Édouard Manet's Tabes Dorsalis: From Painful Ataxia to Phantom Limb.

Édouard Manet (1832-1883) is considered the "father" of impressionism and even of twentieth century modern art. Manet's genius involved getting away from the classical narrative or historical topics and replacing them by the banality of daily life. Technically, he erased volumes into flat two-dimensional coloured planes, and distorted conventional perspective with often gross brushstrokes intentionally giving an "unfinished" aspect to the work. It is little known that Manet had a very painful second part of his life, due to excruciating limb and chest pains, which developed in parallel with proprioceptive ataxia and gait imbalance. Manet always remained discreet about his private life, and we mainly know that his future wife was his family piano teacher, with whom he had a liaison at the age of 17 years. Later, the great but platonic passion of his life was the painter Berthe Morisot (1841-1895), who married Manet's brother Eugène. In fact, we do not know whether he had a mistress at all, although he had several elegant "flirts" in the mundane and artistic milieu. Thus, while Manet's progressive painful ataxia from the age 40 years yields little doubt on its tabetic origin, how he contracted syphilis at least 15-20 years before will probably remain a mystery. It is fascinating that Manet's daily struggle against pain and poor coordination may have led his art to become one of the most significant of modern times, opening the way to twentieth century avant-gardes, along with another victim of syphilis, Paul Gauguin (1848-1903). Manet never showed any sign of general paresis, and like his contemporary, the writer Alphonse Daudet, his clinical picture remained dominated by paroxysmal pain and walking impairment. Difficult hand co-ordination made him quit watercolour painting, and during the last 2 years of his life he had to focus on small format oil works, the subject of which was nearly limited to modest bunches of fresh flowers, now often considered to be his maturity masterpieces. Having become bedridden, he had to be amputated of one leg, which was developing gangrene, probably associated with ergot overuse. While he died shortly thereafter, we have some witness anecdotes suggesting that he experienced a phantom limb: when Claude Monet (1840-1926) visited him and sat down on his bed, Manet violently shouted that he was sitting on his (absent) leg, which provoked terrible pains. With its facts and mysteries, the subtle interaction between Manet's illness and his work output remains one of the most intriguing stories in the neurology of art.

Optic ataxia: beyond the dorsal stream cliché.

This chapter reviews clinical and scientific approaches to optic ataxia. This double historic track allows us to address important issues such as the link between Bálint syndrome and optic ataxia, the alleged double dissociation between optic ataxia and visual agnosia, and the use of optic ataxia to argue for a specific vision-for-action occipitoposterior parietal stream. Clinical cases are described and reveal that perceptual deficits have been long shown to accompany ataxia. Importantly, the term ataxia appears to be misleading as patients exhibit a combination of visual and nonvisual perceptual, attentional, and visuomotor guidance deficits, which are confirmed by experimental approaches. Three major features of optic ataxia are described. The first is a spatial feature whereby the deficits exhibited by patients appear to be specific to peripheral vision, akin to the field effect. Visuomotor field examination allows us to quantify this deficit and reveals that it consists of a highly reliable retinocentric hypometria. The third is a temporal feature whereby these deficits are exacerbated under temporal constraints, i.e., when attending to dynamic stimuli. These two aspects combine in a situation where patients have to quickly respond to a target presented in peripheral vision that is experimentally displaced upon movement onset. In addition to the field effect, a hand effect can be described in conditions where the hand is not visible. Spatial and temporal aspects as well as field and hand effects may rely on several posterior parietal modules that remain to be precisely identified both anatomically and functionally. It is concluded that optic ataxia is not a visuomotor deficit and there is no dissociation between perception and action capacities in optic ataxia, hence a fortiori no double dissociation between optic ataxia and visual agnosia. Future directions for understanding the basic pathophysiology of optic ataxia are proposed.

Édouard Manet's Tabes Dorsalis: From Painful Ataxia to Phantom Limb.

Édouard Manet (1832-1883) is considered the 'father' of Impressionism and even of XXth century modern art. Manet's genius involved getting away from the classical narrative or historical topics and replacing them by the banality of daily life. Technically, he erased volumes into flat two-dimensional coloured planes, and distorted conventional perspective with often gross brushstrokes intentionally giving an 'unfinished' aspect to the work. It is little known that Manet had a very painful second part of his life, due to excruciating limb and chest pains, which developed in parallel with proprioceptive ataxia and gait imbalance. Manet always remained discreet about his private life, and we mainly know that his future wife was his family piano teacher, with whom he had a liaison already at age 17. Later, the great but platonic passion of his life was the painter Berthe Morisot (1841-1895), who got married to Manet's brother Eugène. In fact, we do not know whether he had any mistress at all, although he had several elegant 'flirts' in the mundane and artistic milieu. Thus, while Manet's progressive painful ataxia from age 40 yields little doubt on its tabetic origin, how he contracted syphilis at least 15-20 years before will probably remain a mystery. It is fascinating that Manet's daily struggle against pain and poor coordination may have led his art to become one of the most significant of modern times, opening the way to XXth century avant-gardes, along with another victim of syphilis, Paul Gauguin (1848-1903). Manet never showed any sign of General Paresis, and like his contemporary the writer Alphonse Daudet, his clinical picture remained dominated by paroxysmal pain and walking impairment. Difficult hand coordination made him quit watercolor painting, and during the last 2 years of his life, he had to focus on small format oil works, whose subject was nearly limited to modest bunches of fresh flowers, now often considered to be his maturity masterpieces. Having become bedridden, he had to be amputated of one leg, which was developing gangrene probably associated with ergot overuse. While he died shortly thereafter, we have some witness anecdotes suggesting that he experienced a phantom limb: when Claude Monet (1840-1926) visited him and sat down on his bed, Manet violently shouted at him that he was just sitting on his (absent) leg, which provoked terrible pains. With its facts and mysteries, the subtle interaction between Manet's illness and his work output remains one of the most intriguing stories in neurology of art.

[Professor ZHANG Yu-Lian's experiences in treatment of post-stroke ataxia by regulating marrow sea acupuncture].

The experiences and characteristics of professor ZHANG Yu-lian in regulating marrow sea acupuncture for treatment of post-stroke ataxia is introduced. Professor ZHANG established regulating marrow sea acupuncture based on the fundamentals of traditional Chinese medicine combined with brain functional region projection and cerebrovascular distribution of modern medicine. "Three acupoints regulating balance" "3 acupoints regulating transportation" "3 acupoints regulating tremor" and Fengchi (GB 20), Fengfu (GB 16), Wangu (GB 12), Tianzhu (BL 10) and C3-C6 Jiaji (Ex-B2) acupoints were applied in this kind of manipulation. We combined holding spirits of doctor and patient together, and controlled the sensation transduction with different manipulations to reach the foci, which emphasis on the doctor-patient cooperation and body-mind co-regulation, finally promote patients' comprehensive rehabilitation.

Milestones in ataxia.

The past 25 years have seen enormous progress in the deciphering of the genetic and molecular basis of ataxias, resulting in improved understanding of their pathogenesis. The most significant milestones during this period were the cloning of the genes associated with the common spinocerebellar ataxias, ataxia telangiectasia, and Friedreich ataxia. To date, the causative mutations of more than 30 spinocerebellar ataxias and 20 recessive ataxias have been identified. In addition, there are numerous acquired ataxias with defined molecular causes, so that the entire number of distinct ataxia disorders exceeds 50 and possibly approaches 100. Despite this enormous heterogeneity, a few recurrent pathophysiological themes stand out. These include protein aggregation, failure of protein homeostasis, perturbations in ion channel function, defects in DNA repair, and mitochondrial dysfunction. The clinical phenotypes of the most common ataxia disorders have been firmly established, and their natural history is being studied in ongoing large observational trials. Effective therapies for ataxias are still lacking. However, novel drug targets are under investigation, and it is expected that there will be an increasing number of therapeutic trials in ataxia.

Was the ataxia of Pierre Marie Machado-Joseph disease?: A reappraisal based on the last autopsy case from la Salpêtrière Hospital.

Nosological placement of l'hérédo-ataxie cérébelleuse de Pierre Marie (HAC) has never been established even after several autopsy cases from the original Haudebourg family had been reported. To reappraise the clinical and pathological features of HAC in the current framework of hereditary ataxias, we screened the autopsy records of la Salpêtrière hospital and identified a patient with a diagnosis of HAC who underwent an autopsy in 1943. Clinical features included heredity compatible with autosomal dominant inheritance, spasticity, increased tendon reflexes, mask-like face, visual impairment, nuclear ophthalmoparesis, and exophthalmos in addition to progressive ataxia. Pathological lesions included the spinal cord (spinocerebellar tracts, anterolateral fascicles, and posterior column), cerebellar dentate nucleus, pontine nucleus, pallidum, motor neurons including the oculomotor nucleus, and substantia nigra. The cerebellar cortex and inferior olives were preserved. These clinical and pathological features, similar to those described in patients from the Haudebourg family, a core prototype of HAC, are indistinguishable from those of Machado-Joseph disease. It would then be possible to conclude that some of the patients historically considered to have HAC would today be classified as having Machado-Joseph disease.

The history of the development of the cerebellar examination.

The cerebellar examination evolved from observations of experimental lesions made by neurophysiologists and clinical descriptions of patients with trauma to the cerebellum. At the beginning of the 19th century, neurophysiologists such as Luigi Rolando, Marie-Jean-Pierre Flourens, and John Call Dalton, Jr. ablated portions of the cerebellum of a variety of animals and observed staggering gait, clumsiness, and falling from side to side without loss of strength. They concluded that the cerebellum coordinated voluntary movements. In 1899, Joseph Francois Félix Babinski observed that patients with cerebellar lesions could not execute complex movements without breaking down into their elemental movements and described the defect as dysmetria. In 1902, Babinski coined the term dysdiodochokinesis to describe the inability to perform rapid execution of movements requiring alternate contractions of agonist and antagonist muscles. Gordon Holmes in 1904 described the phenomena of rebound, noting that if a limb ipsilateral to a cerebellar lesion is suddenly released from tension, the appendage will flail. In 1917, Gordon Holmes reported hypotonia and dysmetria in men wounded by gunshot wounds to their cerebellum. These observations were rapidly included in descriptions of the cerebellar examination in popular contemporaneous textbooks of neurology. Modern observations have demonstrated that the cerebellum influences such cognitive functions such as planning, verbal fluency, abstract reasoning, prosody, and use of correct grammar.

Nineteenth-century contributions to the mechanical recording of postural sway.

In the first half of the 19th century, European physicians, including Marshall Hall, Bernardus Brach, and Moritz Romberg, described loss of postural control in darkness by patients with severely compromised proprioception. Late 19th-century neurologists developed instruments to measure and record postural sway in patients with neurologic disease. Principal American contributors were the neurologist Silas Weir Mitchell from Philadelphia, Pa, and his trainees Morris Lewis and Guy Hinsdale. The efforts of these neurologists anticipated later physiologic studies and ultimately the development of computerized dynamic platform posturography.

[Ataxic instable Alajouanine-Akerman's hand: recovery of a semiologic sign]. / Mão instável atáxica de Alajouanine-Akerman. Resgate de um sinal semiológico.

It is aimed to recover, considering its historical value, a semiological sign described in 1931 by an eminent neurologist of Rio de Janeiro, together with a master of the French neurology. In the article by Alajouanine and Akerman, named "Attitude of the hand in an astereognostic monobrachial crisis of multiple esclerosis", a semiologic alteration was described which was characterized by "an instability in the attitude of the fingers, which is observed mainly with the hand extended in the attitude of swearing". This attitude of hand worsened a lot with the eyes closed and was accompanied by sensory ataxia, astereognosis, and impaired deep sensation in the affected member. From the original article, it is possible to consider at the present time the described semiologic alteration as a form of pseudoathetosis localized in the hand.

Staggering gait in medical history.

Drunkenness and senility were recognized early as the basis of a staggering gait. To these were added venereal excesses, hence syphilis. Medical and scientific concerns began to be focused on "locomotor ataxia" in the 19th century with the systematic development of neuroanatomy and physiology. Rolando and Flourens were followed by Romberg and Todd, and later Friedreich, who all gave the spinal cord temporal precedence as a culprit over the cerebellum--and there were some forerunners. New spinal sensory pathways were delineated by Goll, Flechsig, and Gowers. In France, we must specially credit Duchenne as well as Bouillaud, and later Babinski, Marie, and Dejerine, for correcting, differentiating, extending, and underpinning current concepts. Failures of input and output, of conduction and central coordination were invoked and explained, among them the vestibular apparatus, as well as the causation by neoplasms, demyelination, degeneration, and infarction affecting even the frontal lobe, thalamus, and basal ganglia. Clinical testing was brought up to 20th-century standards essentially by Sherrington and Bárány, followed by Dandy, the neurosurgeon who showed how to replace ventricular fluid by air, more recently made obsolete by modern roentgenographic procedures. And tabes dorsalis, once the chief culprit, has practically become a medical anachronism thanks to penicillin.

[Roussy-Levy hereditary areflexic dysstasia. Its historical relation to Friedreich's disease, Charcot-Marie-Tooth atrophy and Dejerine-Sottas hypertrophic neuritis; the present status of the original family; the nosologic role of this entity]. / La dystasie aréflexique héréditaire de Roussy-Lévy. Ses rapports historiques avec la maladie de Friedreich, l'atrophie Charcot-Marie-Tooth et la névrite hypertrophique de Dejerine-Sottas; l'état actuel de la famille originale; la place nosologique de cette entité.

This survey of Roussy-Lévy disease begins with an historical account of the three neurological conditions from which this entity has been separated: Friedreich disease, described in 1861-1863, which proved with time to be a genuine anatomoclinical disorder: Charcot-Marie-Tooth atrophy, described in 1886, particular because of its morphology and evolution but due to various processes: Dejerine-Sottas hypertrophic neuritis, described in 1893, which was the first variant to be individualized within the heterogenous group of primary and familial hypertrophic neuritis. The initial description of Roussy-Lévy disease--in 1926, 1932, and 1934--and the controversies raised by this concept are recalled as well as the present state of the original family: five out of seven members have been examined since 1956 and it has been demonstrated that they are suffering from a form of hypertrophic neuritis. However it is this author's opinion that the concept of an autonomous Roussy-Lévy disease within hypertrophic neuritis is justified by the following criteria: dominant transmission, very precocious onset, extreme slowness of the evolution, remarkable benignity of the prognosis.