Diseases of the Nervous System
George Feuer, Felix A. de la Iglesia in Molecular Biochemistry of Human Disease, 2020
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.
Infectious Diseases
Stephan Strobel, Lewis Spitz, Stephen D. Marks in Great Ormond Street Handbook of Paediatrics, 2019
Tetanus is seen in two forms, generalised or local. The most common presentation is the generalised form, which manifests as trismus (tonic spasms of the masseter muscles/risus sardonicus) (Fig. 3.3) in over 50% of the cases. Other manifestations may include irritability, difficulty in swallowing and rigidity of abdominal muscles. Slight external stimuli may precipitate a sudden burst of painful tonic contractions of all groups of muscles leading to the characteristic opisthotonus posture.
Disaster surgery
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie in Bailey & Love's Short Practice of Surgery, 2018
Early symptoms are painful spasms of the facial muscles resulting in risus sardonicus (Figure29.15). The spasms spread to involve the respiratory and laryngeal musculature. Spasms of the paravertebral and extensor limb musculature produce opisthotonus, an arching of the whole body. Laryngeal muscle spasm leads to apnoea and, if prolonged, to asphyxia and respiratory arrest. The spasms can be brought on by the slightest of sensory stimulus.
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).
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.
Strychnine, old still actual poison: description of poisoning cases reported to French Poison Control Centers over the past thirteen years
Published in Toxin Reviews, 2022
Camille Paradis, Denis Dondia, Audrey Nardon, Ingrid Blanc-Brisset, Arnaud Courtois, Jules-Antoine Vaucel, Magali Labadie
In the gray matter of the spinal cord, Renshaw cells function as inhibitory interneurons associated with an alpha motor neuron. Renshaw cells receive excitatory collaterals from the alpha motor neurone and thus, upon excitation, could release glycine to the alpha motor neurone in order to exert an inhibitory feedback and prevent alpha motor neurone firing. Strychnine is a potent antagonist of glycine receptors thus acting as an inhibitor of an inhibitory signal. This results in a motor disturbance characterized by an increase in muscle tone, associated with muscular hyperactivity. Clinical symptoms of poisoning appear suddenly and rapidly after ingestion, and begin with painful muscle contractures spontaneous or triggered by even the slightest stimulus, trismus, an opisthotonus (a position which the whole body is arched backward in hyperextension) and generalized convulsions for the severe forms. Importantly, during the course of poisoning, consciousness is retained. The clinical picture is complicated by major acidosis and rhabdomyolysis. Death occurs due to the paralysis of respiratory muscles or a cardiac arrest (Baud and Garnier 2017).