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Chemical and Pathological Findings in a Case of Late Infantile Amaurotic Family Idiocy of the Batten Type

Published online by Cambridge University Press:  08 February 2018

A. H. Tingey ,
R. M. Norman ,
H. Urich  and
W. H. Beasley
A. H. Tingey
Affiliation:
From The Burden Neuropathological Laboratory, Frenchay Hospital, Bristol and The Department of Pathology, St. Woolos Hospital, Newport, Mon
R. M. Norman
Affiliation:
From The Burden Neuropathological Laboratory, Frenchay Hospital, Bristol and The Department of Pathology, St. Woolos Hospital, Newport, Mon
H. Urich
Affiliation:
From The Burden Neuropathological Laboratory, Frenchay Hospital, Bristol and The Department of Pathology, St. Woolos Hospital, Newport, Mon
W. H. Beasley
Affiliation:
From The Burden Neuropathological Laboratory, Frenchay Hospital, Bristol and The Department of Pathology, St. Woolos Hospital, Newport, Mon
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Extract

The customary classification of the amaurotic family idiocies according to the time of life at which neurological signs first appear has obvious limitations when consideration is given to cases occupying an intermediate position between the well-established infantile form of Tay-Sachs' and the juvenile form first described by Batten (1903). It was to this group of atypical cases that Bielschowsky (1913–14, 1920) gave the name “late infantile”. This term is convenient clinically but when more examples of the variant had been reported it became evident that the group of cases so designated was not homogeneous. Wyburn-Mason (1943) was able to show in a large clinical material that many of these intermediate forms belonged either to families affected by Tay-Sachs' or by Batten's disease and that in an individual case the ophthalmological findings were usually of decisive importance in classification. The existence of a precocious variant of the classical “juvenile” type of amaurotic idiocy was thus clearly established. Klenk's (1939) discovery that the nerve cells in Tay-Sachs' disease contain large amounts of a glycolipid subsequently called “ganglioside” has provided a further valuable criterion in distinguishing this form of lipidosis from other members of the group. The present case, an example of the precocious juvenile type of amaurotic idiocy or, as we prefer to name it, the subacute form of Batten's disease, is presented as a further contribution to this subject.

Type
Original Articles
Information
Journal of Mental Science , Volume 104 , Issue 434 , January 1958 , pp. 91 - 102
Copyright
Copyright © Royal College of Psychiatrists, 1958 

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References

Aronson, S. M., Volk, B. W., and Epstein, N., Amer. J. Path., 1955, 31, 609.Google Scholar
Batten, F. E., Trans. Ophth. Soc. U.K., 1903, 23, 386.Google Scholar
Idem and Mayou, M. S., Proc. Roy. Soc. Med., 1915, 8, iii (Ophth) 70.Google Scholar
BIELSCHOWSKY, M., Dtsch. Z. Nervenheilk., 1913–14, 50, 7.Google Scholar
Idem, J. f. Psychol. u. Neurol., 1920, 26, 123.Google Scholar
Idem, Z.f. d. ges. Neurol., 1936, 155, 321.Google Scholar
Blix, G., Acta Chem. scand., 1948, 2, 467.Google Scholar
Brante, G., Acta physiol. scand., 1949, 18, suppl. 63.Google Scholar
Brodmann, K., Z. f. d. ges. Neurol., (Ref.), 1914, 10, 91.Google Scholar
Cordes, F. C., and Horner, W. D., Amer. J. Ophth., 1929, 12, 558.Google Scholar
Cumings, J. N., Brain, 1953, 76, 551.Google Scholar
Diezel, P. B., Dtsch. Z. Nervenheilk., 1954a, 171, 344.Google Scholar
Idem, Virchows Arch. path. Anat., 1954b, 326, 89.Google Scholar
Edgar, G. W. F., Myelination studied by quantitative determination of myelin lipids, 1955. Amsterdam.Google Scholar
Idem, Mschr. Psychiat. Neurol., 1956, 131, 274.CrossRefGoogle Scholar
Frets, G. P., and Overbosch, J. F. A., Nederl. Tijdschr. v. Geneesk., 1923, 67, ii, 1091.Google Scholar
Globus, J., Z. f. d. ges. Neurol., 1923, 85, 424.Google Scholar
Greenfield, J. G., Proc. Roy. Soc. Med., 1951, 44, 685.Google Scholar
Idem , Aring, C. D., and Landing, B. H., Neurology, 1955, 5, 732.Google Scholar
Idem and Nevin, S., Trans. Ophth. Soc. U.K., 1933, 53, 170.Google Scholar
Haddenbrock, S., Arch. f. Psychiat., 1950, 185, 129.Google Scholar
Hassin, G. B., Arch. Neurol. Psychiat., 1926, 16, 708.Google Scholar
Idem and Parmelee, A. H., Amer. J. Dis. Child., 1928, 35, 87.Google Scholar
Higier, H., Dtsch. Z. Nervenheilk., 1906, 31, 231.Google Scholar
Holmes, G. M., Proc. Roy. Soc. Med., 1911, 4, iii (Path), 199.Google Scholar
Ichikawa, K., Klin. Mbl Augenheilk., 1909, 47, 73.Google Scholar
Jansky, J., Rev. med. tcheque, 1908, 1, 58.Google Scholar
Jervis, G. A., Amer. J. Dis. Child., 1940, 60, 88.Google Scholar
Klenk, E., Hoppe-Seylers Z. physiol. Chem., 1939, 262, 128.Google Scholar
Idem, Fifth International Neurological Congress, Lisbon, 1953, Vol. I, Reports, p. 253.Google Scholar
Idem and Langerbeins, H., Hoppe-Seylers Z. physiol. Chem., 1941, 270, 185.Google Scholar
Marinesco, G., J. f. Psychol. u. Neurol., 1930, 41, 1.Google Scholar
Norman, R. M., Urich, H., and Lloyd, O. C., J. Path. Bact., 1956, 72, 121.Google Scholar
MüLBERGER, A., Münch. Med. Wschr., 1903, 50, 1969.Google Scholar
Richter, R., and Parmelee, A. H., Amer. J. Dis. Child., 1935, 50, 111.Google Scholar
Rogalski, T., Arch. f. Psychiat., 1910, 47, 1195.Google Scholar
Schlesinger, B., Greenfield, J. C., and Stern, R. O., Arch. Dis. Childh., 1934, 9, 1.Google Scholar
Schmidt, G., Benotti, J., Hershman, B., and Thannhauser, S. J., J. Biol. Chem., 1946, 166, 505.Google Scholar
Schob, F., Z. f. d. ges. Neurol., 1912, 10, 303.CrossRefGoogle Scholar
Smits, G., and Edgar, G. W. F., J. Neuropath. exp. Neurol., 1957. In print.Google Scholar
Steegman, A. T., and Karnosh, L. J., Amer. J. Psychiat., 1936, 92, 1413.Google Scholar
Svennerholm, L., J. Neurochem., 1956, 1, 42.Google Scholar
Tingey, A. H., J. Ment. Sci., 1956, 102, 851.Google Scholar
Torrance, H. W., Glasgow Med. J., 1922, 97, 193.Google Scholar
Wolfsohn, J. M., Arch. Int. Med., 1915, 16, 257.Google Scholar
Wyburn-Mason, R., Brit. J. Ophth., 1943, 27, 145, 193.Google Scholar
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