Movement Disorders

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. (Source: [Alm 2020]. Data for Denmark were from [Steer 2015], and data for the United States are from [Massell et al 1988]. 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.)

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).

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 2 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.)