Margot Noack

Biography

• Born 1909 in Berlin
• 1939 – MD, University of Berlin
• Trained in paediatrics and after holding hospital appointments in Stralsund and Rostock
• 1957 – Moved to Saarbrucken, West Germany
• 1959 – Married and continued to practice as Dr Margot Oegg
• 1978 – Retired from medical practice but continued to serve on paediatric advisory boards.

Medical Eponyms

Noack syndrome (1959)

Autosomal dominant craniosynostosis syndrome with characteristic anomalies of the hands and feet. [aka Pfeiffer syndrome, acrocephalosyndactyly V, ACS5, OMIM 101600]. Three clinical subtypes, which have important diagnostic and prognostic implications, have been identified.

• Type 1, the classic syndrome, is compatible with life and consists of craniosynostosis, midface deficiency, broad thumbs, broad great toes, brachydactyly, and variable syndactyly.
• Type 2 consists of cloverleaf skull with Pfeiffer hands and feet, together with ankylosis of the elbows.
• Type 3 is similar to type 2 but without cloverleaf skull.

In 1959, Noack reported on a 43-year-old man and his 11-month-old daughter. They both exhibited acrocephaly and polysyndactyly with enlarged thumbs and enlarged duplicated great toes. Intelligence was normal.

Pfeiffer followed-up Noack’s kindred in 1964 and 1969 and found the disorder to be acrocephalosyndactyly type V rather than the earlier designation by Noack and MIM as Apert syndrome or acrocephalopolysyndactyly type I.

In 1975 Robinow and Sorauf described an extensively affected kindred with what they termed ‘Noack syndrome’, now referred to as Robinow-Sorauf syndrome. OMIM #180750

Major Publications

• Noack M. Ein Beitrag zum Krankheitsbild der Akrozephalosyndaktylie (Apert) [A contribution to the clinical picture of acrocephalosyndactylia (Apert’s syndrome)]. Archiv für Kinderheilkunde, 1959; 160: 168-171

References

Biography

• Beighton P, Beighton G. Noack, Margot. In: The Man Behind the Syndrome. 1991: 220
• Bibliography. Noack, Margot. WorldCat Identities

Eponymous Term

• Pfeiffer syndrome. OMIM #101600
• Robinow-Sorauf syndrome. OMIM #180750
• Robinow M, Sorauf TJ. Acrocephalopolysyndactyly, type Noack, in a large kindred. Birth Defects Orig. Art. Ser. 1975; XI(5): 99-106.