Systeme Nerveux Research Articles

Jules and Augusta Dejerine: Crossing biographies

Jules and Augusta Dejerine: Crossing biographies

The historical background of Jules and Augusta Dejerines’ work

The historical background of Jules and Augusta Dejerines’ work

Sonic Hedgehog-induced Proliferation Requires Specific Gα Inhibitory Proteins

Proliferation of cerebellar granular neuronal precursors (CGNPs) is mediated by Sonic Hedgehog (Shh), which activates the Patched and Smoothened (Smo) receptor complex. Although its protein sequence suggests that Smo is a G protein coupled receptor (GPCR), the evidence that this receptor utilizes heterotrimeric G proteins as downstream effectors is controversial. In Drosophila, Gα(i) is required for Hedgehog (Hh) activity, but the involvement of heterotrimeric G proteins in vertebrate Shh signaling has not yet been established. Here, we show that Shh-induced proliferation of rat CGNPs is enhanced strongly by the expression of the active forms of Gα(i/o) proteins (Gα(i1), Gα(i2), Gα(i3), and Gα(o)) but not by members of another class (Gα(12)) of heterotrimeric G proteins. Additionally, the mRNAs of these different Gα(i) members display specific expression patterns in the developing cerebellum; only Gα(i2) and Gα(i3) are substantially expressed in the outer external granular layer, where CGNPs proliferate. Consistent with this, Shh-induced proliferation of CGNPs is reduced significantly by knockdowns of Gα(i2) and Gα(i3) but not by silencing of other members of the Gα(i/o) class. Finally, our results demonstrate that Gα(i2) and Gα(i3) locate to the primary cilium when expressed in CGNP cultures. In summary, we conclude that the proliferative effects of Shh on CGNPs are mediated by the combined activity of Gα(i2) and Gα(i3) proteins.

Laryngospasm as preceding symptom of amyotrophic lateral sclerosis

A 62-year-old man presented with paroxysmal sensation of suffocation, each time lasting for about 10 s. They were not triggered by body position, physical exercise, or sleep, and there was no associated dysphagia, dysphonia, cough, stridor, or pyrosis. Clinical examination was normal. A diagnosis of laryngospasm was evoked. Electromyography (EMG), including bulbar exploration, brain and cervical spine MRI, direct laryngoscopy (performed in absence of clinical symptoms), and esophagogastroscopy were normal. Laryngospasms persisted after gastric antisecretory medication. CAG repeat length in the androgen receptor gene, in search for Kennedy’s disease, was normal. One year after laryngospasm onset, progressive distal left arm weakness occurred. Clinical examination revealed left thenar, hypothenar, and interossei muscle weakness and atrophy, fasciculations in both upper limbs and tongue, generalized deep tendon hyperreflexia, left-sided Babinski sign, hypernasal voice, brisk jaw-jerk, and emotional lability. EMG showed reduced compound motor action potential amplitude in the left arm without conduction block on nerve conduction studies, with normal F-wave and sensory nerve conduction studies. Electromyographical exploration showed diffuse fibrillations and positive sharp waves at rest in the left upper limb, both lower limbs, and paravertebral muscles, together with large, long and polyphasic muscle unit potentials during muscle contraction. EMG of bulbar muscles was normal. Positron emission tomography with fludeoxyglucose F 18 showed a severe hypometabolism of bilateral temporal and frontal lobes and anterior cingular region. A diagnosis of amyotrophic lateral sclerosis (ALS) was made in presence of El Escorial criteria. Functional pulmonary exploration was normal. Frequency of laryngospasm remained stable the first year following ALS diagnosis. Laryngospasm is clinically defined as a sudden-onset sensation that air cannot be moved in or out, usually followed by inspiratory stridor, due to a rapid and forceful contraction of the laryngeal sphincter [1]. It sometimes results in loss of consciousness making urgent tracheostomy necessary [2]. A gastroesophageal reflux disease is found in 80% of patients with laryngospasm [3]. ALS is defined by progressive degeneration of upper and lower motor neurons. One-third of ALS patients present with bulbar symptoms [4]. In a large cohort of 44 ALS patients with initial bulbar symptoms, none had isolated laryngospasm [5]. Laryngospasm is reported in 2% of ALS patients in early clinical stage, but seems to be more frequent in later stages of the disease [6, 7]. Kennedy’s disease (KD) is characterized by lower motor neuron dysfunction with predominant bulbar involvement. Laryngospasms have been described in 47% in a series of 49 KD patients [6]. All of these patients had bulbar involvement and 65% had pyrosis. Laryngospasm was observed in all stages of KD and there was no increase in frequency of laryngospasm in later stages of the disease. In two-thirds of the patients with laryngospasm and associated pyrosis, laryngospasm was reduced by gastric antisecretory and prokinetic drugs. Our presented patient is the first case of laryngospasm preceding ALS diagnosis. Laryngospasm was probably related to ALS since there were no arguments for G. Fourcade (&) G. Castelnovo D. Renard P. Labauge Department of Neurology, CHU Nimes, Service des Explorations Fonctionnelles du Systeme Nerveux, Hopital Caremeau Place du Pr Debre, 30029 Nimes Cedex 9, France e-mail: n.fourcade@wanadoo.fr

On the structure and functional relations of the optic thalamus. By Ernest Sachs, A.B., MD, of New York. From Sir Victor Horsley's Laboratory, Department of Pathological Chemistry, University College, London. Brain 1909: 32; 95 186.

Acknowledging the roles of Sir Victor Horsley and Dr R.H. Clarke in suggesting the study and providing the necessary apparatus (see Brain 2007: 130; 1449 – 1452), Dr Sachs starts with an historical account of what is currently known concerning the anatomy and physiology of the thalamus, leaning much on the historical account by Gustave Roussy (La couche optique, 1907). Prior to the pioneering work of Hermann Nothnagel (1874), who first lesioned the thalamus with fluid, discrete but crude areas of damage were created by mechanical probes and electrolytic lesions. This work displaced the erroneous idea dating from Francois Magendie (La systeme nerveux, 1841) that the thalamus is the motor centre to one placing this ganglion as the unconscious reflex centre that relays stimuli from the periphery to form motor images that are the basis for voluntary action. Gradually, through a series of claims (dubious and more plausible), was built up an impressive catalogue of brain functions in which the thalamus plays a role—motor, sensory, autonomic, visual, auditory and behavioural (including a ‘screaming centre’, described by Vladimir Bechterew as ‘Geschrei wie es auf Schmerz auftritt’). Others defined thalamic anatomy but could not agree on the number of nuclei, each having discrete connections with parts of the cortex. Notwithstanding the authority of Constantin von Monakow (1895), Heinrich Obersteiner (1901), Santiago Ramon y Cajal (1902) and Otto Marburg (1904), after spending time in Vienna with Professor Obersteiner and, with help from Horsley, Dr Sachs proposes a classification of seven main thalamic nuclei (anterior, medial, lateral, ventral, centre median, arcuate and pulvinar). Now, he rehearses the state of knowledge concerning connectivities: thalamo-cortical, cortico-thalamic, thalamo-striate and a variety of descending thalamo-fugal projections to the brain stem and medulla but no further. But there is work yet to be done in reconciling the true number …

Freud's Comparative Study of Hysterical and Organic Paralyses

From October 1885 until February 1886, Sigmund Freud (1856-1939) visited Paris to work with Jean-Martin Charcot (1825-1893) at the Salpetriere. His original plan was to continue his neuropathologic studies. During the first months of his stay in Paris, he was disappointed and considered returning to Vienna. His feelings changed after personally meeting Charcot, to whom he proposed translating the third volume of Lemons sur les Maladies du Systeme Nerveux into German. Interestingly, 10 of the lectures from this book were on traumatic hysteria, the reason why Freud added particularly on hysteria to the original French title. 1 Freud was much impressed by Charcot, and his original purpose, the study of neuropathology, changed. As he wrote to Carl Koller (1857-1944) in 1886, I found Charcot there, a teacher such as I had always imagined. 2(p30) In January and February 1886, he was a frequent guest at Charcot's hotel at Boulevard St Germain 217, joining the other students, including Joseph Babinski, Pierre Marie, and Georges Gilles de la Tourette. At the time, he admired Charcot and later on he even named his first born Jean-Martin. 2-4

Distribution of substance P‐like immunoreactive retinal ganglion cells and their pattern of termination in the optic tectum of chick (Gallus gallus)

Substance P-like immunoreactive (SP-LI) neurons were identified within the inner nuclear layer and ganglion cell layer of the chick retina. The SP-LI cells in the inner nuclear layer consisted of several subtypes of neurons, differing in soma size and dendritic arborization. In the ganglion cell layer a population of moderately labelled SP-LI neurons was also present. About 6-9 microns in diameter and spaced 50-80 microns apart, they formed a regular array across the entire retina, with a density of about 400 cells/mm2 in the superior temporal retina, declining to less than 100 cells/mm2 in the peripheral retina. The total number of SP-LI cells in the ganglion cell layer was approximately 75,000. Individual axons could be followed toward the optic nerve head. Lesions near the optic nerve head resulted in axotomy of ganglion cells within a limited portion of the retina. Two days of postaxotomy there were numerous SP-LI swellings in the proximal segments of axotomized axons. SP-LI neurons in the axotomized zone were larger, more numerous, and showed increased staining of their processes. Fourteen days following a retinal lesion, there was depletion of all SP-LI cells in the ganglion cell layer within the axotomized zone, but the SP-LI neurons in the inner nuclear layer were not noticeably affected. Following a localized injection of rhodamine-coupled latex beads into the optic tectum, a population of retinal ganglion cells (RGCs) in the contralateral retina was retrogradely labelled. Many of these cells also exhibited SP-like immunoreactivity. Examination of the optic tectum indicated the presence of SP-LI fibres in laminae 2-13 (nomenclature of Cajal: Histologie du Systeme Nerveux. Vol. 2. Paris: Maloine, '11), with immunoreactive terminal regions present mainly in laminae 2-4, 7, and 9-13. SP-LI cell bodies were found predominantly in laminae 10-12 and 13. Fourteen days following a retinal lesion, SP-LI processes and terminals were depleted from laminae 2 and 3. Immunoreactive cells and processes in the remaining laminae of the optic tectum were not noticeably altered. The present report confirms the existence of SP-LI retinal ganglion cells in the chick retina and demonstrates their contribution to lamina specific SP-LI arborization in the optic tectum.

Les myoclonies dans les maladies degeneratives du systeme nerveux central chez l'enfant

The main degenerative disorders of the central nervous system associated with myoclonias in infancy, childhood and adolescence are briefly reviewed as a function of the usual age of onset. In neonates, non-ketotic hyperglycinemia is most common; whereas in infants and young children, the gangliosidoses and the various types of ceroid-lipofuscinosis are the main progressive myoclonic encephalopathies. In older children and adolescents, Lafora disease and the ‘degenerative’ progressive myoclonus epilepsy are in first place. The nosology of the degenerative types of progressive myoclonus epilepsy is briefly discussed. Attention is drawn to the fact that a majority of the myoclonic epilepsies do not belong to the group of degenerative disorders. Their individual and genetic prognosis is, consequently, much better. Les principales maladies dégénératives du système nerveux central dans l'enfance et l'adolescence associees à des myoclonies sont envisagées en fonction de leur âge de début usuel. Chez le nouveau-né, l'hyperglycinémie sans cétose est l'affection la plus fréquente, cependant que chez le nourrisson et le jeune enfant, les gangliosidoses et les différentes formes de céroïde-lipofuschinoses sont les principales encéphalopathies myocloniques progressives. Chez le grand enfant et l'adolescent, la maladic de Lafora d'une part, les épilepsies-myoclonies «dégénératives, sans aspect morphologique de surcharge, de l'autre, occupent la première place. La situation nosologique des épilepsies-myoclonies dégénératives, en particulier du syndrome de Ramsay-Hunt , est brièvement discutée. L'attention est attirée sur le fait que la majorité des épilepsies avec myoclonies n'appartiennent pas au groupe des affections dégénératives et comportent, en conséquence, un pronostic individuel et génétique beaucoup moins défavorable.

A COMPARISON OF THE SYMPTOMS, PHYSICAL AND LABORATORY FINDINGS OF MYXEDEMA AND PERNICIOUS ANEMIA; WITH A REPORT OF THREE CASES

Article1 November 1936A COMPARISON OF THE SYMPTOMS, PHYSICAL AND LABORATORY FINDINGS OF MYXEDEMA AND PERNICIOUS ANEMIA; WITH A REPORT OF THREE CASESJAMES A. GREENE, M.D.JAMES A. GREENE, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-10-5-622 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThe clinical manifestations of myxedema and pernicious anemia are sufficiently alike to account for the fact that the former disease is frequently mistaken for the latter. The similarities and differences of the two diseases, however, have not been emphasized. The purpose of this report is (1) to compare the clinical manifestations of the two diseases, (2) to present two cases of myxedema in which it was difficult to exclude pernicious anemia, and (3) to report another case of pernicious anemia and myxedema. The data were obtained from the records of 100 unselected cases of pernicious anemia and 33 cases of...Bibliography1. ALT HL: The metabolism in pernicious anemia, Arch. Int. Med., 1929, xliii, 488-503. CrossrefGoogle Scholar2. BALDRIDGEBARER CWA: Studies on the relationship between oxygen consumption and nitrogen metabolism. I. In pernicious anemia, Jr. Clin. Invest., 1931, x, 529-543. CrossrefGoogle Scholar3. CASTLE WB: Observations on the etiologic relationship of achylia gastrica to pernicious anemia. I. The effect of the administration to patients with pernicious anemia of the contents of the normal human stomach recovered after the ingestion of beef muscle, Am. Jr. Med. Sci., 1929, clxxviii, 748-764. CrossrefGoogle Scholar4. STURGISISAACS CCR: Treatment of the anemia of myxedema with liver extract, Proc. Cent. Soc. Clin. Res., Jr. Clin. Invest., 1929-30, viii, 663. Google Scholar5. MEANSLERMANCASTLE JHJWB: The coexistence of myxedema and pernicious anemia, New Eng. Jr. Med., 1931, cciv, 243-248. CrossrefGoogle Scholar6. BALDRIDGEGREENE CWJA: Absence of response of anemia of myxedema to liver extract, Proc. Soc. Exper. Biol. and Med., 1934, xxxi, 1035-1037. CrossrefGoogle Scholar7. LERMANMEANS JJH: Treatment of the anemia of myxedema, Endocrinology, 1932, xvi, 533-540. CrossrefGoogle Scholar8. MUSSIO-FOURNIER JC: Troubles du systeme nerveux dans le myxoedeme, Encephale, 1933, xxviii, 45-62; ibid., 1933, xxviii, 137-159. Google Scholar9. BRIGGS : Cited by Lisser and Anderson.10 Google Scholar10. LISSERANDERSON HEM: Three cases of adult myxedema in women, reported for the purpose of calling attention to their widely different symptomatology and clinical findings, Endocrinology, 1931, xv, 365-381. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Iowa City, Iowa*Received for publication August 10, 1936.From the Department of Internal Medicine, State University of Iowa Medical School. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byAssociation of Gastric Carcinoma with Idiopathic Late-Onset Immunoglobulin DeficiencyPAUL E. HERMANS, M.D., F.A.C.P., KENNETH A. HUIZENGA, M.D.Megaloblastic Anemia Secondary to Folate Deficiency Associated with HypothyroidismJOHN D. HINES, M.D., CHARLES H. HALSTED, M.D., ROBERT C. GRIGGS, M.D., JOHN W. HARRIS, M.D.DEFICIENCY OF VITAMIN B12 IN HYPOTHYROIDISMNeurologic Manifestations of MyxedemaCoincidental Myxoedema and Pernicious Anaemia with Subsequent Development of Polycythaemia after Vitamin B12 TherapyVERONICA HALMOS, M.D., F.R.C.P.(C), ROBERT VOLPÉ, M.D., F.R.C.P.(C)Hypothyroidism with anemia demonstrating abnormal vitamin B12 absorption 1 November 1936Volume 10, Issue 5Page: 622-627KeywordsPernicious anemiaResearch laboratories ePublished: 1 December 2008 Issue Published: 1 November 1936 PDF downloadLoading ...

The Structure of Nerve Cells

THE Spanish histologist, Dr. S. R. Cajal, on p. 9 of his book “Les nouvelles idees sur la structure du Systeme nerveux,” translated into French by Dr. L. Azoulay, makes the following assertion:—“Les cellules nerveuses sont des unites independantes, ne s'anastomosant jamais ni par leurs rameaux protoplasmiques, ni par leurs expansions nerveuses ou cylindres axes.” (The italics are mine.) I venture to bring this statement forward because recently I have discovered that it is not universally correct; in sections of the medulla oblongata of a young snake, Tropidonotus natrix, prepared according to Cox's modification of Golgi's corrosive sublimate process, I have found a pair of cells on the ventral edge distinctly united together by a protoplasmic process, or, as I would propose to term it, a dendrite. Cox's mercurial method is so far better than the chrom-osmium-silver method, inasmuch as the preparations made by it keep much longer.