Movement Disorders

This table demonstrates the principle tremor syndromes, including the relevant frequencies and relationships to rest, posture, and movement. (Contributed by Dr. Maurice Hanson. Reproduced from: Deuschl G, Bain P, Brin M. Consensus statement of the Movement Disorder Society on Tremor. Ad Hoc Scientific Committee. Mov Disord. 1998;13 Suppl 3:2-23.)

(Contributed by Dr. Stewart Factor.)

(Contributed by Dr. Stewart Factor.)

(Public domain.)

Private laboratory device. (Contributed by Dr. Cibele Dal Fabbro.)

Private laboratory device. (Contributed by Dr. Cibele Dal Fabbro.)

This young man develops right hemidystonia after running, and it worsens as he continues to run. He was found to have a de novo pathogenic variant in SLC2A1. (Contributed by Dr. Mariam Hull.)

Brief sequence of repeated activation of the right anterior tibialis can be seen during relaxed wakefulness, at a frequency of about 1 Hz.

Legend: EOG: electrooculogram, Mylo: mylohyoideus muscle,Tib. Ant.: tibialis anterior muscle, ECG: electrocardiogram, Abd. Resp.: abdominal respirogram, R.: right, L.: left.

(Used with permission from Elsevier. From: Baldelli L, Provini F. Fragmentary hypnic myoclonus and other isolated motor phenomena of sleep. Sleep Med Clin 2021;16(2):349-61.)

The EMG channels show alternate contractions in both tibialis anterior muscles at about 1 to 2 Hz.

Legend: EOG: electrooculogram, Mylo: mylohyoideus muscle, Ext. Carpi.: extensor carpi muscle, Tib. Ant.: tibialis anterior muscle, ECG: electrocardiogram, Abd. Resp.: abdominal respirogram, R.: right, L.: left.

(Used with permission from Elsevier. From: Baldelli L, Provini F. Fragmentary hypnic myoclonus and other isolated motor phenomena of sleep. Sleep Med Clin 2021;16(2):349-61.)

The EMG activity originates in the left paraspinalis muscle and then spreads to the more cranial and rostral axial and limb muscles, without any rostro-caudal order as better visible at high paper speed (right part).

Legend: EOG: electrooculogram, Mylo: mylohyoideus muscle,Orbic.: orbicularis muscle,Mass: masseter muscle, Delt.: deltoideus muscle, Wrist Extens.: wrist extensor muscle, Rect. Abd.: rectus abdominis muscle, Parasp.: paraspinalis muscle, Rectus Fem.: rectus femoralis muscle, Tib. Ant.: tibialis anterior muscle, ECG: electrocardiogram, Resp. Thor-Abd.: thoraco-abdominal respirogram, R.: right, L.: left.

(Used with permission from Elsevier. From: Baldelli L, Provini F. Fragmentary hypnic myoclonus and other isolated motor phenomena of sleep. Sleep Med Clin 2021;16(2):349-61.)

Brief, asymmetrical, asynchronous potentials are visible in the explored muscles, sometimes without any associated visible movement.

Legend: EOG: electrooculogram, Mylo.: mylohyoideus muscle, Ext. Carpi: extensor carpi muscle, Tib. Ant.: tibialis anterior muscle, ECG: electrocardiogram, Abd. Resp.: abdominal respirogram, R.: right, L.: left.

(Used with permission from Elsevier. From: Baldelli L, Provini F. Fragmentary hypnic myoclonus and other isolated motor phenomena of sleep. Sleep Med Clin 2021;16(2):349-61.)

PSG recording shows spontaneous propriospinal myoclonus jerks (a) in brief clusters or (b) isolated during relaxed wakefulness preceding sleep onset. (c) The recording of a propriospinal myoclonus jerk shows a slow rostro-caudal propagation of muscular activity. At high paper speed, it is evident that the onset of the EMG activity is in the recti abdominis muscles with ordered spreading to more rostral and caudal muscles (dotted line).

Legend: EOG: electrooculogram, Mylo: mylohyoideus muscle, Mass: masseter muscle, SCM.: sternocleidomastoid muscle, Delt.: deltoideus muscle, Intercostalis: intercostalis muscle, Rect. Abd.: rectus abdominis muscle, Parasp.: paraspinalis muscle, Rectus Fem.: rectus femoralis muscle, Tib. Ant.: tibialis anterior muscle, ECG: electrocardiogram, Abd. Resp.: abdominal respirogram, R.: right, L.: left

Used with permission from Elsevier. From: Baldelli L, Provini F. Fragmentary hypnic myoclonus and other isolated motor phenomena of sleep. Sleep Med Clin 2021;16(2):349-61.

This video demonstrates a woman with malignant catatonia. The first part demonstrates “catalepsy” in which the patient passively adopts the new posture after the examiner elevates her right arm. The second part demonstrates “posturing” in which the patient maintains the fixed posture of the right arm and leg elevation for several minutes. (Contributed by Dr. Pichet Termsarasab.)

The left atrium has dilated and the left ventricle has hypertrophied and may diilate. (Source: Laboratoires Servier, 2016. Creative Commons Attribution-Share Alike 3.0 Unported license, creativecommons.org/licenses/by-sa/3.0/deed.en.)

(Source: Laboratoires Servier, 2016. Creative Commons Attribution-Share Alike 3.0 Unported license, creativecommons.org/licenses/by-sa/3.0/deed.en.)

(Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

There is a double crescendo. The second sound seems reduplicated. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

The murmur occurs throughout diastole, with a gradual increase in intensity as it approaches the first heart sound (S1). No second sound is audible at the apex. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

The place of the murmur and its crescendo character are indicated by the position of the light lines just before the first heart sound (S1) and by their increasing length. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

(Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

Comparison of blood flow during diastole in a normal heart (top) and a heart with mitral stenosis (bottom). In the normal heart, the open mitral valve allows blood to flow from the atrium, and the aortic valve is closed. In the heart with mitral stenosis, blood flowing from the left atrium is obstructed by the thickened and adherent mitral curtains. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

The murmur is heard over the shaded area as well as in the front. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

The murmur is heard over the shaded area as well as in the back. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

Late systolic murmur, which can occasionally be heard in mitral regurgitation. The first sound is clear, and an interval separates the first heart sound and the murmur. Such murmurs generally indicate very slight disease of the valve. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

This represents more severe disease than when the first sound can be heard. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

The heavy lines represent the normal cardiac sounds, and the light lines represent the murmur, which in this case does not replace the first heart sound and characteristically tapers off near the end. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

Comparison of blood flow during systole in a normal heart (top) and a heart with mitral regurgitation (bottom). In the normal heart, the mitral valve is closed during systole, and blood flows through the open aortic valve into the aorta. In the heart with mitral regurgitation, blood flows bidirectionally: retrograde into the left atrium through the abnormal mitral valve and anterograde into the aorta through the aortic valve. (Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

(Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

(Source: Cabot RC. Physical Diagnosis of Diseases of the Chest. Second edition. New York: William Wood and Company, 1903. Public domain.)

Relationship between the heart sounds, muscle contractions, blood flow, position of the valves, and apex in one complete cardiac cycle (either of the right or left side of the heart).

(A) When the first sound is heard, the ventricles contract, the apex strikes the anterior chest wall, the auricular-ventricular (ie, tricuspid and mitral) valves close, the pulmonic and aortic valves open, blood is forced into the pulmonary artery and the aorta, the columnae carnae (ie, the muscular ridges or projections on the walls of ventricles) and cordae tendineae (ie, strong, fibrous connections between the valve leaflets and the papillary muscle) are in tension, and blood flows passively into the auricles from the veins.

(B) When the second heart sound (S2) is heard, pulmonic and aortic valves close, the ventricles relax, blood continues to flow passively into the auricles from the veins, the auricular-ventricular (ie, tricuspid and mitral) valves begin to open, and the apex recedes from the chest wall.

(C) Aortic and pulmonic valves remain closed, the auricular-ventricular (ie, tricuspid and mitral) valves open, and blood flows passively into the ventricles from the auricles and into the auricles from the veins.

(D) The auricles contract, the auricular-ventricular (ie, tricuspid and mitral) valves are thrown wide open, blood flows forcibly from the auricles into the ventricles, the ventricles distend, and the pulmonic and aortic valves remain closed. Then the cycle is repeated. (Source: Slade CB. Physical Examination and Diagnostic Anatomy. Philadelphia and London: W.B. Saunders Company, 1910. Public domain.)

The three positive waves are labeled by convention as waves a, c, and v. The a wave is generated by atrial contraction, the c wave is caused either by transmission of the carotid arterial impulse through the external and internal jugular veins or by the tricuspid valve bulging into the right atrium in early systole, and the v wave is caused the passive increase in pressure and volume of the right atrium as it fills in late systole and early diastole. The descents from a and c are considered x descents, whereas the descent after the v wave is the y descent. (Source: Stanford Medicine. Courtesy of Wikimedia Commons. Public domain.)

The internal jugular vein is exposed on the right after resection of the sternocleidomastoid muscle. The external jugular vein has been transected at the base of the neck on the subject's right but is shown intact external to the sternocleidomastoid muscle on the left. Illustration by Henry Vandyke Carter (1831-1897). (Source: Gray H. Gray's Anatomy. 20th edition. Revised by Warren H Lewis. Philadelphia/New York City: Lea & Febiger, 1918. Public domain.)

A man with congestive heart failure who presented with a markedly elevated jugular venous pressure. The arrow is pointing to the external jugular vein; however, jugular venous pressure is measured by the internal jugular vein, which can also be seen here. (Source: Dr. James Heilman, 2009. Creative Commons Attribution-Share Alike 3.0 Unported license, creativecommons.org/licenses/by-sa/3.0 /deed.en.)

(Source: Wikimedia Commons User:Ksheka. Creative Commons Attribution-Share Alike 3.0 Unported license, creativecommons.org/licenses/by-sa/3.0 /deed.en.)

Two Petri dishes, each filled with trypticase soy agar medium, containing 5% defibrinated sheep's blood. The plate on the left had been stabbed and streaked with an inoculum containing Streptococcus mitis, alpha-hemolytic bacteria, a member of the Viridans group, while the right plate was stabbed with an inoculum containing Group A Streptococcus pyogenes (GAS), a typical beta-hemolytic bacteria. The inoculation was performed using a wire loop, which had been dipped into a primary culture medium. The blood agar plates were incubated in a carbon dioxide-enriched atmosphere at 35°C for 24 hours. All culture organisms were Gram-positive cocci. The alpha-hemolytic organisms on the left plate grew colonies that were surrounded by a hazy, indistinct zone of partial red blood cell (RBC) hemolysis, and the beta-hemolytic bacteria on the right grew colonies surrounded by a clear, colorless zone of complete RBC destruction. Using the "stabbing" method of inoculation makes the qualitative interpretation of the hemolytic reaction much easier, for the results of colonial interactivity with the blood agar medium are much more pronounced, and therefore, easier to discern. (Source: CDC/Dr. Richard R Facklam, 1977. Public Health Image Library, Centers for Disease Control and Prevention, Atlanta, Georgia. Public domain.)

Axial FLAIR T-2 weighted images of the brain showing bilateral hyperintense signals in the thalami and corpus striatum in an 8-year-old girl with Sydenham chorea. (Source: Ali A, Anugwom GO, Rehman U, Khalid MZ, Saeeduddin MO. Sydenham chorea managed with immunoglobulin in acute rheumatic fever. Cureus 2021;13[5]:e14990. Creative Commons Attribution [CC-BY 4.0] license, creativecommons.org/licenses/by/4.0.)

Axial FLAIR T-2 weighted images of the brain showing hyperintense signals in the left frontal lobe in an 8-year-old girl with Sydenham chorea. (Source: Ali A, Anugwom GO, Rehman U, Khalid MZ, Saeeduddin MO. Sydenham chorea managed with immunoglobulin in acute rheumatic fever. Cureus 2021;13[5]:e14990. Creative Commons Attribution [CC-BY 4.0] license, creativecommons.org/licenses/by/4.0.)

Osler was Professor of Clinical Medicine at the University of Pennsylvania from 1884 to 1888 before becoming the first Chairperson and Physician-in-Chief of the Johns Hopkins Department of Medicine in Baltimore. (Source: Collection of the University of Pennsylvania Libraries, Philadelphia. Public domain.)

(Source: Courtesy of the U.S. National Library of Medicine. Public domain.)

From Vanity Fair, October 1902. (Source: Wikimedia Commons. Public domain.)

Photograph by Elliott & Fry, a Victorian photography studio in London. Published in 1909 in LIFE. (Source: Wellcome Collection, London, UK. Creative Commons Attribution 4.0 International [CC BY 4.0] license, creativecommons.org/licenses/by/4.0.)

Photogravure after Lance Calkin (1859-1936), 1895. (Source: Wellcome Collection, London, UK. Public Domain Mark.)

(Source: Guerini V. A history of dentistry from the most ancient times until the end of the eighteenth century. Philadelphia & New York: Lea & Febiger, 1909.)

Three epileptic women each supported by two men. The artwork is widely interpreted as showing the dancing mania of the Middle Ages, referred to as St. John's dance, St. Vitus dance, or La Danse de Saint-Guy. Engraving by Dutch engraver Hendrik Hondius the Younger (1597-1651) in 1642, after Pieter Brueghel (approximately 1525-1569). (Source: Wellcome Collection, London, UK. Public Domain Mark.)

(Source: Wellcome Collection, London, UK. Public Domain Mark.)

(Source: Specht R. Gustav Mahler. Berlin and Leipzig: Schuster and Loeffler, 1913, plate 12. Public domain.)

(Source: Sinkler W. Choreiform affections. In: Dercum FX, editor. A textbook on nervous diseases by American authors. Philadelphia: Lea Brothers, 1895:227-69.)

(Source: La Médecine Illustrée 1880 [December 10.] Public domain. Edited by Dr. Douglas J Lanska.)

(Source: Aubry C. Album Comique de Pathologie Pittoresque, Recueil de Vingt Caricatures Médicales. Paris: Huzard-Courcier for Ambroise Tardieu, 1823. Artist Antoine Chazal (French, 1793-1854). Lithography by Langlumé (French, active 1819-1830). Public domain. Slightly cropped.)

(Source: He C, Zhang P, Li Y, et al. Hemiparkinsonism or hemidystonia with hemiatrophy syndrome: a case series with follow-up. Front Neurosci 2020;14:64. Creative Commons Attribution [CC BY] license, creativecommons.org/licenses/by/2.0.)

(A) Hemiatrophy of the left-sided lower limb. (B) The right lower limb is significantly shorter and smaller than the left lower limb, and the right foot was in an abducted posture. (C) Compared to the right calf, the left calf shows hemiatrophy. (D) Atrophy of the left arm and back muscles. (Source: He C, Zhang P, Li Y, et al. Hemiparkinsonism or hemidystonia with hemiatrophy syndrome: a case series with follow-up. Front Neurosci 2020;14:64. Creative Commons Attribution [CC BY] license, creativecommons.org/licenses/by/2.0.)

(Source: Lenka A, Jankovic J. Peripherally-induced movement disorders: an update. Tremor Other Hyperkinet Mov [N Y] 2023;13:8. Creative Commons Attribution 4.0 International [CC-BY 4.0] license, creativecommons.org/licenses/by/4.0.)

Legend: CBS: corticobasal syndrome, HFS: Hemifacial spasm, HMS: Hemimasticatory spasm, OMD: oromandibular dystonia, PLMT: Painful leg moving toes syndrome. (Source: Lenka A, Jankovic J. Peripherally-induced movement disorders: an update. Tremor Other Hyperkinet Mov [N Y] 2023;13:8. Creative Commons Attribution 4.0 International [CC-BY 4.0] license, creativecommons.org/licenses/by/4.0.)

Sleep hypnogram of a patient with Parkinson disease. Total sleep time is markedly reduced as well as sleep efficiency; frequent and sometimes prolonged infrahypnic awakenings are present throughout the night. All sleep phases are represented; REM latency is increased. (Contributed by Dr. Federica Provini.)

Correlation: r = 0.928, r2 = 0.81. Penicillin was introduced in 1943, in the United States and in Denmark. Both graphs changed from logarithmic to a linear scale. Note: Because of a discontinuity in the US data between 1948 and 1949, related to change of the ICD criteria for diagnosis, the data for mortality before 1949 were adjusted (by Alm) to attempt to compensate for this (Alm 2020). Data for Denmark were from (Steer 2015), and data for the United States are from (Massell 1997). Both graphs changed from logarithmic to a linear scale.

Note: Because of a discontinuity in the US data between 1948 and 1949, related to change of the ICD criteria for diagnosis, the data for mortality before 1949 were adjusted (by Alm) to attempt to compensate for this.

References cited:
Alm PA. Streptococcal infection as a major historical cause of stuttering: data, mechanisms, and current importance. Front Hum Neurosci 2020;14:569519.
Massell BF. Rheumatic Fever And Streptococcal Infection: Unraveling The Mysteries Of A Dread Disease. Boston, MA: Harvard University Press, 1997.
Steer AC. Historical aspects of rheumatic fever. J Paediatr Child Health 2015;51(1):21-7.

LF, 35-year-old white man. Philadelphia Hospital Autopsy. Volume 23, page 1. Physician: Dr. FP Henry. Pathologist: Dr. Karsner. Clinical note: At one time chorea. ... Cyanosis, dyspnea, edema, arrhythmia (extrasystolic), tachycardia, pulsating liver, jugular veins, etc. Signs of double mitral and tricuspid lesion. Died gradually with symptoms of pneumonia. Pathologic diagnosis: Cloudy swelling of heart, with double-sized dilatation. Mitral stenosis; tricuspid insufficiency. Chronic congestion of lungs; hypostatic pneumonia of right; beginning gangrene of left. Cyanotic induration of kidneys; arteriosclerosis; chronic congestion with fatty degeneration of liver, also slight perilobular fibrosis, etc. ... Heart: ... Mitral orifice was not incised; its leaflets are enormously thickened and contracted, showing at the right junction slight calcification; the whole presenting the appearance of the funnel-shaped buttonhole mitral. The chordae tendineae are enormously thickened, retracted and adherent, and are attached to papillary muscles which are much elongated and have sclerotic tips. The fibrosis of the anterior mitral leaflet extends forward along the septum, below the bases of the aortic valves, producing a thickening which extends transversely through the bundle of His. The ventricular cavity is enlarged, contains chicken-fat clot. ... For several years previous to this patient's death the author had from time-to-time opportunities of studying the heart by means of the cardiosphygmograph. Very marked arrhythmia was always present and consisted of the type that Mackenzie describes as "nodal rhythm," the "pulsus irregularis perpetuus" of Hering, but which, according to more recent investigations, is probably due to auricular fibrillation. (Source: Norris GW. Studies in cardiac pathology. Philadelphia and London: W.B. Saunders Company, 1911. Photograph restored and edited by Dr. Douglas J Lanska.)

Jefferson Hospital Laboratories. (Source: DaCosta JC Jr. Principles and Practice of Physical Diagnosis. Third edition. Philadelphia and London: W.B. Saunders Company, 1915. Illustration edited by Dr. Douglas J Lanska.)

Jefferson Hospital Laboratories. (Source: DaCosta JC Jr. Principles and Practice of Physical Diagnosis. Third edition. Philadelphia and London: W.B. Saunders Company, 1915. Illustration edited by Dr. Douglas J Lanska.)

The three-dimensional, computer-generated artistic recreation was based on scanning electron microscopic (SEM) imagery. (Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 22884. Image created by Jennifer Oosthuizen, Medical Illustrator, CDC, 2013. Public domain.)

Digitally colorized scanning electron microscopic (SEM) image depicts four yellow-colored Group A Streptococcus (GAS), Streptococcus pyogenes bacteria, which were atop the surface of neutrophil. (Produced by the National Institute of Allergy and Infectious Diseases [NIAID], 2009. Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 18257. Public domain.)

Petri dish with Streptococcus pyogenes-inoculated trypticase soy agar containing 5% defibrinated sheep's blood that had been streaked and stabbed with a wire loop, which had been dipped into primary culture medium. The blood agar plate (BAP) was incubated in a normal atmosphere at 35°C for 24 hours. In this case, the culture dish grew colonies of Gram-positive, Group A beta-hemolytic streptococci ("GAS") bacteria. The characteristic color changes, including a light-colored halo surrounding each colony in which the red blood cells in the blood agar medium had been destroyed, or hemolyzed, indicated that these bacteria were indeed beta-hemolytic in nature. (Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 8170. Photograph by Dr. Richard R Facklam, CDC, 1977. Public domain.)

(Pappenheim's stain, 900x) (Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 2110. 1954. Public domain.)

(Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 2109. 1979. Public domain.)

The patient exhibited redness and edema of the oropharynx and petechiae on the soft palate. Group A streptococci are spread through direct contact with mucus from the nose or throat of persons who are infected or through contact with infected wounds or sores on the skin. (Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 3183. Photograph by Dr. Heinz F Eichenwald, CDC, 1958. Public domain.)

The patient exhibited redness and edema of the oropharynx and petechiae on the soft palate. Group A streptococci are spread through direct contact with mucus from the nose or throat of persons who are infected or through contact with infected wounds or sores on the skin. (Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 3183. Photograph by Dr. Heinz F Eichenwald, CDC, 1958. Public domain.)

The patient exhibited redness and edema of the oropharynx and petechiae on the soft palate. Group A streptococci are spread through direct contact with mucus from the nose or throat of persons who are infected or through contact with infected wounds or sores on the skin. (Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 3183. Photograph by Dr. Heinz F Eichenwald, CDC, 1958. Public domain.)

(Source: James Heilman MD. Creative Commons Attribution-Share Alike 3.0 Unported License, https://creativeco mmons.org/licenses/by-sa/3.0/deed.en.)

(Source: James Heilman MD. Creative Commons Attribution-Share Alike 3.0 Unported License, https://creativeco mmons.org/licenses/by-sa/3.0/deed.en.)

(Source: Somers LS. The diagnosis and treatment of membranous tonsillitis. Int Clin 1906;1[16th series]:41-56.)

(Source: Somers LS. The diagnosis and treatment of membranous tonsillitis. Int Clin 1906;1[16th series]:41-56.)

(A) Zone of swollen connective tissue. (B) Zone of cellular exudation. (C) Zone of necrosis and plastic exudation. (D) Muscle fibers. (Source: Poynton FJ. Clinical lectures on rheumatic fever. III. The parallelism between the clinical symptoms and the pathological lesions of rheumatic fever. Int Clin 1904;4[13th series]:95-111.)

(A) Zone of swollen connective tissue of the valve. (B) Zone of cellular exudation. (C) Area of necrosis in the vegetation. (Source: Poynton FJ. Clinical lectures on rheumatic fever. III. The parallelism between the clinical symptoms and the pathological lesions of rheumatic fever. Int Clin 1904;4[13th series]:95-111.)

(A) Zone of swollen connective tissue. (B) Zone of cellular exudation. (C) Zone of necrosis and fibrino-cellular exudation. (D) Cardiac muscle. (Source: Poynton FJ. Clinical lectures on rheumatic fever. III. The parallelism between the clinical symptoms and the pathological lesions of rheumatic fever. Int Clin 1904;4[13th series]:95-111.)

(A) The fibrous tissue. (Source: Poynton FJ. Clinical lectures on rheumatic fever. II. Clinical evidences of myocardial damage in rheumatic fever. Int Clin 1903;3[13th series]:226-41.)

(A) A blood vessel with much perivascular fibrosis. (Source: Poynton FJ. Clinical lectures on rheumatic fever. II. Clinical evidences of myocardial damage in rheumatic fever. Int Clin 1903;3[13th series]:226-41.)

(A) Cellular exudation. (Source: Poynton FJ. Clinical lectures on rheumatic fever. II. Clinical evidences of myocardial damage in rheumatic fever. Int Clin 1903;3[13th series]:226-41.)

(Source: Poynton FJ. Clinical lectures on rheumatic fever. II. Clinical evidences of myocardial damage in rheumatic fever. Int Clin 1903;3[13th series]:226-41.)

In this photograph, the necrotic tissue contains myriads of diplococci. (Source: Poynton FJ. Clinical lectures on rheumatic fever. I. Endocarditis in childhood considered as a symptom of infective diseases. Int Clin 1903;3[13th series]:181-94.)

In this photograph, there is much necrotic tissue but no diplococci, and at the base, reparative changes are commencing. (Source: Poynton FJ. Clinical lectures on rheumatic fever. I. Endocarditis in childhood considered as a symptom of infective diseases. Int Clin 1903;3[13th series]:181-94.)

This image of a sectioned human heart, extracted at the time of this patient’s autopsy, depicts a superior view of the aortic valve, which had been damaged due to a case of rheumatic heart disease. Note the thickened, fused valve leaflets and the severe stenosis, or stricture of the aortic lumen, all due to this disease. (Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 848. Photograph by Dr. Edwin P Ewing Jr, CDC, 1973. Public domain.)

Sectioned human heart, extracted during a patient’s autopsy, reveals severe thickening of the mitral valve, thickened chordae tendineae, and the hypertrophic left ventricular myocardium, all due to a case of rheumatic heart disease. (Courtesy of the Centers for Disease Control and Prevention Public Health Image Library. Image 847. Photograph by Dr. Edwin P Ewing Jr, CDC, 1972. Public domain.)

BS, 21-year-old black woman. Philadelphia Hospital Autopsy. Volume 18, page 154. Physician: Dr. Hughes. Pathologist: Dr. McConnell. The woman had acute rheumatic fever 4 years previously and again 2 years later. Beginning with the first episode of rheumatic fever, she developed exertional dyspnea and dizziness. She was tachycardic (heart rate 110), and her heart was enlarged to percussion. A presystolic thrill was palpated within the apex, where a double mitral murmur was heard. Both second sounds were accentuated, especially the pulmonic. Death occurred from progressive congestive heart failure. Pathologic diagnosis: chronic mitral and tricuspid endocarditis; chronic pleurisy; right pleural effusion; congestion and edema of the right lung; bilateral pulmonary infarcts; diffuse nephritis. The heart weighed 340 gm. The mitral orifice barely admitted the tip of the index finger. The tricuspid orifice (the lowest on the plate) is small; leaflets thickened, and on free edges are numerous vegetations. On one leaflet, there is a vegetation that is 1 inch in height, extending directly into the opening. (Source: Norris GW. Studies in cardiac pathology. Philadelphia and London: WB Saunders Company, 1911. Photograph restored and edited by Dr. Douglas J Lanska.)

The dotted line represents the average monthly rainfall at Greenwich. The y-axis for rainfall is on the right, and the y-axis for admissions is on the left. (Source: Gabbett HS. On the seasons of the year and the prevalence of acute rheumatism. Lancet 1883;2:675-7, 720-2. Illustration restored and edited by Dr. Douglas J Lanska.)

The y-axis for first attacks is on the right, and the y-axis for all attacks is on the left. Number of first attack = 791. Total number of all cases = 1908. (Source: Church WS. Acute rheumatism, or rheumatic fever. In: Albutt TC, editor. A System of Medicine, by Many Writers. New York: The MacMillan Co., London: MacMillan & Co., 1898;4:1-37. Illustration restored and edited by Dr. Douglas J Lanska.)

The y-axis for first attacks is on the right, and the y-axis for all attacks is on the left. Number of first attack = 659. Total number of all cases = 2000 (Church 1898). Church's graph was based on the work of British pathologist Henry Singer Gabbett (1851-1926) and, in particular, was used the summary data from Gabbett's table in his 1883 article in Lancet (Gabbett 1883; 1884).

References:
Gabbett HS. On the seasons of the year and the prevalence of acute rheumatism. Lancet 1883;2:675-7, 720-2.
Gabbett HS. Seasonal variations of rheumatism. Pop Sci Month 1884;25:429.
Church WS. Acute rheumatism, or rheumatic fever. In: Albutt TC, editor. A System of Medicine, by Many Writers. New York: The MacMillan Co., London: MacMillan & Co., 1898;4:1-37.

(Illustration restored and edited by Dr. Douglas J Lanska.)

Church was a successful physician to St Bartholomew's Hospital in London, president of the Royal College of Physicians from 1899 to 1905, and president of the Royal Society of Medicine from 1907 to 1909 and also in 1893. (Courtesy of the Wellcome Collection, London, England. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0.)

Photograph by G Jerrard. (Courtesy of the Wellcome Collection, London, England. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0.)

Proliferation and cell infiltration of subendothelial fibrous tissue, resembling the histologic features of subcutaneous nodules. (Source: Cheadle WB. The acute rheumatism of childhood. In: Albutt TC, editor. A System of Medicine, by Many Writers. New York: The MacMillan Co., London: MacMillan & Co., 1898;4:38-55.)

Active proliferation and cell infiltration of fibrous tissue in a 7.5-year-old boy with acute rheumatic fever. Clinical features included chorea, arthritis, endocarditis, pericarditis, pleurisy, and subcutaneous nodules. Hospital for Sick Children, Great Ormond St., London. Around August 6, 1888. (Source: Cheadle WB. The acute rheumatism of childhood. In: Albutt TC, editor. A System of Medicine, by Many Writers. New York: The MacMillan Co., London: MacMillan & Co., 1898;4:38-55.)

Boy, aged 4 years and 3 months. Clinical features included rheumatic nodules, erythema marginatum, chorea, a "double mitral murmur," and arthritis. From the Hospital for Sick Children, Great Ormond St., London. Under the care of English pediatrician William Butler Cheadle (1836-1910). (Source: Cheadle WB. The acute rheumatism of childhood. In: Albutt TC, editor. A System of Medicine, by Many Writers. New York: The MacMillan Co., London: MacMillan & Co., 1898;4:38-55. Illustration restored and edited by Dr. Douglas J Lanska.)

(Source: Wikimedia Commons. User:Adsie on September 11, 2020. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0.)

(Source: Wikimedia Commons. User:Adsie on September 11, 2020. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0.)

Watercolor drawing by Thomas Godart (1821-1887). Godart was employed as Medical Artist at the medical school at St Bartholomew's Hospital. (Source: St. Bartholomew's Hospital and Museum, commonly known as "Barts," a teaching hospital in London. Courtesy of the Wellcome Collection, London, England. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0. Illustration restored and edited by Dr. Douglas J Lanska.)

Watercolor drawing by Thomas Godart (1821-1887). Godart was employed as Medical Artist at the medical school at St Bartholomew's Hospital. (Source: St. Bartholomew's Hospital and Museum, commonly known as "Barts," a teaching hospital in London. Courtesy of the Wellcome Collection, London, England. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0. Illustration restored and edited by Dr. Douglas J Lanska.)

Watercolor drawing by Thomas Godart (1821-1887). Godart was employed as Medical Artist at the medical school at St Bartholomew's Hospital. (Source: St. Bartholomew's Hospital and Museum, commonly known as "Barts," a teaching hospital in London. Courtesy of the Wellcome Collection, London, England. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0. Illustration restored and edited by Dr. Douglas J Lanska.)

(Source: Wikimedia Commons. Photomicrograph by User: Nephron. Creative Commons Attribution-Share Alike 3.0 Unported License, https://creativecommons.org/licenses/by-sa/3.0/deed.en.)

(Source: Wikimedia Commons. Photomicrograph by User: Nephron. Creative Commons Attribution-Share Alike 3.0 Unported License, https://creativecommons.org/licenses/by-sa/3.0/deed.en.)

(Source: Wikimedia Commons. Photomicrograph by User: Nephron. Creative Commons Attribution-Share Alike 3.0 Unported License, https://creativecommons.org/licenses/by-sa/3.0/deed.en.)

This necrotic lesion surrounded by palisaded histiocytes is present on the pleural surface. (Source: Yale Rosen on June 30, 2012. Creative Commons Attribution-Share Alike 2.0 Generic License, https://creativecommons.org/licenses/by-sa/2.0/deed.en.)

Hematoxylin and eosin, 10x magnification. (Source: Heard MA, Green MC, Royer M. Acute rheumatic fever: a review of essential cutaneous and histological findings. Cureus 2021;13[1]:e12577. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0.)

Hematoxylin and eosin, 10x magnification. (Source: Heard MA, Green MC, Royer M. Acute rheumatic fever: a review of essential cutaneous and histological findings. Cureus 2021;13[1]:e12577. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0.)

Hematoxylin and eosin, 2x magnification. (Source: Heard MA, Green MC, Royer M. Acute rheumatic fever: a review of essential cutaneous and histological findings. Cureus 2021;13[1]:e12577. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0.)

(Source: Heard MA, Green MC, Royer M. Acute rheumatic fever: a review of essential cutaneous and histological findings. Cureus 2021;13[1]:e12577. Creative Commons Attribution [CC-BY 4.0] License, https://creativecommons.org/licenses/by/4.0.)

(Source: Centers for Disease Control and Prevention, https://www.cdc.gov/groupastrep/diseases-hcp/acute-rheumatic-fever.html. Accessed June 5, 2022. Public domain.)

Photographs by German internist Friedrich Pineles (1868-1936) at the Medical Clinic in Heidelberg, c 1915. (Source: Pineles F. Hyperkinetic diseases: Infectious chorea. In: Burr CW, editor. Text-Book on Nervous Diseases. Philadelphia: P. Blakiston's Son & Co., 1915:811-6. Illustration restored and edited by Dr. Douglas J Lanska.)

Print. Dutch engraver and printmaker Jacob Houbraken (1698-1780), Amsterdam, after Sir Peter Lely (1618-1680). Lely was a painter of Dutch origin whose career was nearly all spent in England, where he became the dominant portrait painter to the court. (Courtesy of the Rijkmuseum, Amsterdam, The Netherlands. Public domain. Illustration restored, edited, and rematted by Dr. Douglas J Lanska.)

Oil on canvas. (Courtesy of the Wellcome Collection, London, England. Public domain. Illustration restored, edited, and rematted by Dr. Douglas J Lanska.)

Voice of patient with adductor laryngeal dystonia at peak voice 3 weeks after chemodenervation. (Contributed by Dr. Tanya K Meyer.)

Voice of patient with adductor laryngeal dystonia at baseline before chemodenervation. (Contributed by Dr. Tanya K Meyer.)

Patient diagnosed with chronic constriction variant of adductor laryngeal dystonia undergoes LEMG-guided botulinum toxin A injection. (Contributed by Dr. Tanya K Meyer.)

Patient diagnosed with chronic constriction variant of adductor laryngeal dystonia has a strained voice just before LEMG-guided botulinum toxin A injection. (Contributed by Dr. Tanya K Meyer.)

Photo taken September 1985 in Hamburg, Germany. Top: Dr. Stanley Fahn (Co-Founder and President, 1988-1991). Bottom row, left to right: Dr. Eduardo Tolosa (President, 1997-1998), Dr. Andrew Lees (President, 2005-2006), Dr. Joseph Jankovic (President, 1995-1996), and Dr. David Marsden (Co-Founder and President, 1991-1994).

This woman has primary generalized dystonia manifested by dystonic camptocormia and “dromedary” gait. The term "camptocormia" (literally, bent tree trunk) is believed to have been coined in 1914 by Alexandre Achille Souques, a student of Charcot and leading French neurologist of his day. Camptocormia, first reported in soldiers in World War I and believed to represent a psychogenic disorder, is now recognized as a form of axial dystonia seen in patients with isolated dystonia or in patients with Parkinson disease and other movement disorders (Azher and Jankovic 2005; Jankovic 2010). It now has an extensive differential diagnosis. "Dromedary" refers to the desert-dwelling, single-humped camel, which avoids trotting or galloping to conserve energy, instead ambling with a peculiar rolling gait (like the rolling of a boat on rough waters) that results from its lifting both forelegs and hind legs on one side together. The term may have been first used in neurology by Hermann Oppenheim, the acknowledged leader of German neurology, at the end of the 19th century. (Contributed by Dr. Joseph Jankovic.)

This woman with generalized dystonia has a coarse postural tremor of the right hand. Dystonia affecting the trunk is evident when she walks. Her daughter with more severe, generalized dystonia has severe truncal and limb dystonia. Lower limb dystonia makes walking almost impossible. She is markedly improved after bilateral pallidotomy. Some leg dystonia is still present while walking. Both mother and daughter had a GAG deletion in the gene coding for torsinA on chromosome 9, confirming the diagnosis of DYT-TOR1A dystonia. (Contributed by Dr. Joseph Jankovic.)

This young man with DYT-TOR1A generalized dystonia has severe dystonia of the trunk and neck. He is moderately improved by bilateral globus pallidus deep brain stimulation. Jerky head movements are related to electrical stimulation. (Contributed by Dr. Joseph Jankovic.)

A 46-year-old man shows right-side postural tremors causing problems in writing. Patient had been taking sodium valproate for 4 years following two episodes of seizures. Tremors are present on right side, simulating parkinsonism. He did not have akinesia and rigidity. (Contributed by Dr. Ravindra Kumar Garg.)

This 28-year-old woman presented at 11 years of age with toe walking. She has mild intellectual impairment, emotional difficulties, and anger control problems; severe hypokinetic dysarthria, hypophonia, and drooling; marked bradykinesia and rigidity in the neck and all extremities; postural tremor in both hands; facial, oromandibular, and truncal dystonia; and dystonic posturing of her hands, left worse than the right, with flexion of the metacarpophalangeal and distal interphalangeal joints. When she walks, her toes touch the ground before her heels due to dystonic muscle contractions. MRI of the brain shows hypointensity in the medial globus pallidus on T2-weighted images ("eye-of-the-tiger") and hyperintensity on T1 images consistent with pantothenate kinase-associated neurodegeneration (PKAN), formerly known as Hallervorden-Spatz syndrome. PKAN is caused by a mutation in the PANK2 gene (Schneider SA, Bhatia KP, Hardy J. Complicated recessive dystonia parkinsonism syndromes. Mov Disord 2009;24(4):490-9). This patient is a cousin of two other patients with PKAN, shown in other clips, the 32-year-old man with tongue protrusion and his teenage sister. (Contributed by Dr. Joseph Jankovic.)

Three members of a family with alcohol-responsive, myoclonus-dystonia (DYT11) with a mutation in the epsilon-sarcoglycan (SGCE) gene on chromosome 7q (Cazurro-Gutiérrez A, Marcé-Grau A, Correa-Vela M, et al. ε-Sarcoglycan: unraveling the myoclonus-dystonia gene. Mol Neurobiol 2021;58(8):3938-52). All 3 demonstrate the combination of myoclonus and dystonia, the latter chiefly manifested by dystonic writer’s cramp. (Contributed by Dr. Joseph Jankovic.)

A 24-year-old man shows cervical dystonia that improves with sensory tricks. (Contributed by Dr. Ravindra Kumar Garg.)

This man has a 15-year history of blepharospasm. Eyelid myectomies and lid lifts produced some benefit. Polaroid-tinted glasses that reduce brightness and glare are also beneficial. (Contributed by Dr. Joseph Jankovic.)

These are two members of a family with Friedreich ataxia and dystonia. Note the ataxia, marked scoliosis (much improved with Harrington rods), high-arched feet, and dystonic writer's cramp. (Contributed by Dr. Joseph Jankovic.)

Glutamatergic inputs from the cortex or thalamus synapse directly onto the head of the dendritic spine, dopaminergic projections from the SNr (VTA in ventral striatum) synapse at the neck of the spine, and cholinergic interneurons influence MSNs largely through “volume” transmission with a paucity of well-defined synapses. Ach – acetylcholine, DA – dopamine, Glu – glutamate. (Contributed by Dr. Roger Albin)

Midline sagittal MRI of a patient with 20-year history of episodic ataxia type 2 demonstrating atrophy of the superior cerebellar vermis. (Contributed by Dr. Peter Hedera.)