The prominent role of Charcot and the French neurological tradition in Latin America.

The establishment of neurology schools in Latin America during the late-nineteenth and early-twentieth centuries profoundly influenced the French neurology school. In the latter half of the nineteenth century, the neurology department at the Salpêtrière Hospital in Paris held a preeminent position as the global hub of neurology. Professor Jean-Martin Charcot, widely acclaimed as the father of modern neurology, was the most revered neurology professor of the nineteenth century.

Imbalance and gait impairment in Parkinson's disease: discussing postural instability and ataxia.

Gait and balance difficulties pose significant clinical challenges in Parkinson's disease (PD). The impairment of physiological mechanisms responsible for maintaining natural orthostatism plays a central role in the pathophysiology of postural instability observed in PD. In addition to the well-known rigidity and abnormalities in muscles and joints, various brain regions involved in the regulation of posture, balance, and gait, such as the basal ganglia, cerebellum, and brainstem regions like the pontine peduncle nucleus, are affected in individuals with PD.

Ramsay Hunt syndrome: New impressions in the era of molecular genetics.

More frequent use of next-generation sequencing led to a paradigm shift in assessing heredodegenerative diseases. This is particularly notable in progressive myoclonus epilepsy (PME) and progressive myoclonus ataxia (PMA) where a group of disorders linked to novel genetic mutations has now been added to these phenotypical realms. Despite the historical value of Ramsay Hunt's contribution defining the syndrome later known as PMA, recent genetic developments have made this eponym obsolete and a new definition and classification of PMA and PME seem necessary.

Inherited metabolic diseases mimicking hereditary spastic paraplegia (HSP): a chance for treatment.

The syndromic group of hereditary spastic paraplegias has a heterogeneous clinical profile and a broad differential diagnosis, including neurometabolic disorders that are potentially treatable. This group includes 5,10-methylenetetrahydrofolate reductase deficiency, cobalamin C deficiency disease, dopamine responsive dystonia, cerebrotendinous xanthomatosis, biotinidase deficiency, GLUT1 deficiency syndrome, delta-e-pyrroline-carboxylase-synthetase deficiency, hyperonithinemia-hyperammonemia-homocitrullinuria syndrome, arginase deficiency, multiple carboxylase deficiency, and X-linked adrenoleukodystrophy. This review describes these diseases in detail, highlighting the importance of early diagnosis and effective treatment aiming at preserving functionality and quality of life in these patients.

Comparing loss of balance and functional capacity among patients with SCA2, SCA3 and SCA10.

Background: Spinocerebellar ataxia (SCA) presents different rates of functional decline depending on the type of ataxia.

Objective: To compare the progression of disability, imbalance and severity of ataxia in patients with the three most common types of SCA in southern Brazil.

Methods: 126 patients (31-SCA2, 58-SCA3 and 37-SCA10) were stratified into four groups based on disease duration.

Would Francisco Soca have been the first to relate toe phenomenon as pyramidal disorders?

Introduction: The Uruguayan physician Francisco Soca, who specialized in neurology in Jean-Martin Charcot's clinic, defended a thesis at the Paris Faculty of Medicine in 1888 on Friedreich's ataxia in eleven patients. In this work he described the presence of toe phenomenon.

Objective: This historical note presents a toe sign described by the Soca eight years before Babiński's classic description.

Freezing of gait (FOG) in Parkinson's disease patients-the contribution of Garcin and Melaragno.

Raymond Garcin, professor of neurology in Paris, France, and his Brazilian assistant, Professor Roberto Melaragno described in 1948 the phenomenon defined as "bégaiement de la mise en route du mouvement" in patients with Parkinson's disease. This was one of the first descriptions of freezing of gait (FOG) in the world.

Charcot: Buddhist Leanings?

Jean-Martin Charcot, considered the father of modern neurology, had a complex personality featuring well-defined characteristics of introversion, competitiveness, irony, and skepticism. While biographers have described him as Republican, anticlerical, and agnostic, the literature also presents evidence that he came to admire Buddhism toward the end of his life; Charcot's involvement with numerous patients suffering from incurable and insidious neurological diseases may have contributed to this change in attitude.

The Art of Charcot: An Outstanding Caricaturist.

Jean-Martin Charcot is considered the father of modern neurology; alongside his work as a physician, professor, and researcher in this area, he was also artistically gifted with a taste for caricature. This historical note summarizes 8 caricatures by Charcot that exhibit a mixture of humor, satire, irony, and sarcasm.

Is Ataxia an Underestimated Symptom of Huntington's Disease?

Huntington's disease (HD) is a progressive disorder characterized by motor, cognitive and psychiatric features. Cerebellar ataxia is classically considered as uncommon in HD clinical spectrum. To determine the prevalence of cerebellar ataxia in patients with HD, both in the early and in the late stages of HD.

Balance and physical functioning in Spinocerebellar ataxias 3 and 10.

Objectives: Limitations of functional capacity and balance are common features of the natural history of spinocerebellar ataxias (SCA). However, their onset and progression patterns differ according to subtype. The aim of our study was to compare physical functionality and balance parameters in SCA10 and SCA3 patients, correlating with clinical variables.

Follow-Up of Advanced Parkinson's Disease Patients after Clinical or Surgical Emergencies: A Practical Approach.

Background: Patients with advanced stage Parkinson's disease (PD) typically present with a myriad of motor and nonmotor symptoms in addition to comorbidities and, as a consequence, polypharmacy.

Objective: To analyze a series of cases of advanced PD in which a clinical or surgical emergency played a trigger role in the irreversible progression of landmarks of the course of the disease.

Methods: Data were collected during a 13-month observational period of a cohort of 230 PD patients, in 751 medical appointments.

The Role of the Cerebellum in Huntington's Disease: a Systematic Review.

Huntington's disease (HD) is a rare neurological disorder characterized by progressive motor, cognitive, and psychiatric disturbances. Although striatum degeneration might justify most of the motor symptoms, there is an emerging evidence of involvement of extra-striatal structures, such as the cerebellum. To elucidate the cerebellar involvement and its afferences with motor, psychiatric, and cognitive symptoms in HD.

Analysis of diffusion tensor parameters in spinocerebellar ataxia type 3 and type 10 patients.

Introduction: There is a dearth of studies of spinocerebellar ataxias (SCAs) and diffusion tensor magnetic resonance imaging (DTI).

Objective: To analyze changes observed in DTI parameters and correlate these to clinical findings in SCA3 and SCA10 patients.

Methods: SCA3 (n = 19) and SCA10 (n = 18) patients were compared with a similar number of controls and assessed clinically and with the scale for the assessment and rating of ataxia (SARA) before undergoing the same MRI protocol.

Volumetric MRI Changes in Spinocerebellar Ataxia (SCA3 and SCA10) Patients.

Spinocerebellar ataxias type 3 (SCA3) and type 10 (SCA10) are the most prevalent in southern Brazil. To analyze the relationships between volumetric MRI changes and clinical and genetic findings in SCA3 and SCA10 patients. All patients in the study had a confirmed genetic diagnosis.

Overcoming bashfulness: how cocaine aided Freud to summon the courage to meet Charcot.

Jean-Martin Charcot, the most celebrated neurologist of the 19th century, had a profound influence on Sigmund Freud's career. Freud spent the winter of 1885-1886 working in Charcot's neurology department in Paris. During this period, he went to Charcot's house on several occasions to participate in the very famous Tuesday soirées under the guidance of Gilles de la Tourette.

Neuroradiological Findings in the Spinocerebellar Ataxias.

Background: The spinocerebellar ataxias (SCAs) are a group of autosomal dominant degenerative diseases characterized by cerebellar ataxia. Classified according to gene discovery, specific features of the SCAs - clinical, laboratorial, and neuroradiological (NR) - can facilitate establishing the diagnosis. The purpose of this study was to review the particular NR abnormalities in the main SCAs.