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)

Gerstmann's Syndrome Associated with Right Parietal Hemorrhage and Arteriovenous Malformation

No abstract available.

Posterior Type of Alzheimer's Dementia Presenting with Homonymous Hemianopsia

BACKGROUND: Alzheimer's disease is a chronic neurodegenerative condition, mostly affecting the medial temporal lobe and associated neocortical structures. In this report, we present a rare clinical manifestation of this disease. CASE REPORT: A 61-year-old female with word finding difficulty and memory disturbances was diagnosed with Alzheimer's disease. Two years later, she complained of right homonymous hemianopia without optic ataxia, ocular apraxia, and simultagnosia. No findings other than parenchymal disease were apparent in magnetic resonance imaging and laboratory tests. CONCLUSIONS: In this case, in a patient initially diagnosed with Alzheimer's dementia with progressive disease, we found only homonymous hemianopia, without signs of Balint's syndrome or Gerstmann's syndrome. After careful investigation showing that Alzheimer's dementia with visual symptom was not associated with parenchymal disease, we concluded a case of atypical variant of Alzheimer's disease.

Endovascular Treatment of Giant Serpentine Aneurysm of the Middle Cerebral Artery

Giant serpentine aneurysms are uncommon types of aneurysmal disease and have angiographically authentic features. We report a case of a 44-year-old male with headache and seizure. He presented a giant serpentine aneurysm arising from the middle cerebral artery (MCA). It was a large intracranial aneurysm thrombosed as a mass-like lesion while it maintained its outflow drainage into the distal MCA branches. The balloon occlusion test (BOT) was performed to test the tolerance of temporary collateral circulation. Following routine cerebral angiography, we performed an endovascular embolization on the proximal artery of MCA. He was discharged from the hospital with alert mental status and mild Gerstmann syndrome. The short-term follow-up imaging studies showed the decreased mass effect, and the patient presented an improved Gerstmann syndrome. After a careful evaluation of BOT, an endovascular embolization can be one of the powerful therapeutic instruments for giant serpentine aneurysm.

Posterior Cortical Atrophy with Acute Onset and Rapid Progressive Visual Symptoms: A Case Report

BACKGROUND: Posterior cortical atrophy (PCA) is characterized by slowly progressive early onset dementia with cortical visual dysfunction and disproportionate atrophy of the posterior cortex. CASE REPORT: A 55-year-old right-handed woman developed visuo-spatial impairments that progressed rapidly into cortical blindness over the following 3 months. Neuro-psychological evaluation revealed Gerstmann syndrome and severe constructional impairments with all components of Balint syndrome. However, her memory, insight, and judgment were preserved. Her brain MRI was normal. However, 18F fluorodeoxyglucose positron emission tomography revealed a marked hypometabolism in the bilateral parieto-occipital region. CONCLUSIONS: Although rapid progression of visuo-spatial dysfunction without memory impairment occurred, we considered PCA as well.

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.

A case of Posterior Cortical Atrophy Presenting with Features of Atypical Dementia

Posterior cortical atrophy(PCA) is a presenile dementia that presents primarily with signs and symptoms of cortical visual dysfunction, while memory is relatively preserved until the late stage of the disease. We report a patient with PCA, confirmed by brain magnetic resonance imaging (MRI) and F18-fluorodeoxyglucose positron emission tomography(FDG PET). A 58-year-old right-handed woman presented initially with visual dimness and difficulty finding things around her. She had partial Balint's syndrome, partial Gerstmann syndrome, and idiomotor apraxia. She also had a mild memory disturbance, but preserved insight of her disease. Neuropsychological evaluation showed decreased parietal and left temporal functions bilaterally. Brain MRI and F18-FDG PET revealed typical bilateral occipitoparietal atrophy and hypometabolism, which were slightly worse on the right side. Cholinesterase inhibitor administration for 6 months improved the memory impairment slightly, but not the cortical visual dysfunction. This is a typical case of PCA, confirmed by neurologic signs and imaging findings.

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.

Cerebral Amyloid Angiopathy: A Case Report

Symptomatic cerebral amyloid angiopathy (CAA) is characterized by lobar cerebral hemorrhage, transient neurological symptoms, and dementia with leukoencephalopathy in the absence of hypertension or coagulopathy. Contrary to its common appearance in English literature, pathologically confirmed CAA has rarely been reported in Korea. A 77-year-old normotensive, nondemented woman was admitted for a case of incidentally found intracerebral lobar hemorrhage at the left occipito-parietal area. On neurological examination, she displayed symptoms of a drowsy mentality and Gerstmann syndrome. Light-microscopically, medium to large cortical and meningeal vessels appeared to be thickened with amorphous glassy materials showing apple-green birefringence under polarized light on Congo red stain, which is consistent with CAA. The amyloid deposits were detected in the medium-sized vessels of the leptomeninges and cerebral cortex, but not in the capillaries and small arterioles in the white matter. Electron microscopy revealed amyloid fibrils and basal lamina duplication, indicating damaged vessels. Careful cortical biopsy during evacuating hematoma for histopathologic and ultrastructural examination is essential for diagnosis of CAA in case of clinical probability because the ignored sampling procedure and sampling error of patchy, segmental distribution of affected vessels might be ascribed to rare incidence of CAA in Korea.

Clinical analysis of 7 patients with Gerstmann syndrome secondary to cerebral vascular disease / 中国医学科学院学报

<p><b>OBJECTIVE</b>To analyze clinical features of patients with Gerstmann syndrome (GS).</p><p><b>METHODS</b>We retrospectively analysed the clinical manifestations of 7 patients (6 men and 1 woman) with GS secondary to cerebral vascular diseases and reviewed the literatures.</p><p><b>RESULTS</b>The age ranged from 51 to 70 years with a mean of 70 years. They all had sudden onset and the tetrad of GS-finger agnosia, left-right disorientation, agraphia and acalculia, 3 patients accompanied by incomplete aphasia, 3 by anomic aphasia, 2 by alexia and 1 by constructional apraxia. Cranial computed tomographic scan showed low-density focus of the left parietal lobe in 6 cases and high-density focus of the left parietal lobe in 1 case.</p><p><b>CONCLUSION</b>GS has the high value in localization and the lesion is mainly localized to angular gyrus of the dominant hemisphere.</p>

Bedside Evaluation of Neurobehavioral Disorders

This article provides clinical practice guidelines for neurologists in the assessment of neurobehavioral and neurocognitive deficits. It has been known that cognitive functions lie mainly in the cortex and that different cortical areas mediate different cognitive functions. Therefore, the evaluation of cognitive and neurobehavioral symptoms helps localize these symptoms. This article describes the bedside history taking and clinical examinations for patients with neurobehavioral and cognitive dysfunctions. It includes the assessment of aphasia, apraxia, Gerstmann syndrome, visuospatial dysfunctions, neglect syndrome, memory disturbances, and frontal lobe dysfunctions. This guideline will offer better approaches to patients with cognitive deficits and neurobehavioral problems.

A Case of Alzheimer's Disease Manifesting as Posterior Cortical Atrophy

A subgroup of patients with progressive degenerative dementia that begins with higher visual dysfunction has been referred to as posterior cortical atrophy (PCA). A 55-year-old woman presented with progressive visual disturbance for 4 years, which was followed by geographical disorientation, impairment of writing and calculation, and memory distur-bance. Neuropsychological deficits were characterized by Balint syndrome, visuospatial dysfunction, prosopagnosia, Gerstmann syndrome and apraxia. Brain MRI showed mild diffuse atrophy. F18-FDG-PET disclosed bilateral occipi-totemporoparietal hypometabolism, more pronounced on the right. Biopsy from right temporal lobe revealed neu-ropathological findings consistent with Alzheimer's disease.

A Case of Alzheimer's Disease Manifesting as Posterior Cortical Atrophy

A subgroup of patients with progressive degenerative dementia that begins with higher visual dysfunction has been referred to as posterior cortical atrophy (PCA). A 55-year-old woman presented with progressive visual disturbance for 4 years, which was followed by geographical disorientation, impairment of writing and calculation, and memory distur-bance. Neuropsychological deficits were characterized by Balint syndrome, visuospatial dysfunction, prosopagnosia, Gerstmann syndrome and apraxia. Brain MRI showed mild diffuse atrophy. F18-FDG-PET disclosed bilateral occipi-totemporoparietal hypometabolism, more pronounced on the right. Biopsy from right temporal lobe revealed neu-ropathological findings consistent with Alzheimer's disease.

Agrafia lexical en demencia de tipo Alzheimer / Lexical agraphia in dementia of the Alzheimer type

Se presenta el caso de una paciente con demencia de tipo Alzheimer con defecto mnésico, apraxias, afasia sensorial y un síndrome de Gerstman. En este último destaca una agrafia lingüística de tipo lexical, con respeto relativo en el dictado de logotomas. Los autores interpretan estas observaciones como una manifestación de un mayor compromiso del girus angular, sustrato del sistema de imágenes visuales de las palabras, y un respeto relativo del girus supramarginalis, que sería una zona crucial para la conversión de fonemas en grafemas

Traumatic Gerstmann Syndrome: Report of a Case

Gerstmann syndrome that was caused by a traumatic origin was very rare. We have reported a case of typical Gerstmann syndrome which was caused by a localized head injury. The case showed finger agnosia, right-left disorientation, acalculia and dysgraphia. And also the case revealed amnesic dysphasia and autotopagnosia. The lesion was an egg-shell typed depressed fracture(4 cm in diameter) of the left inferior parietal bone associated with the intracerebral hematoma in the lower parietal lobe which seemed to be the transitional area of the angular gyrus and the second occipital convolution.