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PubMed2004

What ever happened to developmental Gerstmann's syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes.

Developmental Gerstmann's syndrome is a neurodevelopmental disorder infrequently described in the literature. The limited literature might result from controversy surrounding developmental Gerstmann's syndrome as a "true syndrome." Developmental Gerstmann's syndrome requires a tetrad of symptoms: left-right confusion, finger agnosia, dyscalculia, and dysgraphia, with constructional dyspraxia often included as a fifth symptom. The etiology of developmental Gerstmann's syndrome is unknown, but several hypotheses have been proposed, and none have been conclusively confirmed. Based on the paucity of recent research on developmental Gerstmann's syndrome, individuals who meet the criteria for the disorder could be given other diagnoses. A clustering of neuropsychologic features across other seemingly related disorders suggests that the conceptualization of the tetrad of symptoms traditionally associated with developmental Gerstmann's syndrome more appropriately reflects soft signs that are commonly associated with a number of other neurodevelopmental disorders. Thus, although developmental Gerstmann's syndrome is of historical interest to neurodevelopmental specialists, there appears to be no basis for considering this disorder as a unique syndrome.

Diagnosis, Differential↗
PubMed1998

Progressive multifocal leukoencephalopathy in a patient with acquired immunodeficiency syndrome (AIDS) manifesting Gerstmann's syndrome.

We reported a case of acquired immunodeficiency syndrome (AIDS) via multiple blood transfusions, who manifested progressive multifocal leukoencephalopathy (PML) about 18 months after the development of AIDS. PML initiated with right hemiparesis, dysphasia, and Gerstmann's syndrome and resulted in death within 2 months after the onset. Neuroimaging examinations revealed white matter lesions mainly in the left posterior parietal lobe. The cortical gray matter also showed abnormal signal intensity. Peripheral CD4+ lymphocyte count was 81/microl. Routine cerebrospinal fluid (CSF) examinations were negative. CSF antibodies against herpes simplex virus, varicella-zoster virus, cytomegalovirus, Epstein-Barr virus as well as serum antibody against toxoplasma gondii were negative. Though autopsy or biopsy of the brain was not performed, JC virus genomes were detected in the CSF sample by a polymerase chain reaction, and their sequencing showed unique alterations of the regulatory regions, characteristic to PML-type JC virus.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome.

Gerstmann-Sträussler syndrome is a rare familial neurodegenerative condition that is vertically transmitted, in an apparently autosomal dominant way. It can also be horizontally transmitted to non-human primates and rodents through intracerebral inoculation of brain homogenates from patients with the disease. The exact incidence of the syndrome is unknown but is estimated to be between one and ten per hundred million. Patients initially suffer from ataxia or dementia and deteriorate until they die, in one to ten years. Protease-resistant prion protein (PrP) and PrP-immunoreactive amyloid plaques with characteristic morphology accumulate in the brains of these patients. Current diagnostic criteria for Gerstmann-Sträussler syndrome incorporate clinical and neuropathological features, as animal transmission studies can be unreliable. PrP is implicated in the pathogenesis and transmission of the condition and in scrapie, an equivalent animal disease. It was discovered by enriching scrapie-infected hamster brain fractions for infectivity. Because there is compelling evidence that the scrapie isoform of PrP is a necessary component of the infectious particle, it seemed possible that the PrP gene on the short arm of human chromosome 20 in Gerstmann-Sträussler syndrome might be abnormal. We show here that PrP codon 102 is linked to the putative gene for the syndrome in two pedigrees, providing the best evidence to date that this familial condition is inherited despite also being infectious, and that substitution of leucine for proline at PrP codon 102 may lead to the development of Gerstmann-Sträussler syndrome.

Chromosome Mapping↗
PubMed1996

Gerstmann's syndrome.

Although Gerstmann's syndrome has been well documented since it was characterised in the latter half of last century, there has not been much literature on it in the last few years. We present a classical case in a patient who was admitted into hospital for an unrelated problem. We conclude that clinical examination still has a valuable role in neurology, despite the availability of excellent imaging techniques.

Gerstmann Syndrome↗
PubMedDate not supplied

[Gerstmann-Straussler syndrome clinical and neuromorphofunctional diagnosis: a case report].

UNLABELLED: The Syndrome of Gerstmann-Straussler is a constellation of typical symptoms that affects the parietotemporal crossroad and also involves the contiguous parts of the occipital lobe of the dominant hemisphere. Is a family ailment that starts with an espinocerebellar ataxy followed by dementia. Diagnosis options such as EEG, TC, RM and brain SPECT-99mTc-HMPAO have been carried out in an adult with Gerstmann Syndrome. RESULTS: The brain SPECT disclosed a disminution of the flow at the parietotemporooccipital areas of both hemispheres. The results revealed a clinical and a neuromorphofunctional correlation. CONCLUSION: Brain SPECT is a complementary test to the structural neuroradiological examinations, such as TC and RM, that occasionally is useful not only to clarify but also to widen the information that these offer.

Gerstmann-Straussler-Scheinker Disease↗
PubMedDate not supplied

[Gerstmann's syndrome: report of a case].

In recent years, several authors have questioned the reliaty of Gerstmann syndrome and have claimed that "aphasia might be regarded as the true Grundstörung of the Gerstmann symptoms". This paper describes a case of Gerstmann syndrome ictally risen with aphasia in a patient suffering from a tumour of the left PTO region. After the remission of aphasia, the syndromic association typical of the Gerstmann syndrome lasted up the exitus.

Aphasia↗
PubMedDate not supplied

[Increased level of Gerstmann's syndrome secondary to thalamic hematoma].

A patient developed enlarged Gerstmann syndrome after left thalamic haematoma. Single photon emission computerised tomography (SPECT) showed left parietal-temporal cortical hypocapture. These findings would lead us to believe that the clinical picture of our patient was due to a thalamic-cortical diaschitic phenomenon.

Child↗
PubMed1995

Right-left confusion in Gerstmann's syndrome: a model of body centered spatial orientation.

Gerstmann's syndrome encompasses the tetrad of finger agnosia, agraphia, acalculia and right-left confusion and is associated with lesions of the dominant angular gyrus. The localizing value of this syndrome has been questioned because multiple mechanisms can account for each of the components of the syndrome. We present the case of a man who developed Gerstmann's syndrome following a focal infarct of the left angular gyrus. The patient's right-left confusion could not be accounted for by either an aphasia or a degraded body schema. A series of experiments that investigated the patient's spatial mapping system by progressively restricting the degrees of freedom for spatial rotation revealed an isolated defect in deriving the relative position of an object along the horizontal axis. Defective horizontal mapping can account for the other components of Gerstmann's syndrome because they all share a common dependency on relative horizontal positioning.

Aged↗
PubMed1992

Gerstmann's syndrome.

Recent case reports describe the occurrence of a more or less pure Gerstmann syndrome in association with a focal lesion in the posterior perisylvian territory of the brain's left hemisphere. In addition, an electrocortical stimulation study reported the Gerstmann symptom combination and a number of other symptom combinations on stimulation of small areas in the left posterior parietotemporal cortex. The neuropsychological implications of these and other recent findings are considered in light of the variety of "syndromes" produced by lesions in this region, the rare occurrence of Gerstmann's syndrome, and its appearance as a consequence of lesions in diverse cerebral areas.

Adolescent↗