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Competing Interests: Declarations. Conflicts of interest: On behalf of all authors, the corresponding author states that there is no conflict of interest. Consent to participate: Not applicable. Consent for publication: Not applicable. Ethics approval: Not applicable.

Objectives: To demonstrate treatment efficacy on composite and non-length-dependent (NLD) punch biopsy specimens from intravenous immunoglobulin (IVIG) in pure small-fiber neuropathy (SFN) with trisulfated heparin disaccharide (TS-HDS), fibroblast growth factor-3 (FGFR-3), or Plexin D1 antibodies. SFN has an increasing prevalence, and over 30% of cases may be immune-mediated. TS-HDS, FGFR-3, and Plexin D1 autoantibodies have been shown to be present in 44%-55% of cryptogenic SFN cases, suggesting an immune mechanism. Reports have shown IVIG to be effective for this condition, but some controversy exists based on length-dependent (LD) post-IVIG treatment data in a recent trial., Methods: In a retrospective review, all pure SFN cases tested for the 3 antibodies from January 2021 to May 2022 were tabulated, and patients who underwent IVIG treatment were separated and analyzed for changes in epidermal nerve fiber density (ENFD) on skin biopsy, as well as SFN-specific questionnaire and pain scores., Results: Ninety-one patients with pure SFN had antibody testing. Sixty of these (66%) were seropositive, and 31 (34%) were seronegative. Seventeen seropositive patients (13 female patients, 4 male patients, 6 FGFR-3, 2 TS-HDS, 4 Plexin D1, 2 with all 3 antibodies, 1 with FGFR-3 and Plexin D1, 1 with FGFR-3 and TS-HDS, and 1 with TS-HDS and Plexin D1) underwent IVIG treatment. Of these, 2 patients stopped treatment due to side effects, and the remaining 15 completed at least 6 months of IVIG. Of these, 12 had a post-IVIG skin biopsy, and of these, 11 (92%) had a 55.1% improved mean composite ENFD (P = 0.01). NLD-ENFD specimens improved by 42.3% (P = 0.02), and LD-ENFD specimens improved by 99.7% (P = 0.01). Composite ENFD in Plexin D1-SFN patients improved by 139% (P = 0.04). In addition, 14 patients had questionnaires pre-IVIG/post-IVIG, and average pain decreased by 2.7 (P = 0.002)., Conclusions: IVIG shows disease-modifying effect in immune SFN with novel antibodies, especially Plexin D1-SFN, as well as significantly improved pain. NLD-ENFD should be examined as well as LD-ENFD to see this effect. Further randomized controlled trials looking at NLD-ENFD as well as LD-ENFD improvement, along with pain and SFN-specific questionnaires, are needed to confirm these findings., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: 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., Methods: 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., Results: 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%., Conclusions: 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., Competing Interests: L. A. Zeidman has received honoraria for participating on scientific advisory boards for Akcea Therapeutics and Takeda, for being on the CIDP speaker's bureau for Grifols, has received fees for expert medicolegal consulting, and has received royalties from Oxford University Press. The remaining authors report no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: L. A. Zeidman has received honoraria for participating on a scientific advisory board for Akcea Therapeutics and for being on the speaker's bureau for Grifols Inc, and has received fees for expert medicolegal consulting. The remaining authors report no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

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.)

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., (© 2020 Wiley Periodicals LLC.)

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., (© 2019 Wiley Periodicals, Inc.)

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: 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., (© 2017 Wiley Periodicals, Inc.)

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., (© 2017 American Academy of Neurology.)

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.

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.

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., (© 2016 S. Karger AG, Basel.)

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., (© 2016 S. Karger AG, Basel.)

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 the Anschluss, many questions remain unanswered about Gerstmann's forced exile and the impact of becoming a refugee on his life and career.

In 1936, Austrian neuroscientists Josef Gerstmann and Ernst Sträussler, 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 Sträussler, 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., (© 2014 American Academy of Neurology.)

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., (© The Author(s) 2013.)

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.

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.

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.

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.

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.

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.

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.

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., (Copyright © 2012 Wiley Periodicals, Inc.)

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.

Introduction: 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., Methods: 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., Results: 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., Conclusions: 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.

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.

Background: HELLP syndrome (a combination of hemolysis, elevated liver enzyme levels, and low platelet count) is a severe variant of preeclampsia that generally occurs before delivery but can occur post partum. This syndrome is more common than eclampsia and frequently leads to devastating neurological consequences such as intracerebral hemorrhage., Objective: Although mentioned in the obstetric literature, there has been sparse reporting in the neurology literature specifically regarding intracerebral hemorrhage in HELLP syndrome. We illustrate such a case and review the existing literature regarding this severe complication., Setting: Obstetric unit at an academic medical center., Patient: A 34-year-old primigravida experienced a pontine hemorrhage and subsequent respiratory arrest 22 hours after a normal delivery. This hemorrhage occurred 7 hours after the sudden onset of hypertension, severe headache, and intermittent abdominal pain., Results: Laboratory and postmortem evidence suggested HELLP syndrome with disseminated intravascular coagulation as the cause of her intracerebral hemorrhage., Conclusions: Our case suggests the importance of the neurology consultant's familiarity with HELLP syndrome and the need for thorough laboratory testing and close monitoring in the puerperal patient with headache and hypertension.