Funtabulously Frivolous Friday Five 334

Warts and molluscum contagiosum.

The picture is of a blister beetle. These insects secrete Cantharides from their exoskeleton when threatened resulting in burning and blistering of the skin.

Cantharidin is extremely hazardous and needs to be used carefully. While its blistering effects has a use, it is highly toxic with an LD50 of 0.5mg/kg causing GI and renal bleeding when ingested. Cantharidin is also aphrodisiac and can cause priapism as a side effect. According to Tacitus a roman historian, the empress to Augustus Caesar would use it to entice members of imperial families to commit sexual indiscretions that she could later bribe them with. The french surgeon Ambroise Paré described severe cases of men with “violent priapism” or satyriasis (nymphomania) and even a priest who swallowed a dose who later died from haematuria.

References

• Augustin Cabanés. Remèdes d’autrefois. Paris: A. Maloine, 1910: 498
• James P. Ancient Inventions. Ballantine Books. 1994: 177

Grisel Syndrome or altlantoaxial rotation

Spontaneous, nontraumatic rotatory subluxation of the atlantoaxial joint following peripharyngeal inflammation or otorhino-laryngologic surgical procedures (e.g. tonsillectomy, adenoidectomy). Occurs most commonly in the pediatric population.

Pathogenesis of Grisel syndrome is still not quite clear, but it is probably a ‘double hit’ phenomenon. Firstly those with a susceptibility for having a lax C1/C2 joint i.e. children or those with ligament disorder or Down syndrome. These people then get a ‘second hit’ from spasms caused by inflammatory mediators carried to cervical muscles by the pharyngeovertebral venous plexus.

In 1830, Sir Charles Bell reported a case of death from atlanto-axial subluxation which resulted in spinal cord compression in a patient with probable syphilitic ulceration of the pharynx.

A patient, who had a deep ulcer in the back part of the throat, was seized with symptoms like those of apoplexy. These symptoms continued for two hours. At this time the patient’s head fell suddenly forward, and he instantly expired. On dissection, it was found that the ulcer had destroyed the transverse ligament, which holds the process of the dentata in its place. In consequence of the failure of this support, the process was thrown back, so as to compress the spinal marrow. The parts are preserved in my collection.

Bell, Case LXIV 1830

Grisel syndrome (La maladie de Grisel) was named after Pierre Grisel (1874–1959), a French surgeon who described the syndrome characterized by dislocation of the C1–C2 joint in patients with pharyngitis and torticollis in 1930

Other differentials for torticollis to keep you awake at night include: spondylodiscitis, osteomyelitis, juvenile idiopathic arthritis, bone tumours, posterior fossa tumours, cervical cord tumours, dystonia, pseudo-tumour cerebri, trochlear nerve palsy, nystagmus, retropharyngeal abscess, Sandifer syndrome, Lemierre syndrome and even upper lobe pneumonia.

References

• Bell C. Case LXIV. In: The nervous system of the human body, embracing the papers delivered to the Royal Society on the subject of the nerves. 1830: 391
• Grisel P. Enucléation de l’atlas et torticolis naso-pharyngien. La Presse médicale, 1930; 38: 50-53
• Osiro S, Tiwari KJ, Matusz P, Gielecki J, Tubbs RS, Loukas M. Grisel’s syndrome: a comprehensive review with focus on pathogenesis, natural history, and current treatment options. Childs Nerv Syst. 2012 Jun;28(6):821-5
• Golzari SE, Ghabili K, Sajadi MM, Aslanabadi S. Early descriptions of Grisel’s syndrome. Childs Nerv Syst. 2013 Mar;29(3):359-60.
• Ciftdemir NA et al. A Rare Case of Torticollis: Grisel Syndrome. Journal of Tropical Pediatrics. 2018;64:245-248
• Anania P et al. Grisel Syndrome in Pediatric Age: A Single-Center Italian Experience and Review of the Literature. World Neurosurg. 2019 May;125:374-382.
• Pini N, Ceccoli M, Bergonzini P, Iughetti L. Grisel’s Syndrome in Children: Two Case Reports and Systematic Review of the Literature. Case Rep Pediatr. 2020 Nov 12;2020:8819758
• Fox SM. Nontraumatic AtlantoAxial Subluxation in Children. PedEMmorsels

Samuel Shem from the book ‘House of God‘

Laws of the House of God:

1. GOMERS don’t die.
2. GOMERS go to ground.
3. At a cardiac arrest, the first procedure is to take your own pulse.
4. The patient is the one with the disease.
5. Placement comes first.
6. There is no body cavity that cannot be reached with a #14G needle and a good strong arm.
7. Age + BUN = Lasix dose.
8. They can always hurt you more.
9. The only good admission is a dead admission.
10. If you don’t take a temperature, you can’t find a fever.
11. Show me a BMS (Best Medical Student, a student at The Best Medical School) who only triples my work and I will kiss his feet.
12. If the radiology resident and the medical student both see a lesion on the chest x-ray, there can be no lesion there.
13. The delivery of good medical care is to do as much nothing as possible.

Shem added four more “Laws” in his reflection of The House of God 34 years later.

14. Connection comes first.
15. Learn empathy.
16. Speak up.
17. Learn your trade, in the world.

His adrenals

JFK had Addison’s, although he denied having a chronic disease through his presidential campaign and beyond. The official autopsy report made no mention of his adrenal glands but later one of the residents made comment that there was barely any adrenal tissue left and certainly no signs of tuberculosis, confirming he had idiopathic Addison’s disease.

Reference:

• Mandel LR. Endocrine and autoimmune aspects of the health history of John F. Kennedy. Ann Intern Med. 2009 Sep 1;151(5):350-4.

The droids are K-2SO, BB8, R2D2 and C3PO (left to right)

The disease is RS3PE

Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE), first described in 1985 by McCarty et al, is a rare syndrome that is a subset of the seronegative symmetrical polyarthritis of older people.

The main characteristics of RS3PE syndrome are:

• Acute onset 
• Raised acute phase reactants
• Bilateral diffuse, symmetrical swelling 
• Rheumatoid factor negative
• Hand and wrist involvement  
• Human Leukocyte Antigen (HLA) B7 Positive
• Dorsal pitting oedema of hands             
• Positive response to low dose corticosteroids
• Symmetrical synovitis               
• X-rays show soft tissue swelling in the absence of erosions.

Olive et al also proposed the following diagnostic criteria for this syndrome:

• Bilateral pitting oedema of both hands
• Sudden onset of polyarthritis
• Age more than 50 years
• Seronegative for rheumatoid factor.

Reference:

• McCarty DJ, O’Duffy JD, Pearson L, Hunter JB. Remitting seronegative symmetrical synovitis with pitting edema. RS3PE syndrome. JAMA. 1985 Nov 15;254(19):2763-7.
• Olivé A, del Blanco J, Pons M, Vaquero M, Tena X. The clinical spectrum of remitting seronegative symmetrical synovitis with pitting edema. The Catalán Group for the Study of RS3PE. J Rheumatol. 1997 Feb;24(2):333-6.