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Stengel's syndrome

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A juvenile form of familial amaurotic idiocy characterized by rapid deterioration of vision and intelligence, with mental disturbances, convulsions, and other neurologic symptoms. Eventually the child becomes blind, bedridden and demented. Both sexes equally affected. Onset at age 5-8 years; death within 10 to 15 years after onset. It is most often seen in Jewish families but may occur in any ethnic group. Transmitted as an autosomal recessive trait. It is the most common form of a group of disorders called neuronal ceroid lipofuscinoses.

The first probable instances of this condition were reported in 1826 by Dr. Christian Stengel in a Norwegian medical journal, who described 4 affected siblings in the small mining community Røros in Norway. Although no pathological studies were performed on these children the clinical descriptions are so succinct that the diagnosis of the Spielmeyer-Vogt syndrome type is fully justified.

At the beginning of the 20th century the Batten brothers, Rayner and Frederick, individually documented separate sets of affected siblings in England. Frederick Eustace Batten published his report in 1903 and in England and in the British literature the name Batten was used for this syndrome. In 1904 Marmaduke Stephen Mayou published an article concerning familial cerebral degeneration and retinal changes. While employed at the Langenhagen Institution, Vogt investigated a number of children with idiocy and amaurosis (i.e. visual problems) and he recognised a specific condition which was familial, with onset in the middle years of the first decade. He compared the natural history of this disorder with the familial amaurotic idiocy previously delineated by Waren Tay and Bernard Sachs, and concluded that these conditions were separate entities and proposed subdivision into infantile and juvenile forms. Following clinical delineation, Vogt undertook histological investigations in his patients and demonstrated the presence of inclusion bodies in neuronal tissue. He submitted an account of his findings for publication, but in November 1905, prior to the appearance of Vogt's account of extensive clinicopathological studies on several families, Walther Spielmeyer reported detailed studies on three siblings at a medical congress in Karlsruhe. In 1908 Wolfgang Stock published an account of the same disorder and the double or triple eponym came into use.

In 1931, the Swedish psychiatrist and geneticist, Torsten Sjögren, presented 115 cases with extensive clinical and genetic documentation and concluded that the disease is genetically separate from Tay-Sachs.

See also:
Bielschowsky's amaurotic idiocy, or late infantile amaurotic idiocy syndrome, under Max Bielschowsky, German neuropathologist, 1869-1940.

Tay-Sachs disease, or amaurotic familial infantile idiocy, under Waren Tay, English ophthalmologist and surgeon, 1843-1927.

Kufs' disease, an adult form of amaurotic familial idiocy, under Hugo Kufs, German neuropathologist, 1871-1955.


  • C. Stengel:
    Beretning om et mærkeligt Sygdomstilfelde hos fire Sødskende. Eyr, 1826.
  • F. E. Batten:
    Cerebral degeneration with symmetrical changes in the maculae in two members of a family.
    Transactions of the Ophthalmological Societies of the United Kingdom, 1902, 23: 386-390.
  • M. S. Mayou:
    Cerebral degeneration with symmetrical changes in the maculae, in three members of a family.
    Transactions of the Ophthalmological Societies of the United Kingdom, 1904, 24: 142-145.
  • H. Vogt:
    Über familiäre amaurotische Idiotie und verwandte Krankheitsbilder.
    Monatsschrift für Psychiatrie und Neurologie, Basel, 1905, 18: 161-171, 310-357.
  • W. Spielmeyer:
    Klinische und anatomische Untersuchungen über eine besondere Form von familiärer amaurotische Idiotie.
    Freiburg im Breisgau, Gotha, 1907. Reprinted in Nissl: Histologische und histopathologische Arbeiten über die Grosshirnrinde 1908, 2: 193-213.
  • W. Stock:
    Über eine bis jetzt noch nicht beschriebene Form der familiär auftretenden Netzhautdegeneration bei gleichzeitiger Verblödung und über Pigmentdegeneration der Netzhaut.
    Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1908, 5: 225-244.
  • K. G. T. Sjögren:
    Die juvenile amaurotische Idiotie. 1931.
  • D. G. Cogan:
    Amaurotic familial idiocy. A case for eponyms.
    New England Journal of Medicine, Boston, 1958, 258: 1212-1213.
  • John Midtdal:
    Stengels syndrom.
    Fjell-folk : årbok for Rørostraktene/Rørosmuseet. Røros, 1995: 20: 51-55.

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