Improving the DSM-5 approach to cognitive impairment: Developmental prosopagnosia reveals the need for tailored diagnoses.

The Diagnostic Statistical Manual of Mental Disorders (DSM-5) recommends diagnosing neurocognitive disorders (i.e., cognitive impairment) when a patient scores beyond - 1 SD below neurotypical norms on two tests. I review how this approach will fail due to cognitive tests' power limitations, validity issues, imperfect reliabilities, and biases, before summarizing their resulting negative consequences. As a proof of concept, I use developmental prosopagnosia, a condition characterized by difficulties recognizing faces, to show the DSM-5 only diagnoses 62-70% (n1 = 61, n2 = 165) versus 100% (n1 = 61) through symptoms alone. Pooling the DSM-5 missed cases confirmed the presence of group-level impairments on objective tests, which were further evidenced through meta-analyses, thus validating their highly atypical symptoms. These findings support a paradigm shift towards bespoke diagnostic approaches for distinct cognitive impairments, including a symptom-based method when validated effective. I reject dogmatic adherence to the DSM-5 approach to neurocognitive disorders, and underscore the importance of a data driven, transdiagnostic approach to understanding patients' subjective cognitive impairments. This will ultimately benefit patients, their families, clinicians, and scientific progress.

Normative data of the Italian Famous Face Test.

The faces we see in daily life exist on a continuum of familiarity, ranging from personally familiar to famous to unfamiliar faces. Thus, when assessing face recognition abilities, adequate evaluation measures should be employed to discriminate between each of these processes and their relative impairments. We here developed the Italian Famous Face Test (IT-FFT), a novel assessment tool for famous face recognition in typical and clinical populations. Normative data on a large sample (N = 436) of Italian individuals were collected, assessing both familiarity (d') and recognition accuracy. Furthermore, this study explored whether individuals possess insights into their overall face recognition skills by correlating the Prosopagnosia Index-20 (PI-20) with the IT-FFT; a negative correlation between these measures suggests that people have a moderate insight into their face recognition skills. Overall, our study provides the first online-based Italian test for famous faces (IT-FFT), a test that could be used alongside other standard tests of face recognition because it complements them by evaluating real-world face familiarity, providing a more comprehensive assessment of face recognition abilities. Testing different aspects of face recognition is crucial for understanding both typical and atypical face recognition.

Relationship between face recognition ability and anxiety tendencies in healthy young individuals: A prosopagnosia index and state-trait anxiety inventory study.

Developmental prosopagnosia (DP) is a condition that indicates the inability to recognize individuals by their faces from birth, without any history of brain damage. The assessment of face recognition ability and diagnosis of DP involve the use of face tests such as the Cambridge Face Memory Test (CFMT) and the Cambridge Face Perception Test, along with self-reported measures like the 20-Item Prosopagnosia Index (PI20). Face recognition accuracy is affected by anxiety. However, previous studies on the relationship between face recognition ability and anxiety have not used the PI20 measure. This study aimed to investigate the relationship between self-reported measures of face recognition ability and anxiety tendencies among healthy young individuals for DP diagnosis and its implications. We used a face recognition test, involving the PI20, CFMT, Visual Perception Test for Agnosia-Famous Face Test (VPTA-FFT), and State-Trait Anxiety Inventory (STAI). We assessed the performance of 116 Japanese young adults (75 females, median age of 20.7 years, with a standard deviation of 1.2). Subsequently, we conducted a statistical analysis to examine the relationship between the outcomes of the face recognition tests and STAI scores using Pearson correlation analysis and single correlation coefficients. The results showed a positive correlation between state anxiety and PI20 (r = 0.308, p = 0.007), and a weak positive correlation was also observed between trait anxiety and PI20 (r = 0.268, p = 0.04). In contrast, there was no correlation between CFMT and VPTA-FFT with respect to STAI. The results of the hierarchical multiple regression analysis also suggested that the correlation between the performance on the PI20 (self-report) and objective measures of face recognition performance (the CFMT and the VPTA-FFT) are driven by differences in anxiety. This study is the first to explore the relationship between face recognition abilities and anxiety using the PI20 self-report measure. There are implications for future research on the diagnosis of DP and the relationship between anxiety and face recognition.

Both identity and non-identity face perception tasks predict developmental prosopagnosia and face recognition ability.

Developmental prosopagnosia (DP) is characterised by deficits in face identification. However, there is debate about whether these deficits are primarily perceptual, and whether they extend to other face processing tasks (e.g., identifying emotion, age, and gender; detecting faces in scenes). In this study, 30 participants with DP and 75 controls completed a battery of eight tasks assessing four domains of face perception (identity; emotion; age and gender; face detection). The DP group performed worse than the control group on both identity perception tasks, and one task from each other domain. Both identity perception tests uniquely predicted DP/control group membership, and performance on two measures of face memory. These findings suggest that deficits in DP may arise from issues with face perception. Some non-identity tasks also predicted DP/control group membership and face memory, even when face identity perception was accounted for. Gender perception and speed of face detection consistently predicted unique variance in group membership and face memory; several other tasks were only associated with some measures of face recognition ability. These findings indicate that face perception deficits in DP may extend beyond identity perception. However, the associations between tasks may also reflect subtle aspects of task demands or stimuli.

A new way of classifying developmental prosopagnosia: Balanced Integration Score.

Despite severe everyday problems recognising faces, some individuals with developmental prosopagnosia (DP) can achieve typical accuracy scores on laboratory face recognition tests. To address this, studies sometimes also examine response times (RTs), which tend to be longer in DPs relative to control participants. In the present study, 24 potential (according to self-report) DPs and 110 age-matched controls completed the Cambridge Face and Bicycle Memory Tests, old new faces task, and a famous faces test. We used accuracy and the Balanced Integration Score (BIS), a measure that adjusts accuracy for RTs, to classify our sample at the group and individual levels. Subjective face recognition ability was assessed using the PI20 questionnaire and semi structured interviews. Fifteen DPs showed a major impairment using BIS compared with only five using accuracy alone. Logistic regression showed that a model incorporating the BIS measures was the most sensitive for classifying DP and showed highest area under the curve (AUC). Furthermore, larger between-group effect sizes were observed for a derived global (averaged) memory measure calculated using BIS versus accuracy alone. BIS is thus an extremely sensitive novel measure for attenuating speed-accuracy trade-offs that can otherwise mask impairment measured only by accuracy in DP.

Are people with autism prosopagnosic?

Difficulties in various face processing tasks have been well documented in autism spectrum disorder (ASD). Several meta-analyses and numerous case-control studies have indicated that this population experiences a moderate degree of impairment, with a small percentage of studies failing to detect any impairment. One possible account of this mixed pattern of findings is heterogeneity in face processing abilities stemming from the presence of a subpopulation of prosopagnosic individuals with ASD alongside those with normal face processing skills. Samples randomly drawn from such a population, especially relatively smaller ones, would vary in the proportion of participants with prosopagnosia, resulting in a wide range of group-level deficits from mild (or none) to severe across studies. We test this prosopagnosic subpopulation hypothesis by examining three groups of participants: adults with ASD, adults with developmental prosopagnosia (DP), and a comparison group. Our results show that the prosopagnosic subpopulation hypothesis does not account for the face impairments in the broader autism spectrum. ASD observers show a continuous and graded, rather than categorical, heterogeneity that span a range of face processing skills including many with mild to moderate deficits, inconsistent with a prosopagnosic subtype account. We suggest that pathogenic origins of face deficits for at least some with ASD differ from those of DP.

Persistent prosopagnosia following COVID-19.

COVID-19 can cause psychological problems including loss of smell and taste, long-lasting memory, speech, and language impairments, and psychosis. Here, we provide the first report of prosopagnosia following symptoms consistent with COVID-19. Annie is a 28-year-old woman who had normal face recognition prior to contracting COVID-19 in March 2020. Two months later, she noticed face recognition difficulties while experiencing symptom relapses and her deficits with faces have persisted. On two tests of familiar face recognition and two tests of unfamiliar face recognition, Annie showed clear impairments. In contrast, she scored normally on tests assessing face detection, face identity perception, object recognition, scene recognition, and non-visual memory. Navigational deficits frequently co-occur with prosopagnosia, and Annie reports that her navigational abilities are substantially worse than before she became ill. Self-report survey data from 54 respondents with long COVID showed that a majority reported reductions in visual recognition and navigation abilities. In summary, Annie's results indicate that COVID-19 can produce severe and selective neuropsychological impairments similar to deficits seen following brain damage, and it appears that high-level visual impairments are not uncommon in people with long COVID.

What is the prevalence of developmental prosopagnosia? An empirical assessment of different diagnostic cutoffs.

The prevalence of developmental prosopagnosia (DP), lifelong face recognition deficits, is widely reported to be 2-2.5%. However, DP has been diagnosed in different ways across studies, resulting in differing prevalence rates. In the current investigation, we estimated the range of DP prevalence by administering well-validated objective and subjective face recognition measures to an unselected web-based sample of 3116 18-55 year-olds and applying DP diagnostic cutoffs from the last 14 years. We found estimated prevalence rates ranged from .64-5.42% when using a z-score approach and .13-2.95% when using a percentile approach, with the most commonly used cutoffs by researchers having a prevalence rate of .93% (z-score, .45% when using percentiles). We next used multiple cluster analyses to examine whether there was a natural grouping of poorer face recognizers but failed to find consistent grouping beyond those with generally above versus below average face recognition. Lastly, we investigated whether DP studies with more relaxed diagnostic cutoffs were associated with better performance on the Cambridge Face Perception Test. In a sample of 43 studies, there was a weak nonsignificant association between greater diagnostic strictness and better DP face perception accuracy (Kendall's tau-b correlation, τb =.18 z-score; τb = .11 percentiles). Together, these results suggest that researchers have used more conservative DP diagnostic cutoffs than the widely reported 2-2.5% prevalence. We discuss the strengths and weaknesses of using more inclusive cutoffs, such as identifying mild and major forms of DP based on DSM-5.

Preference for horizontal information in faces predicts typical variations in face recognition but is not impaired in developmental prosopagnosia.

Face recognition is strongly influenced by the processing of orientation structure in the face image. Faces are much easier to recognize when they are filtered to include only horizontally oriented information compared with vertically oriented information. Here, we investigate whether preferences for horizontal information in faces are related to face recognition abilities in a typical sample (Experiment 1), and whether such preferences are lacking in people with developmental prosopagnosia (DP; Experiment 2). Experiment 1 shows that preferences for horizontal face information are linked to face recognition abilities in a typical sample, with weak evidence of face-selective contributions. Experiment 2 shows that preferences for horizontal face information are comparable in control and DP groups. Our study suggests that preferences for horizontal face information are related to variations in face recognition abilities in the typical range, and that these preferences are not aberrant in DP.

A new approach to diagnosing and researching developmental prosopagnosia: Excluded cases are impaired too.

Developmental prosopagnosia is characterized by severe, lifelong difficulties when recognizing facial identity. Unfortunately, the most common diagnostic assessment (Cambridge Face Memory Test) misses 50-65% of individuals who believe that they have this condition. This results in such excluded cases' absence from scientific knowledge, effect sizes of impairment potentially overestimated, treatment efficacy underrated, and may elicit in them a negative experience of research. To estimate their symptomology and group-level impairments in face processing, we recruited a large cohort who believes that they have prosopagnosia. Matching prior reports, 56% did not meet criteria on the Cambridge Face Memory Test. However, the severity of their prosopagnosia symptoms and holistic perception deficits were comparable to those who did meet criteria. Excluded cases also exhibited face perception and memory impairments that were roughly one standard deviation below neurotypical norms, indicating the presence of objective problems. As the prosopagnosia index correctly classified virtually every case, we propose it should be the primary method for providing a diagnosis, prior to subtype categorization. We present researchers with a plan on how they can analyze these excluded prosopagnosia cases in their future work without negatively impacting their traditional findings. We anticipate such inclusion will enhance scientific knowledge, more accurately estimate effect sizes of impairments and treatments, and identify commonalities and distinctions between these different forms of prosopagnosia. Owing to their atypicalities in visual perception, we recommend that the prosopagnosia index should be used to screen out potential prosopagnosia cases from broader vision research.

Prosopamnesia: a case report of amnesia for faces.

Prosopamnesia is a face-selective memory disorder in which face learning is impaired, while face-perception disorder (prosopagnosia) and memory disorders for stimuli other than faces are not present. To date, only two cases of prosopamnesia have been reported in adults - one congenital and one secondary to brain damage. This article reports a case of a 68-year-old woman complaining difficulties recognizing persons she had got to know recently. Neuropsychological examination revealed face-specific anterograde amnesia in the absence of prosopagnosia and other memory impairments. Brain MRI did not present any focal abnormality; PET-scan revealed hypoactivation mostly in the frontotemporal area bilaterally. This patient represents the first case of late-onset primary prosopamnesia.

Right Fusiform Gyrus Infarct with Acute Prosopagnosia.

A 56-year-old, right-handed man with no known past medical history presented with sudden onset of inability to recognize familiar individuals in person, including his wife and his mother. He also couldn't recognize himself in the mirror. There was no weakness, numbness, visual disturbances, or speech difficulty. Face recognition test, using Warrington Recognition Memory Test (1), showed the presence of complete prosopagnosia. The rest of the neurological and cranial nerves examinations were normal. Magnetic resonance imaging (MRI) of the brain showed restricted diffusion at the right temporal and occipital lobes (the fusiform gyrus) [Figure 1]. Magnetic resonance angiogram (MRA) of the brain was unremarkable. The 24-hours Holter monitoring showed paroxysmal atrial fibrillation. The transthoracic echocardiogram and carotid doppler ultrasound scan were normal. He was then treated with rivaroxaban 20mg daily for secondary stroke prevention in non-valvular atrial fibrillation. Face recognition skill training was started in the ward, which includes compensatory strategies to achieve person recognition by circumventing the face processing impairment, and remediation to enhance mnemonic function for face recognition. His prosopagnosia resolved completely after one week. Prosopagnosia, also known as face blindness, is an impairment in recognizing faces. The core defects are the loss of familiarity with previously known faces and the inability to recognize new faces. Patients with prosopagnosia may present with poor recognition of familiar individuals in person or in the photograph, confusion with plotlines in movies or plays with numerous characters, and difficulty distinguishing individuals wearing a uniform or similar clothing. Stroke is the most common cause of acquired prosopagnosia (2). Other less common aetiologies include traumatic brain injury, carbon monoxide poisoning, temporal lobectomy, and encephalitis. Literature has shown that areas involved in acquired prosopagnosia are the right fusiform gyrus or anterior temporal cortex, or both (3). The fusiform gyrus is part of the lateral temporal lobe and occipital lobe in 'Brodmann area 37' (4). The fusiform gyrus is considered a key structure for functionally specialized computations of high-level vision such as face perception, object recognition, and reading. Individuals with fusiform lesions are more likely to have apperceptive prosopagnosia, while those with anterior temporal lesions have an amnestic variant (5). In summary, prosopagnosia can be the sole presentation for the right fusiform gyrus stroke. It is important to recognize prosopagnosia for early stroke diagnosis and avoid misdiagnosing it as a psychiatric or ocular disorder. Keywords: prosopagnosia, fusiform gyrus, stroke.

An update of the Benton Facial Recognition Test.

The Benton Facial Recognition Test (BFRT) is a paper-and-pen task that is traditionally used to assess face perception skills in neurological, clinical and psychiatric conditions. Despite criticisms of its stimuli, the task enjoys a simple procedure and is rapid to administer. Further, it has recently been computerised (BFRT-c), allowing reliable measurement of completion times and the need for online testing. Here, in response to calls for repeat screening for the accurate detection of face processing deficits, we present the BFRT-Revised (BFRT-r): a new version of the BFRT-c that maintains the task's basic paradigm, but employs new, higher-quality stimuli that reflect recent theoretical advances in the field. An initial validation study with typical participants indicated that the BFRT-r has good internal reliability and content validity. A second investigation indicated that while younger and older participants had comparable accuracy, completion times were longer in the latter, highlighting the need for age-matched norms. Administration of the BFRT-r and BFRT-c to 32 individuals with developmental prosopagnosia resulted in improved sensitivity in diagnostic screening for the BFRT-r compared to the BFRT-c. These findings are discussed in relation to current diagnostic screening protocols for face perception deficits. The BFRT-r is stored in an open repository and is freely available to other researchers.

The Oxford Face Matching Test: A non-biased test of the full range of individual differences in face perception.

Tests of face processing are typically designed to identify individuals performing outside of the typical range; either prosopagnosic individuals who exhibit poor face processing ability, or super recognisers, who have superior face processing abilities. Here we describe the development of the Oxford Face Matching Test (OFMT), designed to identify individual differences in face processing across the full range of performance, from prosopagnosia, through the range of typical performance, to super recognisers. Such a test requires items of varying difficulty, but establishing difficulty is problematic when particular populations (e.g., prosopagnosics, individuals with autism spectrum disorder) may use atypical strategies to process faces. If item difficulty is calibrated on neurotypical individuals, then the test may be poorly calibrated for atypical groups, and vice versa. To obtain items of varying difficulty, we used facial recognition algorithms to obtain face pair similarity ratings that are not biased towards specific populations. These face pairs were used as stimuli in the OFMT, and participants were required to judge whether the face images depicted the same individual or different individuals. Across five studies the OFMT was shown to be sensitive to individual differences in the typical population, and in groups of both prosopagnosic individuals and super recognisers. The test-retest reliability of the task was at least equivalent to the Cambridge Face Memory Test and the Glasgow Face Matching Test. Furthermore, results reveal, at least at the group level, that both face perception and face memory are poor in those with prosopagnosia, and are good in super recognisers.

[The "Tortoni effect" in prosopagnosia]. / El "efecto Tortoni" en la prosopagnosia.

Proposapnosia is a type of visual agnosia characterized by the inability to recognize people's faces. There are basically two variants, apperceptive and associative. The "Tortoni effect" is a phenomenon described by Bekinschtein et al a few years ago in waiters from Buenos Aires, who used this tool to remember the orders of each member of a table. We present a case of prosopagnosia associated with bilateral temporo-occipital injury secondary to head trauma, initially manifested by the lack of face recognition with the use of an associative strategy similar to that described in the "Tortoni effect" as compensation, in a 62-year-old female who suffered a severe head injury. A few months after this event, the patient had difficulty in recognizing familiar people, a fact evidenced by her relatives when at a restaurant table, they changed their seats, remained silent momentarily, and right after the patient kept naming them by their previous location. The magnetic resonance imaging of the brain revealed blunt sequelae lesions in the bilateral temporo-occipital region. Acquired prosopagnosia due to focal lesions in the temporo-occipital region, generally bilateral and right, and less frequently left, is a rare condition. The strategy used in the "Tortoni effect" was one of the initial manifestations of the condition in our patient. Carrying out an ecological neuropsychological test that considers this strategy could be useful in the screening and early detection of this entity.

La prosopagnosia es un tipo de agnosia visual caracterizada por la incapacidad de reconocer los rostros de las personas. Existen básicamente dos variantes, aperceptivas y asociativas. El "efecto Tortoni" es un fenómeno descripto por Bekinschtein y col. hace unos años en mozos de café en Buenos Aires, quienes utilizaban esta herramienta para recordar los pedidos de cada integrante de una mesa. Presentamos un caso de prosopagnosia asociada a lesión temporo-occipital bilateral secundaria a traumatismo encefalocraneano, manifestada en forma inicial por la falta de reconocimiento de rostros, con la utilización de una estrategia asociativa similar a la descripta en el efecto "Tortoni" como compensación. Mujer de 62 años que sufrió un traumatismo encefalocraneano grave. Pocos meses después del evento, presentó dificultad para reconocer personas conocidas, hecho evidenciado por sus allegados cuando en una mesa los integrantes cambiaron su asiento, permanecieron callados por unos instantes, y posteriormente la paciente continuó nombrándolos por su ubicación previa. En la resonancia magnética de cerebro se objetivaron lesiones contusas de aspecto secuelar en región temporo-occipital bilateral. La prosopagnosia adquirida secundaria a lesiones focales en la región temporo-occipital generalmente bilateral, derecha, y raramente izquierda, es un cuadro poco frecuente. La estrategia utilizada en el "efecto Tortoni" fue en nuestra paciente una de las manifestaciones iniciales del cuadro. La realización de un test neuropsicológico ecológico que considere esta estrategia podría ser de utilidad en el rastreo y detección precoz de esta entidad.

El "efecto Tortoni" en la prosopagnosia / The "Tortoni effect" in prosopagnosia

Resumen La prosopagnosia es un tipo de agnosia visual caracterizada por la incapacidad de reconocer los rostros de las personas. Existen básicamente dos variantes, aperceptivas y asociativas. El "efecto Tortoni" es un fenómeno descripto por Bekinschtein y col. hace unos años en mozos de café en Buenos Aires, quienes utilizaban esta herramienta para recordar los pedidos de cada integrante de una mesa. Presentamos un caso de prosopagnosia asociada a lesión temporo-occipital bilateral secundaria a traumatismo encefalocra neano, manifestada en forma inicial por la falta de reconocimiento de rostros, con la utilización de una estra tegia asociativa similar a la descripta en el efecto "Tortoni" como compensación. Mujer de 62 años que sufrió un traumatismo encefalocraneano grave. Pocos meses después del evento, presentó dificultad para reconocer personas conocidas, hecho evidenciado por sus allegados cuando en una mesa los integrantes cambiaron su asiento, permanecieron callados por unos instantes, y posteriormente la paciente continuó nombrándolos por su ubicación previa. En la resonancia magnética de cerebro se objetivaron lesiones contusas de aspecto secuelar en región temporo-occipital bilateral. La prosopagnosia adquirida secundaria a lesiones focales en la región temporo-occipital generalmente bilateral, derecha, y raramente izquierda, es un cuadro poco frecuente. La es trategia utilizada en el "efecto Tortoni" fue en nuestra paciente una de las manifestaciones iniciales del cuadro. La realización de un test neuropsicológico ecológico que considere esta estrategia podría ser de utilidad en el rastreo y detección precoz de esta entidad.

Abstract Proposapnosia is a type of visual agnosia characterized by the inability to recognize people's faces. There are basically two variants, apperceptive and associative. The "Tortoni effect" is a phenomenon described by Bekinschtein et al a few years ago in waiters from Buenos Aires, who used this tool to remember the orders of each member of a table. We present a case of prosopagnosia associated with bilateral temporo-occipital injury secondary to head trauma, initially manifested by the lack of face recognition with the use of an associative strategy similar to that described in the "Tortoni effect" as compensation, in a 62-year-old female who suffered a severe head injury. A few months after this event, the patient had difficulty in recognizing familiar people, a fact evidenced by her relatives when at a restaurant table, they changed their seats, remained silent momentarily, and right after the patient kept naming them by their previous location. The magnetic resonance imaging of the brain revealed blunt sequelae lesions in the bilateral temporo-occipital region. Acquired prosopagnosia due to focal lesions in the temporo-occipital region, generally bilateral and right, and less frequently left, is a rare condition. The strategy used in the "Tortoni effect" was one of the initial manifestations of the condition in our patient. Carrying out an ecological neuropsychological test that considers this strategy could be useful in the screening and early detection of this entity.

[A case suspected of Alzheimer type dementia showing multimodal (face and voice) person recognition disorder from face and voice].

A 90-year-old woman presented with a multimodal (face and voice) person recognition disorder. Although she had moderate general cognitive impairment, her visual cognitive capacity, other than face recognition, was well preserved. She could identify the faces and voices of family members but could not recall the names and voices of relatives whom she met infrequently, famous individuals, or the medical staff. She could remember the first names and some information about prominent individuals when supplied with their surnames. Therefore, we thought that her person-specific semantic memory was intact but she was unable to access it through their faces and voices. MRI revealed predominantly right-sided bilateral anterior temporal lobe and hippocampal atrophy. SPECT images showed decreased blood flow in the bilateral anterior temporal lobes and inferior parietal lobule (heavily and predominantly right-sided), right posterior cingulate gyrus, and precuneus. Progressive person recognition disorder or prosopagnosia has been considered a right temporal variant of frontotemporal lobar degeneration because abnormal behaviors and psychiatric symptoms frequently coexist. However, no such symptoms were observed in this case, therefore we suspected dementia of the Alzheimer type.

Prosopagnosia seizure semiology in a 10-year-old boy: a functional neuroimaging study.

We illustrate a case of post-traumatic recurrent transient prosopagnosia in a paediatric patient with a right posterior inferior temporal gyrus haemorrhage seen on imaging and interictal electroencephalogram abnormalities in the right posterior quadrant. Face recognition area mapping with magnetoencephalography (MEG) and functional MRI (fMRI) was performed to clarify the relationship between the lesion and his prosopagnosia, which showed activation of the right fusiform gyrus that colocalised with the lesion. Lesions adjacent to the right fusiform gyrus can result in seizures presenting as transient prosopagnosia. MEG and fMRI can help to attribute this unique semiology to the lesion.