Aagot Løken

Biographical Timeline

• 1911 – Born Aagot Christie on February 1 in Kabelvåg, Norway
• Early career – Trained first as a laboratory technician at Rikshospitalet, where her skill was quickly recognized by Professor Leiv Kreyberg, who encouraged her to study medicine and specialize in neuropathology.
• Married Johan Christian Alfred Løken taking the formal name of Aagot Christie Løken
• 1930s–40s – Medical studies interrupted by WWII; worked as a community doctor and engaged in resistance activity during the German occupation.
• 1946 – Doctor of medicine, University of Oslo 
• 1948 – Studied neuropathology at the Armed Forces Institute of Pathology in Washington, USA
• 1951 – Appointed prosector in neuropathology at Rikshospitalet, becoming Norway’s first neuropathologist.
• 1958 – Specialist physician in neuropathology at Rikshospitalet, a position she held until retirement in 1978.
• Professional roles – First female chair of the Norwegian Neurological Association; Founding force in the Scandinavian Neuropathological Association; Honorary memberships in Norwegian, British, and Scandinavian pathology/neurology societies.
• 2007 – Died January 1, aged 95 in Oslo, Norway

---

Medical Eponyms

Senior-Løken syndrome (1961)

Løken syndrome (now recognized as part of the nephronophthisis–retinitis pigmentosa spectrum) refers to the association of hereditary renal dysplasia/failure with congenital blindness/retinal aplasia, first described by Løken and colleagues in 1961.

Autosomal recessive disease with renal failure in childhood or adolescence (nephronophthisis) and Leber’s congenital amaurosis (tapetoretinal degeneration). There are two types; juvenile and adult. In the juvenile type, onset is in early childhood; there is blindness and death from renal failure before the age of 10. The adult type is marked by a later onset and a milder course.

1961 (March) – Løken, Hanssen, Halvorsen, and Jølster reported two siblings with congenital blindness and progressive renal disease leading to death in childhood. At autopsy, the kidneys showed renal dysplasia and pyelonephritis, while the eyes revealed retinal aplasia.

Two siblings, a boy and a girl, with congenital blindness died at 8 and 9 years of age from renal failure. Autopsy was performed in one case. The kidneys showed evidence of dysplasia and of pyelonephritis. Earlier suggestions that renal dysplasia might be the primary cause in cases of chronic pyelonephritis in children are supported. Examination of the eyes indicated a congenital defect, retinal aplasia.

The present case seems to display two different congenital defects. Both dysplastic kidneys and retinal dysplasias have been described as genetically determined abnormalities… To the authors’ knowledge the occurrence of hereditary renal disease and congenital blindness has not been reported previously

Løken et al 1961: 183

1961 (November), Senior, Friedmann and Braudo also reported on families with an oculorenal syndrome. They did not cite Løken’s paper. Instead, they framed their findings against Fanconi’s description of familial juvenile nephronophthisis (1951) and other European reports.

As far as we are aware this specific association [juvenile familial nephropathy with tapetoretinal degeneration] as a syndrome has not previously been recorded

Senior 1961

The near-simultaneous publications with neither aware of the other’s work, led to the eponym Senior–Løken syndrome. Both papers emphasised the familial pattern, the association with ocular degeneration, and the progressive course of renal failure.

---

Major Publications

• Loken AC. On the relation of atypical pinealomas to teratoid tumours. Acta Pathol Microbiol Scand. 1957;40(5):417-424.
• Loken AC, Hanssen O, Halvorsen S, Jolster NJ. Hereditary renal dysplasia and blindness. Acta Paediatrica. 1961;50(2):177-184. [Senior-Løken syndrome]
• Loken AC, Brodal A. A somatotopical pattern in the human lateral vestibular nucleus. Arch Neurol. 1970;23(4):350-357.

---

Personal communication: With great thanks to Tove Lill Bjaarstad of Oslo, Norway for the detailed additional information.

---

References

Biography

• Skullerud K, Torvik A. Aagot Christie Løken, nekrolog. Den norske patologforening. 2007

Eponym

• Fanconi G, Hanhart E, von Albertini A, Uhlinger E, Dolivo G, Prader A. Die familiäre juvenile Nephronophthise (die idiopathische parenchymatöse Schrumpfniere) [Familial, juvenile nephronophthisis (idiopathic parenchymal contracted kidney)]. Helv Paediatr Acta. 1951 Feb;6(1):1-49.
• Senior B, Friedmann AI, Braudo JL. Juvenile familial nephropathy with tapetoretinal degeneration. A new oculorenal dystrophy. Am J Ophthalmol. 1961;52:625-633.