Funtabulously Frivolous Friday Five 318

Dix-Hallpike manoeuvre

Margaret Ruth Dix (1902 – 1991) was an English neuro-otologist. Dix, in 1952, with Charles Skinner Hallpike described the Dix-Hallpike manoeuvre, used to diagnose benign paroxysmal positional vertigo.

• Dix MR, Hallpike CS. The pathology symptomatology and diagnosis of certain common disorders of the vestibular system. Proc R Soc Med. 1952; 45(6): 341-54

When asked why she chose a medical career, Margaret would reply: “Because a woman cannot be a clergyman!”

• 1937 she completed her MBBS and began training as a surgeon. Margaret’s supervisors recognised her competence and allowed her to perform tonsillectomy procedures. In 1940 Margaret’s surgical career was cut short when she sustained injuries to the face in an air-raid during the Blitz – Margaret suffered facial disfigurement, a torn eardrum and fragments of glass lodged in her eyes which prevented her from continuing as a surgeon.
• At the end of the war in 1945 Margaret joined the Otological Research Unit at the National Hospital, Queen Square London. Alongside otologist Charles Skinner Hallpike, she studied and practiced neuro-otology – a new branch of medicine at the time – and carried out research on deafness in ex-servicemen.
• She published over 100 articles and received several prizes for her work.

Despite their important contributions, it has been suggested that Dix and Hallpike did not fully understand the physiology of BPPV, as they did not appreciate that the manoeuvres, they used demonstrated pathology in the semicircular canals rather than the utricle as they believed

Churg-Strauss syndrome [Now termed eosinophilic granulomatosis with polyangitis (EGPA)]

Lotte Strauss (1913 – 1985) was a German-America pathologist. Strauss fled Nazi Germany and became the first paediatric pathologist at Mt Sinai Hospital in Manhattan.

• Churg J, Strauss L. Allergic Granulomatosis, Allergic Angiitis, and Periarteritis Nodosa. Am J Pathol. 1951 Apr; 27(2):277–301

EGPA is characterized by extravascular necrotizing granulomas, (usually rich in eosinophils). eosinophilia, and tissue infiltration by eosinophils. However, these abnormalities do not always coexist. The vasculitis typically affects small- and medium-sized arteries. Any organ can be affected, but the lungs, skin, sinuses, cardiovascular system, kidneys, peripheral nervous system, CNS, joints, and gastrointestinal (GI) tract are most commonly affected. Occasionally, pulmonary capillaritis may cause alveolar hemorrhage.

The cause of eosinophilic granulomatosis with polyangiitis is unknown. However, an allergic mechanism, with tissue directly injured by eosinophils and neutrophil degranulation products, may be involved. The syndrome occurs in patients who have adult-onset asthma, allergic rhinitis, nasal polyposis, or a combination. Antineutrophil cytoplasmic autoantibodies (ANCA) are present in about 40% of cases.

For those with an interest in Paediatric pathology who are under the age of 40 can be in the running for the annual Lotte Strauss Prize for work pertaining to pediatric pathology.

Gaskin (all fours) manoeuvre

Ina May Gaskin (1940 – ) first described the manoeuvre in 1977

Gaskin introduced this manoeuvre to the US in 1976 after learning it from a Belizean woman who had in turn learnt it in Guatemala where it is believed to have originated. Gaskin first documented the manoeuvre in the second edition of Spiritual Midwifery (1977), and expanded in an informal article on a series of shoulder dystocias in which the maneuver had proven to be extremely effective (1988). In 1991 Gaskin collaborated with family physicians to publish a formal review 32 cases of shoulder dystocia, all of which had been resolved with the all-fours position.

This position works very well to widen the pelvis in just the way that is required when the shoulders are stuck: instead of the mother’s coccyx being pushed towards the symphysis pubis in the way it is in the seated position, there is no pressure on the coccyx and the baby’s weight is pushing on the symphysis pubis, thereby widening the anterior to posterior diameter a little.

Gaskin 2002: 354

Reference:

• Gaskin MA. All-Fours Maneuver for Reducing Shoulder Dystocia During Labour. J Reproducers Med 1998;43:439-443)

• Georgina Ruth HOGG (1916 – 2002) of Birt-Hogg-Dubé syndrome (1977)
• Monika KNICKENBERG of Hornstein-Knickenberg syndrome (1975)

The autosomal dominant inherited syndromes of Hornstein-Knickenberg (HKS) and Birt-Hogg-Dubé (BHDS) are both characterized clinically by the presence of multiple flesh coloured papules of the skin. Hornstein and Knickenberg termed the peripheral lesions of HKS ‘perifollicular fibromas’ whilst Birt, Hogg and Dubé proposed the name ‘fibrofolliculoma’.

The nosological dilemma is twofold:

• Many authors believe that perifollicular fibromas and fibrofolliculomas are one and the same, and not a reason to suppose that the condition described by Birt, Hogg and Dubé (1977) was a new disorder from that originally described by Hornstein and Knickenberg (1975)
• The importance of ‘extracutaneous cancer proneness’, was postulated in all three papers by Hornstein, but not in the 1977 of Birt, Hogg and Dubé. Numerous authors have corroborated the association between multiple perifollicular fibromas and colonic polyps/cancer as Hornstein and Knickenberg suggested.

Prof Dr Rudolf Happle of the Department of Dermatology, at the University of Freiburg staunchly moved for a name change in 2012, and again in 2020

Birt et al. did neither discover nor describe any new cutaneous disorder heralding proclivity to extracutaneous cancer. Hence, the term ‘Birt-Hogg-Dubé syndrome’ represents a bold distortion of history and should no longer be used to describe the disorder specified by Hornstein and Knickenberg…it is not justified to combine the names of the discoverers of a new genodermatosis heralding extracutaneous cancer proneness with those claiming that they had detected a quite different hereditary skin disorder characterized by ‘fibrofolliculomas’, without any proclivity to extracutaneous cancer…Today, time has come to put this right. The syndrome should be named after the original authors, Hornstein and Knickenberg

Happle 2020

• Schulz T, Hartschuh W. Birt-Hogg-Dubé syndrome and Hornstein-Knickenberg syndrome are the same. Different sectioning technique as the cause of different histology. J Cutan Pathol. 1999; 26(1): 55-61.
• Happle R. Hornstein-Birt-Hogg-Dubé syndrome: a renaming and reconsideration. Am J Med Genet A. 2012;158A(6):1247-1251.
• Happle R. Hornstein–Knickenberg syndrome vs. Birt–Hogg–Dubé syndrome: a critical review of an unjustified designation. Journal of the European Academy of Dermatology and Venereology 2020; 34(4): 885-887

Frey’s syndrome after Łucja Frey-Gottesman (1889 – 1942?) a Polish physician and neurologist. The disorder was first reported by Baillarger in 1853, a neurologist from Poland. Dr Frey provided a more detailed account calling it the ‘auriculotemporal syndrome’ in 1923.

Frey’s syndrome is a result of damage to the auriculotemporal nerve usually secondary to parotid surgery. The auriculotemporal branch of the mandibular (V3) branch of the trigeminal nerve carries parasympathetic fibres to the parotid gland and sympathetic fibres to the sweat glands of the scalp. This results in redness and sweating just in front of the ear with a gustatory stimuli “gustatory sweating”.

Reference:

• Frey L. Le syndrome du nerf auriculo-temporal. Revue Neurologique 1923; II(2): 97-104.