Gerstmann's syndrome and unilateral optic ataxia in the emergency department / Síndrome de gerstmann e ataxia óptica unilateral na sala de emergência

ABSTRACT. A 75-year-old right-handed woman presented to the emergency department with simultanagnosia and right unilateral optic ataxia. Moreover, the patient had agraphia, acalculia, digital agnosia and right-left disorientation, consistent with complete Gerstmann's syndrome. This case highlights the concurrence of Gerstmann's syndrome and unilateral optic ataxia in the acute phase of a left middle cerebral artery stroke.

RESUMO. Uma paciente de 75 anos e destra se apresentou à sala de emergência com quadro de simultanagnosia e ataxia óptica unilateral à direita. Além disso, a paciente tinha agrafia, acalculia, agnosia digital e desorientação direita-esquerda, compatíveis com a síndrome de Gerstmann. O presente caso destaca a ocorrência simultânea da síndrome de Gerstmann com ataxia óptica unilateral na fase aguda do acidente vascular cerebral da artéria cerebral média esquerda.

Transient Gerstmann syndrome as manifestation of stroke: Case report and brief literature review / Síndrome de Gerstmann transitória como manifestação de acidente vascular encefálico: relato de caso e breve revisão de literatura

ABSTRACT Gerstmann Syndrome (GS) is a rare neurological condition described as a group of cognitive changes corresponding to a tetrad of symptoms comprising agraphia, acalculia, right-left disorientation and finger agnosia. It is known that some specific brain lesions may lead to such findings, particularly when there is impairment of the angular gyrus and adjacent structures. In addition, the possibility of disconnection syndrome should be considered in some cases. The purpose of this article is to report a case of a young, cardiac patient, non-adherent to treatment, who presented with a stroke in which transient clinical symptoms were compatible with the tetrad of GS. The case report is followed by a discussion and brief review of the relevant literature.

RESUMO A síndrome de Gerstmann (SG) é uma condição neurológica rara, caracterizada por um grupo de alterações cognitivas que correspondem a uma tétrade composta por agrafia, acalculia, desorientação direita-esquerda e agnosia para dedos. Sabe-se que certas lesões encefálicas específicas podem levar a tais achados, particularmente quando ocorre acometimento do giro angular e estruturas adjacentes. Além disso, a possibilidade de síndrome de desconexão deve ser considerada em alguns casos. O propósito deste artigo é relatar o caso de um paciente jovem cardiopata e não aderente ao tratamento que se apresentou com uma síndrome encéfalo-vascular associada a alterações clínicas transitórias compatíveis com a tétrade da SG. Este relato de caso é acompanhado de discussão e breve revisão de dados pertinentes da literatura.

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)

Progress in posterior cortical atrophy research / 复旦学报（医学版）

Posterior cortical atrophy (PCA) is a kind of neurodegenerative dementia,which is characterized by the progressive decline of visuoperceptual,visuospatial,reading and writing ability and praxic skills.Neuroimaging usually shows atrophy or metabolic decrease in posterior brain regions.The most common neuropathologic changes of PCA are amyloid plaques deposition and neurofibrillary tangles in posterior cortex.In general,PCA is considered as a variant form of Alzheimer's disease.

Clinical Progress of Gerstmann's Syndrome with Left Frontal Lobe Lesion: Two Cases

Gerstmann's syndrome, assigned to a lesion of the dominant parietal lobe, is a neurological disorder characterized by acalculia, agraphia, right-left disorientation and finger agnosia. Some studies report that these symptoms are also shown in other brain lesions. We report two patients who presented with this tetrad of symptoms in initial assessment. Their Brain MRI images both showed lesion of left frontal lobe. Over time, these symptoms became better but some still remained in last assessment. Accordingly, we suggest that a left frontal lesion cause Gerstmann's syndrome.

The Homogeneity of Phenomenology of Gerstmann Syndrome: The Study in Patients with Alzheimer's Disease

BACKGROUND: It remains unclear whether the four signs of Gerstmann syndrome are a cluster because the neuronal nets responsible for these symptoms are closer together, or because they shares a common networks. If the latter is correct, then with degenerative disorders such as Alzheimer's disease, each sign associated with Gerstmann syndrome should correlate with the other three signs more closely than they correlate with other cognitive dysfunctions. METHODS: Cluster and correlation analyses for various cognitive deficits including signs of Gerstmann syndrome were done among sixty-nine patients with probable Alzheimer's disease. RESULTS: The four signs of Gerstmann syndrome did not cluster together. With the exception of calculation and writing, other signs including right-left orientation and finger naming placed in other groups and did not significantly correlate each other. CONCLUSIONS: A detailed statistical analysis of the tetrad showed that Gerstmann syndrome was not attributable to a common neuronal network, and the phenomenological association of the four signs may be related to the anatomical proximity of the different networks mediating these functions.