De Morsier Research Articles

La description du syndrome de Charles Bonnet par Georges de Morsier dans les Annales Médico-Psychologiques

More than 30 years after having introduced the term “Charles Bonnet syndrome”, Professor Georges de Morsier fixed its definition in a 1967 study published in Annales Médico-Psychologiques. In this study, he reaffirmed the intellectual and mental integrity of the elderly patients among whom this syndrome occurs. He denied that the hallucinations associated with this syndrome were caused by any visual pathology, claiming instead that they were the result of lesions in the visual areas of the brain. This study, which is often cited as the first description of the syndrome, was challenged in its day. Today, the role of visual pathologies in the etiology of the syndrome is widely acknowledged. One may explain de Morsier's mistake by pointing to his strict adherence to de Clérambault's organicist doctrine.

Refractory Central Serous Retinal Detachment in the Presence of Optic Disc Pit – Case Report

Among congenital optic disc anomalies are megalopapilla, optic nerve aplasia and hypoplasia (de Morsier’s syndrome) and optic disc excavations. The latter are attributed to abnormal fetal fissure’s closure. There is a wide spectrum of these anomalies ranging from single optic disc pit, to multiple pits, to morning glory syndrome. Excavated defects of the optic disc are usually associated with retinal detachment in the macular region. It is usually confined to the macular region though a subtotal or total retinal detachment may occur. It requires treatment (a surgical approach in most cases) though a spontaneous resolution of the subretinal fluid within a short period of the detachment onset has been observed. We report a case of unilateral central serous retinal detachment in the presence of a bilateral optic disc pit refractory to primary treatment.

Septo-optic Dysplasia (SOD) or de Morsiers’s Syndrome: a Case Report

Background: Septo-optic dysplasia (SOD) or de Morsiers’s syndrome is a rare congenital disorder characterized by optic nerve hypoplasia, hypothalamic-pituitary axis hypoplasia, and midline abnormalities of the brain, agenesis of septum pellucidum and corpus callosum. Initially, it was described in 1941 by Reeves and further discussed by the French-Swiss neurologist de Morsier in 1956. The phenotype of SOD is highly heterogeneous, and the existence of at least two features is considered sufficient for diagnosis. Objective: The aim is to emphasize the importance of including SOD in the differential diagnosis of young patients, presenting with strabismus, especially if very low vision is documented in one or both eyes. Case report: In this case, a 13-year-old male presented to our institution with a complaint of deviating left eye outwards in the last few years. During the examination, left eye exotropia, as well as low visual acuity (light perception) of the left eye were documented. The findings of the right eye were normal and visual acuity was 1.0. Magnetic resonance imaging, optical coherence tomography, and fundus photography revealed optic nerve, brain midline, and retinal abnormalities. The patient was diagnosed as having SOD with optic nerve hypoplasia, septum pellucidum agenesis, and hypothalamic-pituitary axis hypoplasia. Conclusion: Although SOD is not curable, many aspects of this syndrome can be improved through a multidisciplinary approach consisting of hormonal replacement, corrective ophthalmological surgery, as well as neuropsychological treatment, and support if necessary.

ODP529 Update on the Prevalence and Pathogenesis of Central Hypothyroidism

Abstract Background Central hypothyroidism (CH) is a rare form of hypothyroidism characterized by insufficient stimulation of a normal thyroid gland by the thyroid-stimulating hormone (TSH). It can be of pituitary origin (secondary hypothyroidism) or hypothalamic origin (tertiary hypothyroidism). The diagnosis is suspected by the finding of low serum free thyroxine associated with low, normal, or slightly elevated serum TSH, in the absence of goiter. This review presents an update on the prevalence and pathogenesis of different types of CH. Methods A systematic search of literature was conducted using the search terms hypothyroidism, central hypothyroidism, congenital central hypothyroidism, acquired central hypothyroidism, TSH deficiency, prevalence, and pathogenesis. Results The prevalence of overt hypothyroidism in the US population is at least 0.3% and the prevalence of subclinical hypothyroidism ranges from 4% to 10%. Primary hypothyroidism is more prevalent in females. The prevalence of CH ranges from 1: 13,000 to 1: 120,000. CH is observed in less than 1% of hypothyroid subjects, with no evidence of sex predominance. Itcan occur prenatally (congenital CH) or after birth (acquired CH). Congenital CH, which is a rare cause of CH (prevalence around 1: 13,000), may result from genetic/malformative causes [e. g., pituitary-specific transcription factor defects, TSH-beta subunit gene mutations, isolated TSH-releasing hormone (TRH) deficiency, TRH receptor gene mutation, immunoglobulin superfamily member 1 deficiency, De Morsier syndrome, Prader Willi syndrome]. In 60% of the cases, congenital CH is associated with other pituitary hormone deficiencies and in 40% of the cases, congenital CH is due to isolated TSH deficiency. Acquired CH, which is the most common cause of CH andusually associated with other pituitary hormone deficiencies, results from the destruction or inhibition of the pituitary and/or hypothalamus. In decreasing order of frequency, acquired CH can be secondary to invasive and/or compressive lesions (e. g., pituitary macroadenomas, craniopharyngiomas, pituitary metastases, carotid aneurysm), iatrogenic factors (e. g., surgery or irradiation, glucocorticoids, dopaminergic drugs, somatostatin analogs, immune checkpoint inhibitors), trauma (e. g., brain injury), vascular causes (e. g., pituitary apoplexy, postpartum pituitary necrosis, hemorrhage), autoimmunity (e. g., lymphocytic hypophysitis), infiltrative lesions (e. g., hemochromatosis, sarcoidosis), and infections (e. g., tuberculosis, syphilis). Conclusion CH is a rare cause of hypothyroidism resulting from a variety of congenital or acquired causes. Acquired CH is the most common cause of CH. In many cases, both congenital and acquired CH are associated with multiple pituitary hormone deficiencies. Isolated TSH deficiency is rare and usually of congenital origin. Presentation: No date and time listed

De Morsier plus syndrome: A rare congenital disorder presenting with adult onset seizure

De Morsier syndrome, also known as septo-optic dysplasia (SOD), describes a triad of optic nerve hypoplasia, hypothalamic-pituitary dysfunction and midline abnormalities such as absent septum pellucidum or dysgenesis of the corpus callosum. It is a rare congenital disorder that an estimated incidence of 1/50,000 was recorded. This disease has a variety of clinical symptoms, which include visual manifestations (85-90%), intellectual delay (42.5-60%), seizures (27.5-55%) and endocrine abnormalities (50-55%). However, those patients with symptoms of global developmental delay and motor deficits could not be explained by the corpus callosum hypoplasia alone and were noted to manifest other underrecognized cortical malformations such as schizencephaly and polymicrogyria, hence termed De Morsier plus syndrome. In the Philippines, the existence of De Morsier plus syndrome with a lack of developmental disorders detected during adulthood is uncommon with no reported case yet. This report is a case of a 24-year-old, male, who presented with adult-onset seizure with associated eye symptoms. Cranial magnetic resonance imaging revealed bilateral schizencephaly in both parietal lobes with absent septum pellucidum, polymicrogyria, agyria-pachygyria and optic nerve atrophy. On electroencephalogram, epileptiform discharges were noted in the left parietal area. This case was managed under anti-seizure medication with no seizure recurrence and a good outcome.

Chapter 5 - Septo-optic dysplasia

Septo-optic dysplasia (SOD) or de Morsier's syndrome is a rare congenital disorder characterized by a classic triad of: (a) optic nerve hypoplasia, (b) agenesis of septum pellucidum and corpus callosum, and (c) hypoplasia of the hypothalamic-pituitary axis. This chapter will outline the key information regarding the etiology and epidemiology of this syndrome with a focus on its comprehensive management. Particular attention will be paid to the diagnostic stage and the most relevant differential diagnosis, before moving to the complexities of its treatment. In fact, although SOD is not curable, many aspects of this syndrome can be improved through a tailored multidisciplinary approach consisting in hormonal replacement, corrective ophthalmological surgery, management of epileptic seizures, and active neuropsychological support.

Ocular manifestations of de-Morsiers syndrome

A two-year old male child presented to the ophthalmology outpatient department (OPD) with parents complaining of child’s inability to make an eye contact. On examination, the patient had horizontal manifest nystagmus with no fixing and following. The pupillary reactions and the anterior segment examination were normal. Fundus examination in both eyes revealed small optic disc with 360 degree hyperpigmented ring in both the eyes (Figure 1 A and B). The macula was >2 disc diameter (DD) in distance from optic disc, suggestive of optic nerve hypoplasia. Magnetic resonance imaging (MRI) brain showed absent septum pellucidum (Figure 1 C and D). Serum growth hormone levels were significantly high. Based on the examination and investigations, diagnosis of de Morsier’s syndrome was made.

Síndrome de De Morsier: relato de dois casos na cidade de Petrópolis

A Residência Pediátrica (RP) é uma revista eletrônica trimestral, em publicação contínua, da Sociedade Brasileira de Pediatria (SBP), disponível em português e inglês, destinada aos médicos residentes de nossa especialidade, áreas afins e aos pediatras. O conteúdo da revista pode ser acessado livremente. A missão da RP é contribuir para a formação acadêmica dos residentes e incentivá-los a uma participação ativa na produção científica sobre temas pediátricos e relacionados à adolescência.

Septo-optic dysplasia with cerebellar hemiagenesis - a rare congenital malformation.

Septo-optic dysplasia (De morsier’s syndrome) is a rare congenital malformation of the central nervous system and represents a clinical spectrum rather than a specific entity. It is defined by any combination of pituitary hypoplasia with or without consequent panhypopituitarism,optic nerve hypoplasia and midline neurological abnormalities such as absent septum pellucidum and corpus collasum agenesis or thinning. Septo-optic dysplasia with associated cerebellar hemiagenesis is a much rarer congenital anomaly and may represent one end of this spectrum.

Neuro-Ophthalmological Manifestations Of Septo-Optic Dysplasia: Current Perspectives.

Septo-optic dysplasia (SOD), also known as de Morsier syndrome, is a rare congenital disorder belonging to the group of mid-line brain malformations. Despite the highly variable phenotypic penetration, its classical triad include a) optic nerve hypoplasia (ONH), b) agenesis of septum pellucidum and corpus callosum, and c) hypoplasia of the hypothalamo-pituitary axis. SOD has stringent diagnostic criteria requiring 2 or more features of the classic triad, therefore it represents a separate entity from other conditions such as ONH and achiasmia syndromes which share only some of these aspects, or SOD plus syndrome which is characterized by additional cortical abnormalities. Starting from its etiology and epidemiology, this narrative review focuses on the management of SOD patients, including their diagnosis, treatment and follow-up. To date, SOD is not curable; nonetheless, many of its symptoms can be improved through a tailored approach, consisting of hormonal replacement, corrective ophthalmological surgery and neuropsychological support.

Charles Bonnet syndrome – disturbing ‘playthings of the brain’

In his paper on page 518 of this issue, Dr Eric Nieman describes his medical journey of a progressive visual deterioration due to glaucoma, accompanied by the gradual development of...

Neonatal hypoglycemia in the De Morsier syndrome

The article discusses the causes and diagnostic criteria of the septo-optic dysplasia or De Morsier syndrome. De Morsier syndrome attracts attention of endocrinologists due to the development secondary hypofunction of the endocrine glands and somatotropic deficiency associated with this disease. Septo-optic dysplasia is a polyetiologic disease. The relationship of the disease with the antenatal influence of alcohol, narcotic substances, neurotropic drugs, significant perinatal infections, maternal endocrine diseases, gene mutations, in particular, mutations in the HESX1 gene, encoding the pituitary transcription factors involved in the embryogenesis of the adenohypophysis. We report a clinical case of the disease accompanied by congenital hypopituitarism symptoms. The disease manifested in the neonatal period. The child had severe hypoglycemia in combination with neurological symptoms in the form muscle hypotonia and oculomotor disturbances. The diagnosis of the De Morsier syndrome was verified by the results of MRI of the brain. Endocrine disorders in this patient were characterized by low levels of ACTH, cortisol, IGF-1, and free T4. The administered therapy corrected endocrine disorders in the child. In summary, septo-optic dysplasia or De Morsier syndrome is the subject to interdisciplinary attention of neonatologists, endocrinologists, neurologists, optometrists, and geneticists.

Neonatal hypoglycemia in the De Morsier syndrome

The article discusses the causes and diagnostic criteria of the septo-optic dysplasia or De Morsier syndrome. De Morsier syndrome attracts attention of endocrinologists due to the development secondary hypofunction of the endocrine glands and somatotropic deficiency associated with this disease. Septo-optic dysplasia is a polyetiologic disease. The relationship of the disease with the antenatal influence of alcohol, narcotic substances, neurotropic drugs, significant perinatal infections, maternal endocrine diseases, gene mutations, in particular, mutations in the HESX1 gene, encoding the pituitary transcription factors involved in the embryogenesis of the adenohypophysis. We report a clinical case of the disease accompanied by congenital hypopituitarism symptoms. The disease manifested in the neonatal period. The child had severe hypoglycemia in combination with neurological symptoms in the form muscle hypotonia and oculomotor disturbances. The diagnosis of the De Morsier syndrome was verified by the results of MRI of the brain. Endocrine disorders in this patient were characterized by low levels of ACTH, cortisol, IGF-1, and free T4. The administered therapy corrected endocrine disorders in the child. In summary, septo-optic dysplasia or De Morsier syndrome is the subject to interdisciplinary attention of neonatologists, endocrinologists, neurologists, optometrists, and geneticists.

Septo-Optic Dysplasia: A Case Study

Septo-optic dysplasia (SOD) is a rare congenital heterogeneous malformation. SOD was formerly known as de Morsier syndrome, which associated a midline brain defect such as an absent septum pellucidum with optic nerve hypoplasia. The diagnosis of SOD is made when there are two or more characteristics of the classic triad. The triad consists of optic nerve hypoplasia, pituitary hormone abnormalities, and midline brain defects, although it can vary in the severity of clinical presentation and phenotype. The purpose of this article is to review a case and analyze the literature regarding prevalence, etiology, clinical presentation, diagnosis, and management of SOD.

On visual hallucinations and cortical networks: a trans-diagnostic review.

Our current clinical approach to visual hallucinations is largely derived from work carried out by Georges de Morsier in the 1930s. Now, almost a century after his influential papers, we have the research tools to further explore the ideas he put forward. In this review, we address de Morsier’s proposal that visual hallucinations in all clinical conditions have a similar neurological mechanism by comparing structural imaging studies of susceptibility to visual hallucinations in Parkinson’s disease, Alzheimer’s disease, Dementia with Lewy bodies and schizophrenia. Systematic review of the literature was undertaken using PubMed searches. A total of 18 studies across conditions were identified reporting grey matter differences between patients with and without visual hallucinations. Grey matter changes were categorised into brain regions relevant to current theories of visual hallucinations. The distribution of cortical atrophy supports de Morsier’s premise that visual hallucinations are invariably linked to aberrant activity within visual thalamo-cortical networks. Further work is required to determine by what mechanism these networks become predisposed to spontaneous activation, and whether the frontal lobe and hippocampal changes identified are present in all conditions. The findings have implications for the development of effective treatments for visual hallucinations.

Is there an encephalographic trait to septo-optic dysplasia? (de Morsier syndrome)

Objectives. Septo-optic dysplasia (SOD), also known as de Morsier syndrome is a rare congenital syndrome involving variable midline brain structures, characterized by visual impairment, pituitary deficiencies and specific brain abnormalities (especially midbrain malformations). The clinical phenotype is highly variable, from mild to extremely severe. The purpose of this paper is to present the electroencephalographic abnormalities in two clinical cases and correlate with the ones that have been described in the literature in order to find a specific trait of this syndrome. Methods and results. A 12-year-old girl was hospitalized in the pediatric neurology clinic for focal seizures, developmental delay and cerebral palsy. Clinical examination showed visual disturbances (nystagmus and oculomotor nerve palsy), unilateral pyramidal syndrome – left hemiparesis. Electroencephalography (EEG) revealed bilateral photosensitive epileptic form discharges, but asymmetrical, right more than left, with abnormal, slower background. MRI showed optic nerve hypoplasia and hypoplasia of the corpus callosum. The second case was also a girl, a 7-year-old who had only 2 focal right motor seizures with secondary generalization and developmental delay. Clinical examination showed visual impairment and bilateral nystagmus. The standard EEG evaluation didn’t reveal any abnormalities, with normal background. Cerebral MRI showed midline brain defects – agenesis of the septum pellucidum, agenesis of corpus callosum and optic nerve hypoplasia. Endocrine evaluation for pituitary function showed in the first case – increased thyroid-stimulating hormone and in the second case – decreased IGF1. Antiepileptic treatment was started in both cases, but seizures were refractory to treatment and persistent EEG abnormalities still present in the first case, with good evolution of epilepsy in the second case. Conclusions. The epileptiform abnormalities associated to SOD are present only if there is an associated cortical malformation.

Septo-optic dysplasia plus: a case report

BackgroundSepto-optic dysplasia, also referred to as de Morsier syndrome, is a congenital condition characterized by classic triad features: midline brain abnormalities, optic nerve hypoplasia and pituitary endocrine dysfunction. Sometimes, other various malformations appear within syndrome.Case presentationAn 11 and 1/2-year-old Caucasian Southeast European female patient with earlier established diagnoses of growth hormone deficiency, diabetes insipidus, seizures, mental retardation, optic nerve atrophy and right ptosis, was directed to us for consultative examination.The girl of short stature and low weight for her age had bilateral optic nerve hypoplasia, poor vision, nystagmus and right eye oculomotor palsy. Electroencephalogram revealed epileptic changes. Magnetic resonance imaging showed an empty sella syndrome, partial hypoplasia of corpus callosum, cavum of pellucid septum and diffuse polymicrogyria of the left temporal lobe. We found all elements of septo-optic dysplasia plus syndrome with right oculomotor nerve involvement.ConclusionBy earlier findings and evaluation, we established a diagnosis of septo-optic dysplasia plus. The case confirms the existence of various malformations within the syndrome and the need for the cooperation of several specialists in the diagnosis and treatment of children with the syndrome.

Optic Nerve Hypoplasia Syndrome: A Review of the Epidemiology and Clinical Associations

Optic nerve hypoplasia (ONH) has developed into a leading cause of congenital blindness. The frequently associated features of hypopituitarism and absent septum pellucidum were felt to have embryonic linkage as "septo-optic dysplasia" or "de Morsier's syndrome." More recent studies have suggested these associations are independent of one another. This review provides an assessment of the historical and recent evidence linking neuroradiologic, endocrinologic and developmental morbidity in patients with ONH. The prenatal risk factors, heritability, and genetic mutations associated with ONH are described. Recognition of the critical association of ONH with hypopituitarism should be attributed to William Hoyt, not Georges de Morsier. De Morsier never described a case of ONH or recognized its association with hypopituitarism or missing septum pellucidum. Hypopituitarism is caused by hypothalamic dysfunction. This, and other more recently identified associations with ONH, such as developmental delay and autism, are independent of septum pellucidum development. Other common neuroradiographic associations such as corpus callosum hypoplasia, gyrus dysplasia, and cortical heterotopia may have prognostic significance. The predominant prenatal risk factors for ONH are primiparity and young maternal age. Presumed risk factors such as prenatal exposure to drugs and alcohol are not supported by scrutiny of the literature. Heritability and identified gene mutations in cases of ONH are rare. Children with ONH require monitoring for many systemic, developmental, and even life-threatening problems independent of the severity of ONH and presence of brain malformations including abnormalities of the septum pellucidum. "Septo-optic dysplasia" and "de Morsier's syndrome" are historically inaccurate and clinically misleading terms.

The sound of silence

In January, 2011, a previously well 68-year-old woman, presented to our neurology outpatients department with a 6 week history of hearing a male voice singing and gradual bilateral hearing loss over the course of 1 year. The singing started suddenly with songs she knew from her childhood, and more recently she also reported hearing a choir. The singing was perceived as external, heard only in the left ear, and continued all day and night. She reported that the voices were irritating, particularly when trying to sleep. They stopped when she fell asleep and did not interfere with her daytime activities. She had insight that the voices were imaginary but said that she could not “switch them off ” and did not recall any specifi c triggers. There was no alteration of the symptoms with her mood or tiredness. There was no reported change in her mood, with no signs of depression or mania, nor thoughts or intentions of self harm. She did not report tinnitus, other sensory hallucinations, or any symptoms of epileptic seizures such as vacant attacks, odd tastes or smells, incontinence, tongue biting at night, or psychomotor automatisms. She had no history of thought insertion, withdrawal, broadcasting, or any other features of schizophrenia. She had a medical history of hypertension, and was on aspirin, atenolol, and olmesartan. Amitriptyline was prescribed for insomnia. She did not smoke, drink alcohol, or use recreational drugs. On examination she was anxious, but alert and oriented with no cognitive impairment. A full mini-mental state examination could not be done because her hearing loss made the repetition of a sentence impossible. However, she scored 27/27 points on the incomplete test. She was oriented in time, person, and place, with no evidence of alteration of consciousness. She had bilateral hearing loss. Neurological examination was unremarkable other than absent vibration sensation and ankle refl exes. Routine blood tests were normal. An electroencephalogram done whilst she was experiencing the singing, did not show epileptic activity or any other focal or generalised brain abnormalities. CT of the head was normal. MRI of the head (with T1, T2, and FLAIR sequences) showed only a few white matter hyper intensities of the T2 and FLAIR sequences, which can be associated with smallvessel disease secondary to hyper tension. Ear, nose, and throat examination and pure tone audiogram showed bilateral sensorineural hearing loss only. Psychiatric assessment ruled out the possibility of mental illness. Our patient was diagnosed with musical ear syndrome because there was no evidence of neurological or psychiatric disease. This is a syndrome comparable to Charles Bonnet Syndrome in which people with visual impairment have visual hallucinations. Our patient was reassured about the benign nature of her condition. Multiple anticonvulsants were tried with mixed results. In February, 2012, at last follow-up, she had marked improvement of her symptoms after starting Clonazepam. Charles Bonnet was a Swiss scientist who documented complex visual hallucinations in his blind but otherwise healthy grandfather in 1760. Later the blind Charles Bonnet also had similar hallu cinations. Diagnostic criteria have since been developed for the disorder fi rst described as Charles Bonnet Syndrome by a Swiss neurologist George de Morsier, in 1967. Clinical features are often stereotyped, complex visual hallucin ations in a partially sighted patient. The patient has insight that the hallucinations are not real, and there is no psychosis, neurological or cognitive impairment, intox ication, or metabolic abnormality. No other sensory hallu cinations should be present. Generally the hallucinations are self limiting. No clear evidence for treatment exists. The mechanism of Charles Bonnet Syndrome is poorly understood. Few reports about complex auditory hallucinations in people with hearing impairment exist; however, some have suggested that they are quite common. Whether the diagnostic criteria of Charles Bonnet Syndrome should be extended to include complex auditory hallucinations, or these considered separately as Musical Ear Syndrome, is debatable. Our patient’s case is a reminder that hallucinations are not always due to delirium or to psychological or neurodegenerative conditions. Other conditions, such as Charles Bonnet Syndrome or musical ear syndrome, should also be considered.

Gaëtan Gatian de Clérambault (1872–1934) II. Son héritage psychiatrique

The name of G. de Clerambault is nowadays used to designate two distinct psychiatric syndromes: in France and in Russia, the “mental automatism”; in USA and Great Britain, the erotomanic delusion. The mental automatism becomes Clerambault's syndrome at the congress of French alienists of Blois (1927), by the proposition of Angelo Hesnard. The denomination is adopted by Eugen Minkowski in 1933, whereas Clerambault is still alive. The “great automatism” includes the “first rank symptoms” of the schizophrenia described by Kurt Schneider (1939). The symptoms of the “little automatism” are near from the schizophrenic thought disorders of Bleuler (De Morsier, 1929; Heuyer, 1950). Better than the theory of John Hughlings Jackson, they foreshadow the dichotomy between positive and negative symptoms in the schizophrenia (Berrios). The divergences with the “organo-dynamic” theory of Henri Claude and of his pupil Henri Ey (1934, 1948) are more about the psychopathology of the hallucinations, in relation to the delusion, than about the organic origin of the syndrome. “Pure” erotomanic delusion is discussed by Capgras (1923) and then by the pupils of Henri Claude (Lacan, 1932, Ferdière, 1937). It is named Clerambault's syndrome 20years later in the American textbooks of psychiatry (Arieti, 1959; Lehman, 1967). Its comorbidity with several other psychiatric disorders is described in many papers. An “erotomanic type” of the delusional disorder is isolated in the DSM-III-R. After their quarrel of 193l, Jacques Lacan makes a “return to Clerambault” in the years following the 2nd World War and presents him as his “only master in psychiatry” (1966). Many influential French psychiatrists (Guiraud, Heuyer, Daumezon, Baruk, Sivadon) present him as a pionneer. At the end of the 20th century, a biography and a film are exclusively devoted to him.