallervorden Affair, which pertained to the 1953 International Neuropathology Conference in Lisbon. Former Dutch neuroscientist resistor Rademaker and the Dutch delegation withdrew when they found out Hallervorden was presenting, leading to Hallervorden and Spatz’s withdrawal but only after fiery correspondence on both sides of the argument. Some collaborator neuroscientists continued to support sterilization, and even spoke against victims’ rights. Overall, only three of 25 (12%) of collaborators ever publicly repented for their involvement in Nazi programs, suggesting against an ability to rehabilitate for this directly involved group.

The Kaiser Wilhelm Institutes (KWI) in Germany were supposed to be bastions of internationally renowned science, but were just as easily “coordinated” under National Socialism in Germany as the universities and public hospitals and clinics. The KWIs for brain research in Berlin and Munich, even partially founded by a Jew in the case of the latter, became primary sites for research related to the Nazi “euthanasia” programs. The transition from physiologic to pathologic research at each institute, facilitated by replacement of dissident neuroscientists with more loyal and ethically pliable neuroscientists, such as Spatz and Scholz, helped to set the stage for euthanasia research. Illustrated in this chapter is the fact that at least seven replacements of non-Aryans or dissidents at various levels significantly facilitated the coordination of the KWIs. One of these, Marthe Vogt, was not actually a dismissal, but a voluntary emigration from Germany in rejection of the Nazis.

In at least 26 instances of “non-Aryan” neuroscientist dismissal, their replacements were involved in racial hygiene consequences such as aiding the vehement forced sterilization program, euthanasia of neuropsychiatric patients, or collection of brain tissue and research on these expendable victims. The hardest-hit departments were in the major German cities, especially Berlin, where both the university and multiple smaller hospitals and institutes were decapitated and where Jews had been directors prior to the Nazi power seizure. University neurology departments in Frankfurt, Hamburg, Munich, Bonn, Heidelberg, and Breslau were also heavily incapacitated by dismissals. Those who took over the positions of dismissed neuroscientists were often members of not only the Nazi party, but multiple subsidiary agencies, such as the SS, SA, and others, likely reflecting deeper commitment to Nazi ideology. Six known “Aryan” neuroscientists emigrated from Germany, reflecting the fact that support of the regime was neither mandatory, nor the only solution.

The Austrian neuroscience consolidation came swiftly and terribly on “non-Aryans.” Austrian anti-Semitism was arguably even more virulent than in Germany. And laws had already escalated in Nazi Germany to the point that Jewish physicians at most could only treat other Jews as derogatorily called “sick treaters”; these laws were instantly applicable in “annexed” Austria, with no stepwise progressive disfranchisement. Even “Aryan” neurologists who were thought to be unsympathetic to the Nazi movement were dismissed shortly after the “annexation.” The Vienna university neurology clinic was taken over primarily by SS neurologists who had been “illegal” Nazis before the annexation and were extremely dedicated to the Nazi cause. At least one, Walther Birkmayer, spoke of expanding the sterilization law to other hereditary conditions not stipulated already by the law. At least nine racial or political neuroscientist replacements, including directors of institutes, led to racial hygiene consequences, including execution of sterilization and euthanasia programs.

In recent decades, there has been a more critical examination of the Nazi past within German and Austrian neuroscience. The Spiegelgrund euthanasia brains and brain parts in Vienna were finally buried by 2012 and victims were commemorated. More anonymous brain burials occurred in Munich and Tübingen in the early 1990s, which likely did not adequately commemorate victims and, furthermore, a recent comprehensive investigation of all brain specimens held by the Max Planck Society is underway. The Hugo Spatz Prize was renamed by the German Neurological Society, but the Heinrich Pette Prize still exists. This society and another have laudably conducted investigations leading to publications about Nazi-era neuroscience, but much work must still be done. Additionally, Hallervorden–Spatz disease has largely been renamed, but other collaborator eponyms remain in use and raise the question of what response the neuroscience community should take toward these, and toward experimental data from Nazi-era investigations.

Several variants of Guillain-Barré syndrome have been described. The Fisher syndrome (FS) presents with ataxia, areflexia, and ophthalmoparesis. The pharyngeal-cervical-brachial (PCB) variant presents with bulbar weakness, along with arm and neck weakness. The 2 variant syndromes can overlap. Both the isolated and overlap syndromes respond to immunomodulatory treatment, thus are important to recognize clinically. Ganglioside antibodies are detectable in the variant syndromes and may aid in their diagnosis. The FS typically is associated with anti-GQ1b antibodies, and PCB is typically associated with anti-GT1a antibodies, whereas the overlap syndrome may have both ganglioside antibody subtypes. We present a case of overlap FS-PCB syndrome with a novel ganglioside antibody profile of GM1 and GD1b antibodies, which typically are associated with other variant syndromes. This case suggests the need for all ganglioside antibodies to be tested in suspected variant Guillain-Barré syndromes. The antibodies may prove especially useful in cases in which the clinical diagnosis is ambiguous.

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

In 1936, Austrian neuroscientists Josef Gerstmann and Ernst Straussler, along with expatriate Russian neuroscientist Ilya Mark Scheinker, described the familial prion disorder later named for them from a case they mutually treated at a Viennese neurologic hospital. In 1938, Austria was annexed to Nazi Germany in the Anschluss , effectively ending any collaboration between the 3 men. Gerstmann and Scheinker eventually immigrated to America, and Straussler, although dismissed from his faculty position, remained protected from persecution in Vienna throughout the war likely because of his marriage to an “Aryan woman.” Although he attained some degree of success in exile, Gerstmann was never again director of a hospital and primarily maintained a private practice after some brief consulting positions in New York in the 1940s. His medical degree was retroactively stripped by the Nazis without his knowledge, and was not reinstated until 1955. Gerstmann also became embroiled in a bitter struggle to regain his confiscated property in Vienna. Scheinker, aided by the refugee resettlement committee, settled in Cincinnati where he had several successful years and published 3 textbooks, but was denied university tenure and entered private practice until his untimely death. All 3 neurologists lost significant career momentum, and had to pick up the pieces of their fractured lives after the war or their forced exile. Their stories reflect many of the tragic realities of Nazi persecution of Jewish physicians.

OBJECTIVES The combined sensory index (CSI), a sensitive composite score of 3 median sensory comparison studies, may still be underutilized in diagnosing mild cases of carpal tunnel syndrome (CTS). Our goal was to compare the effectiveness of the "standard" median digit 2 (D2) sensory study to a CSI algorithm in diagnosing mild CTS. METHODS We retrospectively identified patients with typical CTS symptoms and signs. Electrodiagnostically normal patients and those having mild CTS diagnosed by D2 or CSI algorithm were separated into groups. RESULTS Seventy-four patients were included, and 51 (68.9%) were diagnosed with mild CTS. Of the 51, 31 (60.8%) were diagnosed using the CSI algorithm, and 20 (39.2%) were diagnosed using D2 (P < 0.001). CONCLUSIONS Our data suggest that the CSI algorithm is significantly more effective than the D2 to diagnose mild CTS. If mild CTS is diagnosed earlier, treatment can be initiated sooner and morbidity can likely prevented.

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.

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.

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.

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.

Introduction: Prior studies have demonstrated superiority of the combined sensory index (CSI) algorithm in diagnosing mild carpal tunnel syndrome (CTS) and have compared presenting symptoms to CTS grade. However, CTS symptoms, signs, and outcomes, including CSI-diagnosed cases, have not been compared with CTS grade. Methods: We retrospectively studied 294 CTS hands from 2010 to 2013; stratified them into mild, moderate, and severe grades; and analyzed the association between CTS grade and presenting symptoms/signs and outcomes. Results: Sensorimotor symptoms (P = 0.017) and signs (P < 0.001) were significantly associated with CTS grade. Regardless of CTS grade, 94% of hands improved with surgery compared with 42% with conservative treatment (P < 0.001). Even in mild CTS, 100% improved with surgery vs. 33% with conservative management (P = 0.011). Discussion: These results corroborate prior studies that compared symptoms to CTS grade and suggest that more objective signs associate even better. CTS grades associate with outcomes, but additional studies are required. Muscle Nerve 57: 45–48, 2018

Several neuropathologists conducted brain research on victims of so-called euthanasia programs carried out by the National Socialist (Nazi) regime in Germany from 1940 to 1945. Some published their results in German journals or books during and after the war. One of these neuropathologists was Hans Jacob of Hamburg, a former Nazi party member and the leader of the same laboratory previously run by Alfons Jakob (Creutzfeldt-Jakob disease). Though much has been published on the unethical actions of Jacob's fellow neuropathologist from Berlin, Julius Hallervorden, Jacob's actions were remarkably similar and have not been previously analyzed in the neuroscience literature. Jacob dissected at least 42 patient brains from euthanasia centers near Hamburg, and saved the specimens from at least 17 of them. He published a 1956 book chapter featuring 2 such specimens. Jacob was denazified, had a notable career, and never publicly addressed his actions during the war. His ethical violations may not have been on the same scale as Hallervorden’s, but the effect of his work echoes to the modern era. As responsible researchers, we must always be conscious of the provenance of material provided and not succumb to opportunistic temptation despite the ethical consequences.

The 1920s were a booming decade for neuroscience, and perhaps nowhere was this truer than in Germany. Following the rise of Hitler's regime and the persecution of Jews and others, however, Germany and Austria lost numerous world-class neuroscientists. Vacant posts were quickly filled with 'Aryan' and academic staff loyal to the Nazis. Indeed, many physicians and scientists went even further and became engaged in National Socialist (NS)-euthanasia programs. In recent years, the medical community has become more aware of the ethical burden associated with eponyms derived from scientists of the Third Reich. This book chapter reviews 53 neurological eponyms derived from physicians who worked in the Nazi era. Among them are victims who were forced out of the country or murdered in concentration camps, protestors who risked their academic careers and often their lives, beneficiaries who published on brains from 'euthanized' children, and collaborators who were directly involved in the planning and execution of NS-euthanasia programs.

Neuroscientists played central roles in the victimization of colleagues and their patients during the era of National Socialism from 1933 to 1945. After helping dismiss Jewish and nonideologically aligned colleagues, German neuroscientists were among the physicians and researchers who joined the Nazi Party and affiliated groups in record numbers. Forced sterilization and then so-called 'euthanasia' of neurological and psychiatric patients were planned and executed by prominent German and Austrian neuroscientists. Other neuroscientists collaborated indirectly by using patients for unethical experimentation to discover the cause of multiple sclerosis or to try to induce epileptic convulsions in a hypoxic state. Some merely used neuropathological material from murdered patients for publications in scientific journals. In the totalitarian state, research funding and academic advancement were awarded to physicians engaged in eugenics research. Opportunism and ideologically tainted science without regard to medical ethics were the motivating factors for collaborating neuroscientists. Some German and Austrian neuroscientists tried to resist Nazi policies, although much more passively than their colleagues in German-occupied countries. French, Dutch, Norwegian, and Danish neuroscientists actively resisted the Nazification of their profession from the beginning and helped to save some patients and colleagues, at great personal risk. Many German, Austrian, Czech, and Polish neurologists were murdered in the Holocaust, and hundreds of thousands of neurological and psychiatric patients were sterilized or murdered in just 12 years. The Nazis used the 'successful' techniques developed in the 'euthanasia' programs to carry out the mass murder of millions in the Holocaust. Today's neuroscientists are obligated to learn of the ethical violations of their predecessors 70-80 years ago. No law will prevent abandonment of the basic principles of ethical patient care and professionalism that can occur in any totalitarian state, but neuroscientists can possibly prevent it.

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.

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.

Neuroscientists played central roles in the victimization of colleagues and their patients during the era of National Socialism from 1933 to 1945. After helping dismiss Jewish and nonideologically aligned colleagues, German neuroscientists were among the physicians and researchers who joined the Nazi Party and affiliated groups in record numbers. Forced sterilization and then so-called 'euthanasia' of neurological and psychiatric patients were planned and executed by prominent German and Austrian neuroscientists. Other neuroscientists collaborated indirectly by using patients for unethical experimentation to discover the cause of multiple sclerosis or to try to induce epileptic convulsions in a hypoxic state. Some merely used neuropathological material from murdered patients for publications in scientific journals. In the totalitarian state, research funding and academic advancement were awarded to physicians engaged in eugenics research. Opportunism and ideologically tainted science without regard to medical ethics were the motivating factors for collaborating neuroscientists. Some German and Austrian neuroscientists tried to resist Nazi policies, although much more passively than their colleagues in German-occupied countries. French, Dutch, Norwegian, and Danish neuroscientists actively resisted the Nazification of their profession from the beginning and helped to save some patients and colleagues, at great personal risk. Many German, Austrian, Czech, and Polish neurologists were murdered in the Holocaust, and hundreds of thousands of neurological and psychiatric patients were sterilized or murdered in just 12 years. The Nazis used the 'successful' techniques developed in the 'euthanasia' programs to carry out the mass murder of millions in the Holocaust. Today's neuroscientists are obligated to learn of the ethical violations of their predecessors 70-80 years ago. No law will prevent abandonment of the basic principles of ethical patient care and professionalism that can occur in any totalitarian state, but neuroscientists can possibly prevent it.

The 1920s were a booming decade for neuroscience, and perhaps nowhere was this truer than in Germany. Following the rise of Hitler's regime and the persecution of Jews and others, however, Germany and Austria lost numerous world-class neuroscientists. Vacant posts were quickly filled with 'Aryan' and academic staff loyal to the Nazis. Indeed, many physicians and scientists went even further and became engaged in National Socialist (NS)-euthanasia programs. In recent years, the medical community has become more aware of the ethical burden associated with eponyms derived from scientists of the Third Reich. This book chapter reviews 53 neurological eponyms derived from physicians who worked in the Nazi era. Among them are victims who were forced out of the country or murdered in concentration camps, protestors who risked their academic careers and often their lives, beneficiaries who published on brains from 'euthanized' children, and collaborators who were directly involved in the planning and execution of NS-euthanasia programs.

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.

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.

Meningiomas are dural-based brain tumors that are typically histologically benign. Some meningiomas grow slowly or seemingly not at all with planimetric measurement. Volumetric measurement may be more accurate because tumors may grow in different directions than the planimetric axes. Twenty-one patients (with 22 tumors) had serial MRI brain scans available for review. We reviewed the charts and measured tumor dimensions on the MRI scans. Relative growth rates were calculated for volume and maximum initial diameter using published formulas. Patient demographics, tumor location, and special radiologic characteristics (calcification, T2 hypointensity, dural tail, mass effect, and midline shift) were compared to the volumetric growth rate. Patients included 17 females and 4 males; age at diagnosis 36 to 74 years (mean 61). Follow-up was 2.08 to 10.83 years (mean 3.64). Most tumors were located in the convexity (27.27 %), sphenoid wing (27.27 %), or cerebellopontine angle (13.04 %). Two meningiomas (9.09 %) demonstrated no growth. The mean relative volumetric growth rate was 5.82 %/year, and planimetric was 2.00 %/year (difference 3.82 %/year, p-value < 0.0001). Convexity location had near significant association with slower relative volumetric growth. There were no significant associations between other tumor locations, age, gender, or radiologic characteristics and volumetric growth. The mean volumetric growth rate was significantly greater than the planimetric growth rate, suggesting that volumetric measurement conveys more information and is superior in assessing tumor growth. This information could have clinical value in determining the frequency of follow-up imaging and the urgency of surgical intervention.

A 44-year-old woman with a history of hypothyroidism taking daily levothyroxine was admitted to an outside hospital with subacute cognitive decline. Her symptoms had started 3 weeks previously with headache, sore neck, and upper respiratory symptoms for which she sought care at a local emergency room. She did not complain of confusion or demonstrate signs of cognitive decline at that time and was discharged home with a prescription for antibiotics. A few days later, she was hospitalized after appearing disoriented, exhibiting signs of confusion, responding slowly, and running into things at work.