Cases of hereditary ataxia (a family of five).

Goodhart, Sir James Frederick, 1846-1916 ;
Carpenter, George Alfred, 1859-1910.

Journal : Transactions of the Clinical Society of London, vol. 21.

London : Longmans, Green and Co., 1888.

Description : pp. 268-276, [1 l.] pl. ; ill.: 1 leaf photos., 5 text figs., 1 tbl. ; 22 cm.

Photograph : one leaf with two images, (unverified).

Photographer : W. Talbot Brett.

Subject : Congenital disorders — Ataxia.

Notes :

           THE exact nature of these cases is open to doubt. The symptoms undoubtedly most resemble insular sclerosis, and they are wanting in the usual feature of Friedreich's disease, viz. evidences of posterior sclerosis. The patellar reflex is exaggerated, not absent. Still, as the absence of that reflex is not an invariable feature, it may well be questioned whether Friedreich's disease should not receive a somewhat ampler definition, so as to include cases of this sort. The points which appear to be of especial interest and of probable importance in any attempt to establish the true nature of the disease in the present instance are these :
          1. All the children have had convulsions.
          2. That the youngest child has, as yet, only nystagmus, and the next to it, a boy of three years, is much less affected than are the three elder children.
          3. That the baby, and perhaps the boy of three years, alone have a properly developed head.
          It appears, therefore, that as these children advance in age so they leave the development of their brains behind them ; and this is shown by the cyrtometer tracings of the several heads. — Page 268.


Drs. Goodhart's and G. A. Carpenter's Cases of Hereditary Ataxia.
  1. Rose, aged 8 years.
  2. Wallace, aged 6 1/2 years.
  3. Herbert, aged 5 years.
  4. Teddy, aged 3 years.
  5. Maria, aged 14 months.
  From photographs by Mr. W. Talbot Brett.

•    •    •

The two clinical photographs represent five affected siblings with a nurse attendant standing behind the two oldest children shown in the top photo. Text figures indicate the transverse profiles of the subjects' skulls. NLM lists only one other paper co-authored by Goodhart and Carpenter, titled, Tubercular peritonitis; its natural history, diagnosis, and treatment (1891).

©All rights reserved.