The making of a syndrome: The English translation of Gerstmann's first report.

The label Gerstmann syndrome indicates the co-occurrence of four symptoms in persons with acquired brain lesions: finger agnosia, left-right disorientation, agraphia, and acalculia. The syndrome is often associated with a lesion affecting the posterior parietal lobe of the left cerebral hemisphere. Virtually every paper discussing this tetrad of symptoms refers back to Josef Gerstmann's (1924) first report published in German. To make it accessible to the wider community of scholars and thus enable a more in-depth appreciation of the origins of this enigmatic syndrome, here we publish, for the first time in English, a translation of Gerstmann's initial report. In this paper, the syndromal construct had not yet crystallized into its four cardinal symptoms; Gerstmann's attention was mainly focused on finger agnosia and, to a lesser extent, on left-right disorientation by virtue of their significance for the body scheme theory. Although isolated agraphia and acalculia seemed to be at least as severe as finger agnosia, Gerstmann did not consider them of consequential importance (with the exception of agraphia's localisation value). It is also worth noting that the described patient presented a picture of Gerstmann syndrome associated with other symptoms such as hemianopia, balance impairment, and light memory and reasoning disorders.

Gerstmann syndrome: historic and current perspectives.

This chapter offers a perspective on the origin, operational definition, historic vicissitudes, and current status of Gerstmann syndrome. The main issues and controversy accompanying Gerstmann syndrome throughout the years are reviewed. The clinical picture of Gerstmann syndrome as it emerges from a series of modern-day pure cases is described. In current clinical practice, a diagnosis of Gerstmann syndrome indicates the concomitant presence of four acquired symptoms: finger agnosia, acalculia, left-right disorientation, and agraphia. Finally, based on empiric work conducted in recent years, the chapter concludes with a new interpretation of Gerstmann syndrome. If seen as an instance of intraparietal disconnection, this classic parietal syndrome will acquire fresh clinical and theoretic significance.

Síndrome de Gerstmann completo / Whole Gerstmann syndrome

El síndrome de Gerstmann es una rara enfermedad neurológica de causa vascular que afecta al lóbulo parietal. Se reporta el caso de un paciente masculino de 63 años con antecedentes patológicos personales de miocardiopatía dilatada. El 24 de enero de 2016 en horas de la mañana presentó de forma súbita pérdida de la conciencia con caída al suelo. Después de recuperarse tuvo estado confusional, desorientación topográfica de breve duración y dificultad para la expresión del lenguaje y la nominación de los objetos, por esta causa se decide su ingreso, durante el cual se realiza resonancia magnética simple de cráneo donde se observó imagen hipointensa en FLAIR en región parietal izquierda compatible con lesión isquémica. Se efectuó evaluación neuropsicológica encontrándose: agnosia digital, agrafia, acalculia y confusión derecha-izquierda. Se diagnostica un síndrome de Gerstmann completo(AU)

Gerstmann syndrome is a rare neurological disease of vascular cause that affects the parietal lobe. The case is reported of a 63-year-old male patient with a personal pathological history of dilated cardiomyopathy. In the morning of January 24, 2016, the patient presented sudden loss of consciousness with a fall to the ground. After recovering, he experienced a confusion state, short-lived topographical disorientation, and difficulty in expressing language and the naming of objects, a reason why his admittance was decided. During the admittance, simple magnetic resonance imaging to the skull was performed, which permitted to observe a hypo-intense image in FLAIR in the left parietal region and that was compatible with an ischemic lesion. A neuropsychological evaluation was performed: digital agnosia, agraphia, acalculia and confusion regarding right or left. A whole Gerstmann syndrome was diagnosed.(AU)

Síndrome de Gerstmann completo / Whole Gerstmann syndrome

El síndrome de Gerstmann es una rara enfermedad neurológica de causa vascular que afecta al lóbulo parietal. Se reporta el caso de un paciente masculino de 63 años con antecedentes patológicos personales de miocardiopatía dilatada. El 24 de enero de 2016 en horas de la mañana presentó de forma súbita pérdida de la conciencia con caída al suelo. Después de recuperarse tuvo estado confusional, desorientación topográfica de breve duración y dificultad para la expresión del lenguaje y la nominación de los objetos, por esta causa se decide su ingreso, durante el cual se realiza resonancia magnética simple de cráneo donde se observó imagen hipointensa en FLAIR en región parietal izquierda compatible con lesión isquémica. Se efectuó evaluación neuropsicológica encontrándose: agnosia digital, agrafia, acalculia y confusión derecha-izquierda. Se diagnostica un síndrome de Gerstmann completo(AU)

Gerstmann syndrome is a rare neurological disease of vascular cause that affects the parietal lobe. The case is reported of a 63-year-old male patient with a personal pathological history of dilated cardiomyopathy. In the morning of January 24, 2016, the patient presented sudden loss of consciousness with a fall to the ground. After recovering, he experienced a confusion state, short-lived topographical disorientation, and difficulty in expressing language and the naming of objects, a reason why his admittance was decided. During the admittance, simple magnetic resonance imaging to the skull was performed, which permitted to observe a hypo-intense image in FLAIR in the left parietal region and that was compatible with an ischemic lesion. A neuropsychological evaluation was performed: digital agnosia, agraphia, acalculia and confusion regarding right or left. A whole Gerstmann syndrome was diagnosed.(AU)

"With a smile through tears": the uprooted career of the man behind Gerstmann syndrome.

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.