He Gerstmann's syndrome Is a rare neurological and neuropsychological disorder in the general population (Deus, Espert and Navarro, 1996).
At the clinical level, this syndrome is defined by a classic set of symptoms consisting of digital agnosia, acalculia, agraphia and left-right spatial disorientation (Deus, Espert and Navarro, 1996).
Gerstmann's syndrome is associated with the presence of brain damage in posterior parieto-occipital areas (Benítez Yaser, 2006).
Numerous causes have been described at the etiological level, among which the Cerebrovascular accidents And tumor processes (Mazzoglio and Nabar, Muñiz, Algieri and Ferrante, 2016).
It is a neurological syndrome that is usually detected during the infantile stage, in the preschool or school phase (Mazzoglio and Nabar, Muñiz, Algieri and Ferrante, 2016).
Its diagnosis is eminently clinical and is based on the identification of classic symptomatology (Mazzoglio and Nabar, Muñiz, Algieri and Ferrante, 2016). The most common is to use a detailed neuropsychological evaluation of cognitive functions along with the use of various neuroimaging tests.
The medical intervention with this disorder is based on the treatment of the etiological cause and on the management of the secondary symptomatology. Generally speaking, Cognitive stimulation Early, Neuropsychological rehabilitation , Special education and speech therapy intervention.
A large number of clinical and experimental studies consider that Gerstmann syndrome tends to disappear with maturation and biological growth (Fournier del Castillo, García Peñas, Gutiérrez-Solana and Ruiz-Falcó Rojas, 200).
However, the most recent research indicates that most of the alterations persist over time (Fournier del Castillo, García Peñas, Gutiérrez-Solana and Ruiz-Falcó Rojas, 200).
Features of the Gerstmann's syndrome
The National Institute of Neurological Disorders and Stroke (2008) defines Gerstmann syndrome as a neurological disorder that results in the development of various cognitive alterations resulting from brain damage.
Disorders, pathologies and diseases classified within the group of neurologists are characterized by producing a wide variety of symptoms associated with an abnormal and deficient functioning of the nervous system.
Our nervous system is made up of different structures, such as brain , he cerebellum , the spinal cord Or the peripheral nerves. All these are essential to control and regulate each of the functions of our body (National Institutes of Health, 2015).
When various pathological factors disrupt its structure or normal functioning, a wide variety of signs and symptoms will appear: difficulty or inability to remember information, Alteration of consciousness , Difficulty communicating, difficulty moving, breathing, maintaining attention, etc. (National Institutes of Health, 2015).
In the case of Gerstmann syndrome, its clinical characteristics are associated with lesions in the Parietal brain areas , Related to sensation, perception and compression of sensory information (National Institute of Neurological Disorders and Stroke, 2008, National Organization for Rare Disorders, 2016).
Although it is usually related specifically to damage in the left region of the parietal lobe angular gyrus National Institute of Neurological Disorders and Stroke (2008).
This syndrome is usually associated with four basic signs and symptoms:
- Digital Agnosia : Difficulty or inability to recognize one's own or others' fingers.
- Acalculia : Alterations and disorders related to mathematical and computational skills.
- Agrafía: Alterations and disorders related to writing.
- Disorientation : Spatial disorientation related to a difficulty or inability to recognize right and left.
Despite its wide manifestations, this disorder was initially identified by Josef Gerstmann as a secondary condition to a brain injury (Deus, Espert and Navarro, 1996).
In her first clinical report she referred to a 52-year-old woman who was unable to recognize her own or another person's fingers. She had suffered a cerebrovascular accident and had no aphasic characteristics (Deus, Espert and Navarro, 1996).
He completed the picture of this syndrome through the analysis of new cases similar to the original one. Finally, in the year 1930, he was able to define it at the clinical level exhaustively (Deus, Espert and Navarro, 1996).
All of these descriptions refer mostly to adult patients suffering from lesions in the left parietal areas.
However, these were added to other pediatric populations, so this pathology has also been called the developmental Gerstmann syndrome (Roselli, Ardila and Matute, 2010).
About us
Gerstmann's syndrome is a rare neurological disorder in the general population (National Institute of Neurological Disorders and Stroke, 2008).
A very small number of cases have been described in the medical and experimental literature (National Institute of Neurological Disorders and Stroke, 2008).
No specific data on its prevalence and incidence are known worldwide (National Oganization for Rare Disorders, 2016).
As for the sociodemographic characteristics of Gerstmann syndrome, no significant association has been identified with sex, particular geographic regions or ethnic and racial groups.
Regarding the typical age of onset, Gerstmann syndrome predominates in the infantile phase, in the preschool or school phase (Benitez Yaser, 2006).
Signs and symptoms
Gerstmann's syndrome is characterized by four basic symptoms: digital agnosia, acalculia, agraphia and spatial disorientation (Deus, Espert and Navarro, 1996).
Agnosia Digital
Digital agnosia is considered the central sign of Gerstmann's syndrome. This term is used to refer to an inability to recognize the fingers of one's own hand or of another person (Fournier del Castillo, García Peñas, Gutiérrez-Solana, Ruiz-Falcó Rojas, 2000).
In its definition some important characteristics are included (Deus, Espert and Navarro, 1996; Gerstmann, 1927, 1985):
- It is not characterized as a type of digital weakness.
- It is not characterized as a difficulty or inability to identify fingers as such.
- It is defined as an absence or lack of ability to name, select, identify, recognize and orient the fingers at the individual level.
- It affects both the fingers themselves and those of other people.
- There are no other changes related to vision or denomination,
In most cases, digital agnosia is presented in a partial or specific way (Benitez Yaser, 2006).
The affected person can identify to a greater or lesser extent the fingers of the hand. However, it usually presents serious difficulties associated with the three fingers located in the area or middle area (Benitez Yaser, 2006).
It manifests bilaterally, that is, it affects both hands. It is also possible to identify cases of unilateral association.
In addition, patients are not aware of their errors (Lebrun, 2005), so a variable degree of Anosognosia .
Digital agnosia is one of the most prevalent signs in the early ages, along with left right discrimination and manual motility (Benitez Yaser, 2006).
When a specialist examines these types of alterations, the person usually presents a marked difficulty in pointing out and naming the fingers of the hand he has previously designated (Benitez Yaser, 2006).
Acalculia
With the term Acalculated We refer to the presence of various disorders related to math and calculus skills associated with acquired brain injury (Roselli, Ardila and Matute, 2010).
We must distinguish it from the term dyscalculia, used to refer to a set of numerical alterations associated with child development (Roselli, Ardila and Matute, 2010).
In Gerstmann's syndrome, another of the fundamental findings is the identification of a difficulty or inability to perform operations with numbers or arithmetic calculations (Deus, Espert and Navarro, 1996).
A large number of authors such as Botez, Botez, Oliver (1985), Lewinsky (1992) or Strub and Geschwind (1983) characterize acalculia as a loss of those skills or basic concepts related to the calculation previously acquired (Deus, Espert and Navarro , nineteen ninety six).
In many of the affected, this alteration is accompanied by other types of deficits (Mazzoglio and Nabar, Muñiz, Algieri and Ferrante, 2016):
- Difficulty or inability to maintain and follow an order.
- Difficulty or inability to design sequences.
- Difficulty or inability to manipulate numbers efficiently.
At the clinical level, the most common is that those affected are not able to perform written or mental calculations. In addition, they make various errors related to the interpretation or correct reading of mathematical signs (Mazzoglio and Nabar, Muñiz, Algieri and Ferrante, 2016).
The term agraph refers to the presence of an acquired disorder of writing skills and abilities (Roselli, Ardila and Matute, 2010).
It is defined as an acquired loss or alteration of written language resulting from a cortical brain injury (Deus, Espert and Navarro, 1996).
In Gerstmann's syndrome, agraphia can have different dimensions: praxis, linguistics or visuospatial (Mazzoglio and Nabar, Muñiz, Algieri and Ferrante, 2016).
Those affected present alterations associated with word dictation, spontaneous writing and copied writing (Mazzoglio and Nabar, Muñiz, Algieri and Ferrante, 2016).
In some cases the anatomy is related to problems of motor organization. Anomalies may occur in sensorimotor programs essential for the writing of individual letters or words (Mazzoglio and Nabar, Muñiz, Algieri and Ferrante, 2016).
At the clinical level, affected individuals have the following characteristics (Fournier del Castillo, García Peñas, Gutiérrez-Solana, Ruiz-Falcó Rojas, 2000):
- Difficulty in correctly performing the shape and stroke of the letters.
- Alterations in symmetry of letters.
- Persistent reductions in strokes.
- Alterations in the orientation of the lines.
- Use multiple fonts.
- Presence of invented characters.
Spatial disorientation
In Gerstmann's syndrome, an alteration of the orientation concept can be observed (Fournier del Castillo, García Peñas, Gutiérrez-Solana, Ruiz-Falcó Rojas, 2000):
It is frequent to identify a significant inability or difficulty to distinguish between left and right. This deficit is associated with both lateral and spatial orientation (Fournier del Castillo, García Peñas, Gutiérrez-Solana, Ruiz-Falcó Rojas, 2000).
At the clinical level, patients will not be able to name the right or left areas of different objects, body parts or spatial locations (Deus, Espert and Navarro, 1996).
Cause
The origin of Gerstmann syndrome is associated with lesions or cortical abnormalities located in the parietal lobe (Hayashi, Kinoshita, Furuta, Watanabe, Nakada and Hamada, 2013).
Approximately 95% of those affected by a complete clinical picture of Gerstmann's syndrome have left parietal lesions (Fournier del Castillo, García Peñas, Gutiérrez-Solana, Ruiz-Falcó Rojas, 2000).
Lesions may also extend to other posterior areas, such as Occipital regions (Fournier del Castillo, García Peñas, Gutiérrez-Solana, Ruiz-Falcó Rojas, 2000).
Among the triggers of this type of injury in Gerstmann syndrome are cerebrovascular accidents and the development of Brain tumors (Mazzoglio and Nabar, Muñiz, Algieri and Ferrante, 2016).
In adults, this syndrome is most commonly associated with episodes of ischemia or cerebrovascular haemorrhages (National Organization for Rare Disorders, 2016).
The number of diagnosed cases that have been linked to traumatic brain injury or the presence of brain tumors is reduced (National Organization for Rare Disorders, 2016).
Diagnosis
The suspicion of Gerstmann syndrome is usually evident in the presence of alterations related to orientation, calculation skills, writing ability or digital recognition.
Diagnosis is usually based on neurological and neuropsychological examination.
In the case of neurological assessment, it is important to identify the etiologic cause and the location of the lesion or brain lesions.
The most common is the use of imaging tests such as Computed tomography , the magnetic resonance wave Tractography .
In the neuropsychological examination of the affected person it is necessary to make an assessment of the cognitive capacities through clinical observation and the use of standardized tests.
Treatment
Like the diagnosis, the treatment of Gerstmann's syndrome has a neurological and a neuropsychological side.
In neurological intervention, medical approaches focus on the treatment of etiologic cause and possible complications. Standard procedures designed in cases of stroke or brain tumors are common.
Neuropsychological intervention usually employs an individualized and multidisciplinary intervention program. It is fundamental to work with the cognitive areas affected.
One of the most important objectives is for the patient to recover an optimal level of functioning, as close to the original. In addition, the generation of compensatory cognitive strategies is also essential.
In the child population, the use of special education and specific curricular programs is also beneficial.
References
- God, J., Espert, R., & Navarro, J. (1996). Gerstmann syndrome: current perspective. Behavioral Psychology , 417-436.
- Fournier del Castillo, C., García Peñas, J., Gutiérrez-Solana, L., & Ruiz-Falcó Rojas, M. (2000). Gerstmann syndrome in a 9-year-old male. Rev Neurol .
- Hayashi, Y., Kinoshita, M., Furuta, T., Watanabe, T., Nakada, M., & Hamada, J. (2013). Right upper longitudinal fasciculus: Implications for visuospatial neglect mimicking Gerstmann's syndrome. Clinical Neurology and Neurosurgery , 775-777.
- Lebrun, Y. (2005). Gerstmann's Syndrome. Journal of Neurolinguistics , 317-326.
- Mazzoglio and Nabar, M., Muñiz, M., Algieri, R., & Ferrante, M. (2016). Gerstmann syndrome: neuroanatomo-clinical correlate and differential semiological characteristics.
- NIH. (2008). Gerstmann Syndrome . Retrieved from the National Institute of Neurological Disorders and Stroke.
- NORD. (2016). Gerstmann Syndrome . Retrieved from the National Organization for Rare Disorders.
- Ramíerez Benitez, Y. (2006). Developmental Gerstann syndrome. Rev Mex Neuroci .
- Rosselli, M., Matute, E., & Ardila, A. (2010). Neuropsychology of Child Development.