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Adrenoleukodystrophy
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
At the other end of the spectrum, three infants have been reported [39], whose phenotype was that of a peroxisomal disorder, such as neonatal ALD (Chapter 63). They had profound hypotonia, failure to thrive, and cholestatic hepatic disease. Two had seizures and the third episodic opisthotonus. In a single autopsy, the adrenals were small and fibrotic.
Rabies and other lyssaviruses
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
Thiravat Hemachudha, Jiraporn Laothamatas, Henry Wilde
Tetanus resembles rabies only in the form of reflex spasms [1]. All tetanus patients have a clear sensorium. Rabies patients do not have persistent rigidity or sustained contraction of axial musculature such as the jaw, neck, back, and abdomen as in tetanus. Spasms in rabies predominantly involve accessory respiratory muscles and the diaphragm, whereas in tetanus, spasms occur in axial muscles. Opisthotonos is present extremely rarely, if ever, in rabies [28].
Diseases of the Nervous System
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
This condition is a rare degenerative disorder of the nervous system transmitted as an autosomal recessive trait. It is also called maple syrup disease, due to the distinctive peculiar odor of the urine and perspiration, reminiscent of the natural maple syrup. Branched chain ketoaciduria manifests during the first week of life with varying severity and causes early death. The symptoms show progressive involvement of the nervous system and prominent features include seizures, irregular respiration, and muscular rigidity alternating with opisthotonus and flaccidity. Some untreated children develop spasticity and survive for several years with severe mental retardation.
Fatal serotonin syndrome: a systematic review of 56 cases in the literature
Published in Clinical Toxicology, 2021
Sanjay Prakash, Chaturbhuj Rathore, Kaushik Rana, Anurag Prakash
Seizure is not reported as a common manifestation in most of the reviews on SS. The prevalence of seizures varies from 2 to 5% in different reviews [53, 61]. In this review, convulsions were observed in 20 patients (36%), and it was the second most common symptom, again indicating that it may be a poor prognostic factor. The muscle rigidity is considered less common and less severe with SS compared to NMS [56]. Sternbach’s criteria did not include rigidity as a clinical feature. Although rigidity/hypertonicity is a part of the Hunter criteria, it was not a very common and prominent symptom in their database. They considered the presence of rigidity in severe and life-threatening SS [5]. Rigidity was noted in about 27% of our cases, and it was severe in many cases, leading to trismus, hyperextended spine and legs, opisthotonus, and inelicitable deep tendon reflex/clonus. Identification of trismus is very important as these patients may need an urgent tracheostomy. Our observation indicates that severe rigidity can occur in patients with severe SS and is again a poor prognostic factor. This can also cause diagnostic confusion with NMS.
Jean-Martin Charcot´s medical instruments: Electrotherapeutic devices in La Leçon Clinique à la Salpêtrière
Published in Journal of the History of the Neurosciences, 2021
Francesco Brigo, Albert Balasse, Raffaele Nardone, Olivier Walusinski
La Leçon Clinique à la Salpêtrière (A Clinical Lesson at the Salpêtrière) is a famous oil painting made in 1887 by the French painter André Brouillet (1857–1914), held at the Hôpital de la Salpêtrière in Paris. It represents Charcot delivering a clinical lecture and a demonstration of hypnosis in front of a large audience. Charcot’s pupil Joseph Babinski (1857–1932) is holding a patient up. She is Marie Wittman, a famous hysterical patient known as “Blanche” (1859–1912). Contrary to what is sometimes claimed (Geisler 2011; Harris 2005; Justice-Malloy 1995; Morlock 2007), in this painting the woman is not having a hysterical attack. Her posture resembles that of opisthotonus (or arc de cercle), which defined the second phase (période des grands mouvements) of grande hystérie, the prototypical form of hysteria (Richer 1881). However, Blanche’s left arm and hand are held in a contorted posture, which indicates a hypnotically induced lethargy. The first phase of the hypnotical state was catalepsy: The patient’s eyes remained open but unblinking, the subject was endowed with waxy flexibility, and the body and limbs could maintain any position into which they were molded (Richer and Gilles de la Tourette 1889). The following stage was lethargy, characterized by eye closure, muscle flaccidity resembling that of a corpse before the onset of rigor mortis, complete unconsciousness, and total anesthesia (Richer and Gilles de la Tourette 1889).
Current challenges in the pathophysiology, diagnosis, and treatment of paroxysmal movement disorders
Published in Expert Review of Neurotherapeutics, 2021
Cécile Delorme, Camille Giron, David Bendetowicz, Aurélie Méneret, Louise-Laure Mariani, Emmanuel Roze
Opisthotonos posturing, such as seen in Psychogenic non-epileptic seizures (PNES), can also be observed in FPMD [188]. FPMD and PNES are usually reported and studied separately in the literature. We think this distinction is quite artificial as PNES can manifest without conscience alteration and FPMD can present with altered responsiveness. This distinction is biased since FPMD are usually seen by movement disorders specialists, whereas PNES are often seen by epileptologists [189]. Several studies have highlighted the clinical similarities between FPMD and PNES [190,191]. Clinical clues that have been identified for PNES, such as the frequency of vocalizations, opisthotonos, undulating motor activity, rhythmical pelvic movements, side-to-side head shaking, ictal crying, huffing, or puffing, and a waxing and waning pattern during the paroxysmal event can therefore be also useful additional clues for FPMD[192–194]. Likewise, induction by a placebo procedure may be a clue for a functional mechanism, as studied in PNES [195].