Alfred Vulpian and Jean-Martin Charcot in each other's shadow? From Castor and Pollux at La Salpêtrière to neurology forever

While Alfred Vulpian (1826-1887) is not completely forgotten, he cannot match the uninterrupted celebrity which Jean-Martin Charcot (1825-1893) still enjoys today. After becoming interne (residents) at the same institute in 1848, both were involved in shaping the cradle of what would become modern neurology. Both started work as chiefs at a La Salpêtrière service on January 1, 1862, making common rounds and studies, with several common publications. While their friendship remained 'for life', as stated by Charcot at Vulpian's funeral, their career paths differed. Vulpian progressed quicker and higher, being appointed full professor and elected at the Académie Nationale de Médecine and the Académie des Sciences several years before Charcot, as well as becoming dean of the Paris Faculty of Medicine. These positions also enabled him to support his friend Charcot in getting appointed full clinical professor and becoming the first holder of the chair of Clinique des Maladies du Système Nerveux in 1882. Before studying medicine, Vulpian had worked in physiology with Pierre Flourens, and his career always remained balanced between physiology and neurology, with remarkable papers. He introduced Charcot to optic microscopy during their La Salpêtrière years, indirectly helping him to become his successor to the chair of pathological anatomy in 1872. While Vulpian succeeded so well in local medical affairs, Charcot spent his time building up a huge clinical service and a teaching 'school' at La Salpêtrière, which he never left for over 31 years until his death. This 'school' progressively became synonymous with clinical neurology itself and perpetuated the master's memory for decades. Vulpian never had such support, although Jules Déjerine was his pupil and Joseph Babinski was his interne before becoming Charcot's chef de clinique (chief of staff) in 1885. This unusual switch in Parisian medicine contributed to Charcot's unaltered celebrity over more than a century, while Vulpian was progressively relegated to the studies of historians. However, Vulpian and Charcot remain inseparable in the memory of a lifelong friendship which gave birth to neurology.     

Une page de l’histoire de la psychiatrie à la Salpêtrière

The author explains the motives of this very special opportunity of the jubilee. He gives homage to the “boss”, chairman of psychiatry and master of school of la Salpêtrière, to the teacher and “pedagogious” to the intellectual authority, and finaly to the human qualities of the public and private human being of the 80 years old master.     

Prosper Ménière and his disease.

In 1861, Prosper Ménière presented a paper before the French Academy of Medicine in which he described a series of patients with episodic vertigo and hearing loss. He also mentioned the postmortem examination of a young girl who experienced vertigo after a hemorrhage into the inner ear. Prior to that time, vertigo was thought to be a cerebral symptom similar to epileptic seizures. Ménière pointed out that vertigo frequently had a benign course and that common treatments, such as bleeding, often did more harm than good. He was not attempting to define a disease or syndrome but rather to emphasize that vertigo could originate from damage to the inner ear. Confusion regarding the clinical and pathologic features of Ménière disease persisted well into the 20th century.     

Adolphe Gubler (1821–1879) or Parisian neurology outside La Salpêtrière in the age of Jean-Martin Charcot

Adolphe Gubler (1821–1879) is a typical example of a 19th century hospital physician in Paris. Head of a medical unit at Beaujon hospital in 1855, he was nominated to the treatment and pharmacognosia Chair in 1868. He trained many students who became his disciples and remained very close to him. Gubler published prolifically in all areas of medicine. His most well-known work is clearly his contribution to the study of vascular accidents affecting the brain stem, which Auguste Millard worked on simultaneously; hence the eponymous Millard-Gubler syndrome, an example of crossed hemiplegia. Following a brief biography, we will present Gubler's main publications in the area of neurology: on migraine, neurological damage during acute rheumatic fever, aphasia, and the autonomic nervous system. Much of this work was carried out through student theses that Gubler directed. The fame of his contemporary Jean-Martin Charcot (1825–1893) eclipsed that of Gubler, even though the latter was well known and respected among Parisian professors. By tying together the diverse threads of his work, we hope to renew interest in this 19th century neurologist.     

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.     

Effects of intratympanic gentamicin and intratympanic glucocorticoids in Ménière’s disease: a network meta-analysis

Journal of Neurology - Intratympanic therapies, usually including glucocorticoid and gentamicin, are becoming worldwide used in clinical practice of Ménière’s disease today....     

Endolymphatic space size in patients with vestibular migraine and Ménière’s disease

Ménière’s disease (MD) is characterized by episodic vertigo, fluctuating hearing loss and tinnitus. Vestibular migraine (VM) is a relatively new disorder that is characterized by episodic vertigo or dizziness, coexisting migraine and absence of hearing loss. It is occasionally difficult to distinguish between VM and vestibular MD with headache. Because endolymphatic hydrops (EH) is a characteristic sign of MD, we attempted to evaluate endolymphatic space size in both diseases. Endolymphatic space size in the vestibule and the cochlea was evaluated in seven patients with VM and in seven age- and sex-matched patients with vestibular MD. For visualization of the endolymphatic space, 3T magnetic resonance imaging was taken 4 h after intravenous injection of gadolinium contrast agents using three-dimensional fluid-attenuated inversion recovery and HYbriD of reversed image of positive endolymph signal and native image of positive perilymph signal techniques. In the vestibule of VM patients, EH was not observed, with the exception of two patients with unilateral or bilateral EH. In contrast, in the vestibule of patients with vestibular MD, all patients had significant EH, bilaterally or unilaterally. These results indicate that endolymphatic space size is significantly different between patients with VM and vestibular MD.     

Alzheimer’s disease: past,present, and future

As we look to a world where more individuals will suffer from dementia, it is important to reflect on our past accomplishments as we work to create a better future. Attempts to develop better medications for Alzheimer’s disease focus on symptomatic treatments for both cognitive and behavioral symptoms. Some progress has been made in the areas through use of cholinesterase inhibitors and novel neuroleptics. Quality of life is a central concept to improving our interventions and to developing more effective treatments based on our understanding of pathogenesis. In the past, almost hundred years since the pioneering work in Emil Kraepelin’s laboratory that we are celebrating in this series of papers, we have come far but we have further to go.     

Trouble sévère du sommeil à l’adolescence : réflexions théorico-cliniques

In the adolescence, period of the existence in the course of which the physical and psychic modifications are considerable, it is hardly always easy to be able to put into words apprehensions and incomprehensions. The body can then serve as vector, even release of the unspeakable connected in the multiple new and potentially stressful experiences, which the teenager realizes. It is frequent that we speak about psychosomatic phenomena when the demonstrations of the body and the spirit are confidentially connected. One of the possible expressions presented to the adolescence consists in the diverse modalities of the disturbances of the sleep. After a brief reminder of sleeping disorders in the adolescence and their understanding, we shall evoke a clinical label to develop a reflection on some marks at the level of the therapeutic support. It is from our experience within a unit of psychiatry of connection in a general hospital that we shall discuss an original model of pedopsychiatric care psychosomatic phenomena.     

Spinocerebellar Ataxia Type 7: Clinical Course, Phenotype–Genotype Correlations, and Neuropathology

Spinocerebellar ataxia type 7 is a neurodegenerative polyglutamine disease characterized by ataxia and retinal degeneration. The longitudinal course is unknown, and relationships between repeat expansion, clinical manifestations, and neuropathology remain uncertain. We followed 16 affected individuals of a 61-member kindred over 27 years with electroretinograms, neurological examinations including the Brief Ataxia Rating Scale, neuroimaging in five, and autopsy in four cases. We identified four stages of the illness: Stage 0, gene-positive but phenotypically silent; Stage 1, no symptoms, but hyperreflexia and/or abnormal electroretinograms; Stage 2, symptoms and signs progress modestly; and Stage 3, rapid clinical progression. CAG repeat length correlated inversely with age of onset of visual or motor signs (r?=??0.74, p?=?0.002). Stage 3 rate of progression did not differ between cases (p?=?0.18). Electroretinograms correlated with Brief Ataxia Rating Scale score and were a biomarker of disease onset and progression. All symptomatic patients developed gait ataxia, extremity dysmetria, dysarthria, dysrhythmia, and oculomotor abnormalities. Funduscopy revealed pale optic discs and pigmentary disturbances. Visual acuity declined to blindness in those with longer CAG expansions. Hyperreflexia was present from Stage 1 onwards. Restless legs syndrome and sensory impairment were common. Neuropathological hallmarks were neuronal loss in cerebellar cortex, deep cerebellar nuclei, inferior olive, and anterior horns of the spinal cord, and axonal loss in spinocerebellar tracts, dorsal nerve roots, and posterior columns. Retinal pathology included photoreceptor degeneration and disruption of retinal pigment epithelium. Spinocerebellar ataxia type 7 evolves through four clinical stages; neuropathological findings underlie the clinical presentation; electroretinograms are a potential biomarker of disease progression.     

Intérêt d’une unité mobile multidisciplinaire extra-hospitalière de neuro-psychogériatrie intervenant en EHPAD : analyse de 288 interventions

IntroductionGeriatrics Mobile Units are a new organisation operating in nursing homes. Their mission is to propose globally oriented neuro-psychiatric and geriatric care. The purpose of the study is to assess their activity and impact over a 21-month period.MethodA prospective single center study of UMNPG's data including intervention characteristics, patient characteristics, recommendations and reassessment after intervention. The Neuropsychiatric Inventory Nursing Home version (NPI-NH) was measured during intervention and reassessed after 30 days (Student's t-test).ResultsFrom March 2014 to December 2015, UMNPG conducted 288 interventions mainly for medical advices (81%), clinical assessments (54%) and health care team support (46%). The average age was 84.6 ± 7.3 years, 73.3% of whom were women. The patients were dependent (62% of GIR 1 or 2) with dementia (60%) and under several medications (83.7%). The symptoms were mainly agitation/aggression (76.4%), anxiety (75%), depression (66.7%), irritability (60.4%), aberrant motor behaviour (55.9%) and delusions (48.6%). The main proposals of UMNPG were a change in treatment (79.5%), a health care team support (85.4%) and hospitalization (8.4%). The rate of follow-up on recommendation was 83% on the 15th day and 80% on the 30th day. The rate of avoided hospitalizations was 16%. The average NPI-NH decreased (on day 0 NPI = 50 ± 19.2; on day 30 NPI = 33.9 ± 19.6, p < 0.001).ConclusionUMNPG-EHPAD intervenes for frail elderly residents with multiple disorders in crisis situations. Medical recommendations help to support people in nursing homes and decrease NPI-NH. UMNPG-EHPAD is part of geriatric network strengthening the city/hospital connection.