Charcot and vascular Parkinsonism

O parkinsonismo vascular de Charcot

ABSTRACT

Jean-Martin Charcot (1825-1893), recognized as the founder of Neurology and the first formal teacher of nervous system diseases, died on August 16, 1893, from acute pulmonary edema secondary to myocardial infarction. In his last years, there were several descriptions of his gait and posture disorders, suggesting the diagnosis of “lower-half parkinsonism” due to cerebrovascular disease.

Parkinson’s disease; brain diseases

RESUMO

Jean-Martin Charcot (1825-1893) pode ser reconhecido como o fundador da Neurologia, bem como o primeiro professor de doenças do sistema nervoso. Ele morreu no dia 16 de Agosto de 1893, em decorrência de edema agudo de pulmão, secundário a infarto agudo do miocárdio. Nos últimos anos da vida de Charcot, ocorreram várias descrições de anormalidades da sua marcha e da sua postura, sugerindo o diagnóstico de parkinsonismo vascular.

doença de Parkinson; encefalopatias

Jean-Martin Charcot (1825-1893) is widely recognized as the founder of Neurology as well as the first formal teacher of nervous system diseases¹,². His contribution to neurology is outstanding, describing several diseases, including Charcot-Marie-Tooth disease, amyotrophic lateral sclerosis, and multiple sclerosis, among others¹,². During his tenure at the Paris Medical School as professor of diseases of the nervous system (from 1882 to 1893), Charcot suffered from chronic low back pain and angina pectoris attacks¹,²,³,⁴,⁵. He died on August 16, 1893, from acute pulmonary edema secondary to myocardial infarction¹,²,³,⁴,⁵. The aim of this historical review is to analyze clues that may indicate the diagnosis of vascular parkinsonism over the course of Charcot’s later life.

CHARCOT’S HEALTH PROFILE

Biographical data about Jean-Martin Charcot (Figure) demonstrates that, overall, he led an unhealthy life. He had a short stature, short neck, was obese (predominantly in the abdominal area), had poor physical hygiene, was sedentary with a voracious appetite, in addition to having been a chronic heavy smoker (especially cigars), combined with a disturbed sleep cycle due to intense intellectual activity stretching late into the night¹,²,³,⁴,⁵. Charcot’s personality was defined as authoritarian, shy, taciturn, skeptical, brusque, gloomy, and competitive¹,²,³,⁴,⁵. Bounduelle considered that Charcot’s silence hid “a prelude to an explosion of rage”³. He suffered from chronic low back pain and intermittent episodes of precordial discomfort, suggestive of coronary insufficiency, associated with anxiety³,⁴,⁵. Modern literature confirms the relationship between anxiety, anger and mortality risk among patients with myocardial infarction.

Figure
Jean-Martin Charcot (1825-1893).

CHARCOT’S CORONARY ATHEROSCLEROTIC DISEASE

During the new year’s eve of 1890, at the age of 65, Charcot had an acute episode of precordial pain, and Professor Potain, a famous Parisian clinician, confirmed the diagnosis of angina pectoris, suggesting a potentially fatal outcome within two years¹,². Guillain, in his famous biography on Charcot (J-M Charcot. His Life – His Work, edited by Bailey in the USA), wrote about this cardiovascular episode, citing Léon Daudet’s memoirs “Charcot was dining in his house, with several friends, including Louis Pasteur, and suddenly felt a violent constriction around the heart. He left the table, became terribly pale, and his handsome features revealed intense pain”¹. Professor Potain, a famous clinician, who lived across the street from Charcot’s house on Boulevard Saint Germain, examined him extensively and prescribed medication¹. After that, Professor Potain shared with Daudet a fateful prognosis “Charcot knows what ails him, but I succeeded in reassuring him a little – two years and a half, my poor friend”¹. According to other sources published by Guinon and Souques in 1925⁴,⁵, both entitled Charcot Intime, there was no mention of other vascular arterial diseases suggestive of arterial insufficiency episodes, such as intermittent claudication, a condition previously described by Charcot himself⁶,⁷, or transient ischemic attack (cerebrovascular disease).

CHARCOT’S VASCULAR PARKINSONISM

In the last years of his life there were several descriptions, one of them from his assistant Souques, of Charcot’s gait and posture disorders (shuffling, apraxic gait with “petit pas”, and flexed posture of the trunk), mimicking Parkinson’s disease¹,²,³,⁴,⁵. Interestingly enough, there was no reference to the presence of upper limb symptoms, or tremor (typical pill-rolling rest tremor), suggesting the diagnosis of “lower-half or lower-body parkinsonism” indicative of vascular parkinsonism¹,²,³,⁴,⁵. In 1925, Garrison described Charcot as “so sedentary that his very gait was regarded as that of a man who could not walk properly because he had forgotten how. With bent back and head thrust forward, he seemed to propel himself by short, quick, shuffling steps. Toward the last, when he mimicked the propulsion in paralysis agitans, he seemed to be a patient.⁸

Critchley was a pioneer in this topic, describing, for the first time, “arteriosclerotic parkinsonism” in 1929⁹. Nowadays, vascular parkinsonism represents the second most common cause of secondary parkinsonism, resulting from cerebrovascular disease¹⁰. Normal pressure hydrocephalus represents another important cause of secondary parkinsonism¹⁰. Vascular parkinsonism is characterized by the presence of bradykinesia and rigidity, predominantly in the lower limbs, associated with a peculiar pattern of gait and postural disorder, frequent falls, cognitive decline, pyramidal signs, and other focal deficits¹⁰. Vascular parkinsonism has remained a controversial clinical entity and brain MRI is the most important paraclinical test for the diagnosis, demonstrating microvascular brain disease, with subcortical white matter lesions (leukoaraiosis), and lacunar infarcts¹⁰.

In conclusion, we speculate that Professor Charcot may have presented with features indicative of vascular parkinsonism in his last years of life, associated with other vascular disorders and risk factors (angina pectoris, probable cerebrovascular disease and, eventually, myocardial infarction).

References

• ¹
  Guillain G. J-M. Charcot. 1825-1893: his life- his work. New York: Paul B. Hoeber; 1959.
• ²
  Goetz CG, Bonduelle M, Gelfand T. Charcot: constructing neurology. New York: Oxford University Press; 1995.
• ³
  Bonduelle M. The intimate Charcot. Rev Neurol (Paris). 1994;150:524-8.
• ⁴
  Guinon G. Charcot intime. Paris Medical. 1925:511-6.
• ⁵
  Souques A. Charcot intime. Presse Medicale. 1925:693-8.
• ⁶
  Lacombe M. [The history of intermittent claudication due to arterial disease]. Ann Chir. 2005;130:440-4. French. https://doi.org/10.1016/j.anchir.2005.03.008
  » https://doi.org/10.1016/j.anchir.2005.03.008
• ⁷
  Teive HAG, Munhoz RP, Barbosa ER. Little-known scientific contributions of J-M Charcot. Clinics. 2007;62:211-4. https://doi.org/10.1590/S1807-59322007000300003
  » https://doi.org/10.1590/S1807-59322007000300003
• ⁸
  Garrison FH. Charcot. Int Clin.1925;35:244-72.
• ⁹
  Critchley M. Arteriosclerotic parkinsonism. Brain. 1929;52:237-40.
• ¹⁰
  Munhoz RP, Werneck LC, Teive HA. The differential diagnoses of parkinsonism: findings from a cohort of 1528 patients and a 10 years comparison in tertiary movement disorders clinics. Clin Neurol Neurosurg. 2010;112(5):431-5. https://doi.org/10.1016/j.clineuro.2010.03.003
  » https://doi.org/10.1016/j.clineuro.2010.03.003

Publication Dates

• Publication in this collection
  Mar 2017

History

• Received
  13 June 2016
• Reviewed
  08 Nov 2016
• Accepted
  07 Dec 2016