Pure Gerstmann's syndrome from a focal lesion

It is controversial whether a focal lesion can specifically induce Gerstmann's syndrome (dyscalculia, left-right disorientation, finger agnosia, and agraphia). Also, Gerstmann's tetrad has been attributed to other cerebral symptoms, particularly aphasia. We examined a patient who had all four symptoms of Gerstmann's syndrome, without other symptoms or signs, and who had a discrete left parietal lesion.     

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.     

Developmental Gerstmann syndrome without aphasia in Fragile X syndrome

Considerable controversy surrounds Gerstmann's syndrome, with some authors questioning its localizing significance, and others doubting its existence as a distinct entity. This literature is reviewed, and a case is presented of a young boy with Fragile X syndrome who showed all five symptoms of the developmental Gerstmann syndrome. He was one of a group of individuals found to be cytogenetically positive for Fragile X syndrome, who were observed to possess three or more of Gerstmann's symptons in the complete absence of aphasic disorder.     

Alexia with agraphia in multiple sclerosis

Alexia with agraphia has not been reported in multiple sclerosis (MS), while aphasia is rare. This report documents these findings in a woman with relapsing MS. Associated features included dysmnesia, achromatopsia and elements of Gerstmann's syndrome. Cranial CT showed a large contrast-enhancing area of demyelination in the left temporo-parietal region.     

The Gerstmann syndrome in Alzheimer's disease

BACKGROUND: It remains unclear from lesion studies whether the four signs of the Gerstmann syndrome (finger agnosia, acalculia, agraphia, and right-left confusion) cluster because the neuronal nets that mediate these activities have anatomical proximity, or because these four functions share a common network. If there is a common network, with degeneration, as may occur in Alzheimer's disease, each of the signs associated with Gerstmann's syndrome should correlate with the other three signs more closely than they correlate with other cognitive deficits. METHODS: Thirty eight patients with probable Alzheimer's disease were included in a retrospective analysis of neuropsychological functions. RESULTS: The four Gerstmann's syndrome signs did not cluster together. Finger naming and calculations were not significantly correlated. Right-left knowledge and calculations also did not correlate. CONCLUSIONS: The four cognitive functions impaired in Gerstmann's syndrome do not share a common neuronal network, and their co-occurrence with dominant parietal lobe injuries may be related to the anatomical proximity of the different networks mediating these functions.     

Developmental Gerstmann's syndrome

The tetrad of finger agnosia, dysgraphia, dyscalculia, and right-left disorientation make up Gerstmann's syndrome. The tetrad has been infrequently described in children with learning disability and has been called developmental Gerstmann's syndrome (DGS). Developmental Gerstmann's syndrome may occur in brain-damaged and apparently normal children. Five children in whom DGS occurred in association with brain abnormalities underwent long-term observation, which indicated persistence of the deficits. The identification of these cases suggests that DGS may not be as rare as previously thought and may often be unrecognized. Testing for the Gerstmann elements in learning-disabled children may identify otherwise undiagnosed cases of DGS and should be routinely employed in the neurologic examination. Until appropriate teaching methods for DGS are found, "bypassing" the deficits and utilizing the child's strengths, plus counseling, seem to offer an effective treatment approach.     

Epilepsy and bilateral occipital calcifications: 3 cases

We report 3 cases of epilepsy with bilateral occipital calcifications followed up for several years. These cases were compared with 21 published cases and were found to differ from the classical Sturge-Weber syndrome on several points: 1) the disease appeared around the age of 5 years and consisted of focal epilepsy without neurological or mental disorders; 2) the epilepsy was easy to control during 2 to 5 years. This was followed by a diffuse encephalopathy with severe, treatment-resistant epilepsy, Gerstmann's syndrome, optic ataxia, cerebellar syndrome and slow activity at EEG. It appears from these 3 cases that: 1) occipital calcifications may be unilateral at the onset of the disease; 2) visual evoked potentials are affected at a late stage, and 3) CT scans are of considerable value in the prognosis of benign epilepsy in childhood.     

Gerstmann's syndrome (critical review of the literature)]

Serious doubts have been casted upon the validity of the Gerstmann's syndrome since the paper of Benton in 1961. On the other hand, the results of several recent works suggest that it could not be a mere artifact. The analysis of this results, and mostly the theoretical works of Luria on the physiology of higher cortical functions, make it possible to imagine a functional unity behind the heterogenous signs of this syndrom, and the other manifestations of left parieto-occipital lesions. The data collected in the litterature are discussed, with special reference to the theoretical implications of Gerstmann's syndrome.     

Ictal Gerstmann's syndrome in a patient with symptomatic parietal lobe epilepsy]

A 34-year-old man with astrocytoma in the left parietal lobe had symptomatic partial epilepsy, and he presented transient episodes of acalculia, agraphia and finger agnosia. Occasionally he had difficulty in finding appropriate letters when making an e-mail, and difficulty in writing and calculation. Neurological examinations revealed ictal symptoms of Gerstmann's syndrome without right to left disorientation. No other higher cortical dysfunction or neurological deficits were noted. Scalp EEGs showed frequent, regional ictal discharges in the left parietal area lasting for 60-240 seconds. These clinico-electrographical observations strongly support that epileptic seizures produced a loss of cortical higher function manifesting Gerstmann's syndrome.     

Posterior cortical atrophy. Two case reports and a review of the literature

We present two cases of progressive early-onset dementia with apraxia and visuospatial disability as initial manifestations. In the later stages of the illness Gerstmann's and Balint's syndromes developed. Structural neuroimaging demonstrated parieto-occipital atrophy and functional imaging revealed bilateral hypometabolism and hypoperfusion in these areas. These cases fulfil the diagnostic criteria of posterior cortical atrophy (PCA). Frontal lobe involvement became evident as the disease progressed. Alzheimer's disease also typically features this anterior spread and possibly this is the underlying pathological substrate for this clinical syndrome, although definite pathology is lacking. In this report, we describe longitudinal evolution in these two cases of PCA.     

Cortical sensory loss in a patient with posterior cortical atrophy: a case report

Patients with posterior cortical atrophy (PCA) who present with initial symptoms of higher visual function deficits eventually develop alexia, aphasia, and components of Balint's syndrome or Gerstmann's syndrome. Recently, pathological findings were reported for these patients that are generally suggestive of Alzheimer's disease even though Creutzfeldt-Jakob disease (CJD) was presumed as an alternative cause of some autopsy-diagnosed PCA cases. Here, we report a case with a four-year progression of cognitive and higher visual function deterioration, and with features not described in previously reported PCA cases (i.e., a distinct sensory complaint and early frontal lobe involvement). To summarize, this case belongs to perceptual-motor syndrome of asymmetric cortical degeneration and the underlying neuropathology is more suggestive of Alzheimer's disease than of Creutzfeldt-Jakob disease.     

Developmental Gerstmann's syndrome: a distinct clinical entity of learning disabilities

The symptom complex of finger anomia, right-left disorientation, dysgraphia, and dyscalculia constitutes Gerstmann's syndrome. It is mostly described in adults and is caused by acquired lesions of the dominant parietal lobe. It is infrequently described in children with learning disabilities and has been designated developmental Gerstmann's syndrome. Developmental Gerstmann's syndrome goes unnoticed if not specifically sought by clinicians. A detailed evaluation will reveal subtle neurologic deficits, behavioral problems, and neuropsychologic and specific speech and language abnormalities. Ten such patients are reported; six of the children demonstrated improvement with intensive speech training. Early identification and intervention is therefore crucial, and even more important in cultures in which students are required to be biliterate or triliterate, further increasing the constraints on writing. A selective writing, reading, or calculation abnormality in the presence of normal oral communication triggers several interesting possibilities for the brain mechanisms behind normal language processing. Similarly, the association of acalculia with finger anomia and agraphia with right-left disorientation may have specific implications in the neuropsychologic processing of the evolution of calculation and writing. A theoretical possibility of oral and written language processing from the observation of the language behavior of these children is also described.     

The writing of arabic numerals,kanji, and kana in brain-damaged patients

This study investigated the neural mechanisms involved in the writing of Arabic numerals, kanji, and kana. Tasks involving writing numerals in Arabic, kanji, and, kana were administered to four patients with Gerstmann's syndrome and to five Wernicke aphasics. The results indicated that the ability to write Arabic numerals was well preserved in the Wernicke aphasics despite their serious phonological disturbances. The patients with Gerstmann's syndrome, who have a deficit with the concept of number, could write kanji numerals better than Arabic and kana numerals. Unlike Arabic numerals (ideogram) and kana (syllabogram), kanji (morphogram) have both semantic and phonetic values. The results suggested that Arabic numerals may be somesthetic and linked directly to the concept of number bypassing phonological analysis.     

A study of right unilateral spatial neglect in left hemispheric lesions: the difference between right-handed and non-right-handed post-stroke patients

We report 20 cases of right unilateral spatial neglect caused by lesions in the left cerebral hemisphere. Differences in neuropsychological symptoms and lesions sites are discussed in connection with handedness. Of the right-handed patients, 6 had severe aphasia, 4 had Gerstmann's syndrome, and 1 had pure agraphia, but unilateral spatial neglect in these cases disappeared after a number of months. Six of the non-right-handed patients had moderate-to-severe aphasia, while the other 3 cases had no aphasia at all. Eight of the 9 cases in this group continued to have right unilateral spatial neglect for more than 6 months. Lesion site as determined by CT differed as to hemisphere, but all fell into the common area previously mentioned in connection with such disorders: i.e., the temporal, parietal and occipital lobes.     

Angular gyrus syndrome mimicking depressive pseudodementia

A 67-year-old left-handed woman with a diagnosis of pseudodementia was being treated for depression with little benefit. Neuropsychological evaluations revealed features of angular gyrus syndrome, namely, agraphia, alexia, Gerstmann's syndrome and behavioural manifestations such as depression, poor memory, frustration and irritability. A computed tomographic scan showed a right occipito-temporal infarction, which had occurred 18 months earlier. The patient demonstrated aspects of language dysfunction associated with the syndrome and showed reversed lateralization of cerebral functions. Recognizing and distinguishing between angular gyrus syndrome and depression is important because the appropriate therapies differ. The use of the term pseudodementia can be misleading.     

Left superior parietal cortex involvement in writing: integrating fMRI with lesion evidence

Writing is a uniquely human skill that we utilize nearly everyday. Lesion studies in patients with Gerstmann's syndrome have pointed to the parietal cortex as being critical for writing. Very little information is, however, available about the precise anatomical location of brain regions subserving writing in normal healthy individuals. In this study, we used functional magnetic resonance imaging (fMRI) to investigate parietal lobe function during writing to dictation. Significant clusters of activation were observed in left superior parietal lobe (SPL) and the dorsal aspects of the inferior parietal cortex (IPC) bordering the SPL. Localized clusters of activation were also observed in the left premotor cortex, sensorimotor cortex and supplementary motor area. No activation cluster was observed in the right hemisphere. These results clearly indicate that writing appears to be primarily organized in the language-dominant hemisphere. Further analysis revealed that within the parietal cortex, activation was significantly greater in the left SPL, compared to left IPC. Together with lesion studies, findings from the present study provide further evidence for the essential role of the left SPL in writing. Deficits to the precise left hemisphere parietal cortex regions identified in the present study may specifically underlie disorders of writing observed in Gerstmann's syndrome and apractic agraphia.     

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.     

Right hemiplegia and spatial neglect with apraxia and agraphia without aphasia in a right-handed patient

A 65 year-old right-handed woman was admitted after the sudden onset of a right dense hemiplegia. C.T. showed a large left infarction in the middle cerebral artery territory. There was a slight anosognosia and neglect of the right space without confusion. She had aprosodia but no aphasia. On the other hand, there was a severe apraxia and all the components of Gerstmann's syndrome were present. This suggests an unusual sattering of hemispheric functional dominances.     

Optic ataxia: clinical-radiological correlations with the EMIscan.

After coronary by-pass surgery, a 47 year old, right-handed man developed Gerstmann's syndrome, a visual-spatial perceptual deficit, and a gross impairment of movememt under visual guidance ("optic ataxia"). Visual fields and extraocular movements were intact; he had a left hemiparesis. The EMIscan showed three lesions: a left parietal-occipital lesion; a posterior callosal lesion, and a right frontal lesion. It is hypothesized that optic ataxia in both visual fields requires bilateral lesions which, in the present case, were strategically placed so as to effectively disconnect motor cortex from visual input.