Læknablaðið - 01.02.1978, Qupperneq 49

LÆKNABLAÐIÐ
19
o.s.frv., og er þetta áberandi hjá bræðrum
þeim, sem hér um ræðir.
Með aldrinum læra sjúkl. að nota aðrar
tegundir skynjunar sér til varnar. Er það
þá gjarnan túlkað svo að sársaukaskynið
hafi komið með aldrinum. Að sjálfsögðu
er mjög torvelt að sanna eða afsanna þetta,
þar sem um er að ræða huglægt fyrirbæri.
Hár arcus pedis var mjög áberandi hjá
systkinum, sem skoðuð voru og móður
þeirra, og bræðurnir hafa allir byrjandi
skalla, sem minnir á þann, sem sést við
dystrophia myotonica.
SUMMARY
Analgesia congenita in an Icelandic family.
This syndrome was first described in 1932 by
Dearborn and since then about 60 cases have
been reported. In some instances more than
one case has been found in a family, but the
majority are isolated. In this study three
brothers were found to be affected. The
criteria for diagnosis, clinical, neurological and
electrophysiological examinations of the af-
fected and examinations of unaffected rela-
tives are described. Differential diagnoses and
theories of etiology are discussed.
HEIMILDIR
1. Aguayo, A.J., Nair CPV, Bray GM: Peri-
pheral nerve abnormalities in the Riley-
Day syndrome. Arch.Neurol. Vol. 24:106-
116, 1971.
2. Behse, F. and Buchtal F.: Normal sensory
conduction in the nerves of the leg in man.
J.Neurol.Neurosurg.Psychiat. 34:404-414,
1971.
3. Bleuler, E.: Lehrbuch der . Psychiatrie
Umgearbeitet von Manfred Bleuler. Sprin-
ger 1966.
4. Buchthal, F. and Rosenfalck, A.: Evoked
action potential and conduction velocity in
human sensory nerves. Brain Research 3
(special issue) 1-122, 1966.
5. Burke, D., Skuse NF, Lethlean AK: Sen-
sory conduction of the sural nerve in poly-
neuropathy. J.Neurol.Neurosurg.Psychiat.
37:647-652. 1974.
6. Dearborn GVN: A case of congenital gene-
ral pure analgesia. J. Ner\'.Ment.Dis., Vol.
75:612-615, 1932.
7. Denny-Brown, D.: Hereditary sensory radi-
cular neuropathy. J.Neurol.Neurosurg.Psy-
chiat., 14:237-252, 1951.
8. DiBenedetto, M.: Sensory nerve conduction
in lower extremities. Arch.Phys.Med.Rehab.
51:253-285. 1970.
9. Dyck, PJ., Lambert, EH: Dissociated sen-
sation in amyloidosis.Arch.Neurol 20:490-
507, 1969.
10. Madonick, M.J.: Insensitiveness to pain.
Neurology 4.554-557, 1954.
11. Mayer, D.J. and Liebeskind, J.C.: Pain re-
duction by focal electrical stimulation of
the brain: An anatomical and behavioral
analysis. Brain Research, 68:79-93. 1974.
12. Melzack R. and Wall, PD: Pain mechan-
isms: A new theory. Science 150, 971-979,
1965.
13. Murray, TJ: Congenital sensory neuro-
pathy. Brain 96:387-394, 1973.
14. Ogden, T.E., Robert, F. and Carmichael,
E.A.: Some sensory syndromes in children:
Indifference to pain and sensory neuro-
pathy.Neurol.Neurosurg.Psychiat., 22:267-
276, 1959.
15. Osuntokun, B.O., Odeku, E.I., Luzzato, E.I.:
Congenital pain asymbolia and auditory
imperception. J.Neurol.Neurosurg.Psychiaf.,
31:291-296. 1968.
16. Pétursson, Ezra: Nýjar hugmyndir um sárs-
aukaskynjun og tilfinningasemi. Lbl. 43.
árg., bls. 31-42, 1959.
17. Pétursson, E., Tryggvason, G. and MeCully,
R.S.: Infantile („Congenital") Indifference
to Pain — A Clinical Study of an Icelandic
Family in which three brothers showed the
Characteristic syndrome. Proc. of the Third
World Congress of Psychiatry. Montreal
1961. 495-500.
18. Riley, C.M., Day, R.L., Greeley, D.M. and
Langford, W.S.: Central autonomic dys-
function with defective lacrimation. Pedia-
trics, 3:468-478, 1949.
19. Siogelman, S.S. et al.: Congenital in-
difference to pain. Am.J. Roent. 97:242-
247, 1966.
20. Silverman, F.N., Gilden, J.J.: Congenital
insensitivity to pain: A neurological
syndrome with bizarre skeletal lesions.
Radiology, 72:176-190, 1959.
21. Sternbach, RA: Congenital insensitivity to
pain. A critique. Psychol., Bull. 60:252-264,
1963.
22. Thrush, DC: Congenital insensitivity to
pain. A clinical genetic and neurophysio-
logical study of four children from the
same family. Brain 96:369-386, 1973.