Rod densitometry in congenital stationary night blindness

Johan E. E. Keunen; Gerard J. van Meel; Dirk van Norren

doi:10.1364/AO.27.001050

Applied Optics
Vol. 27,
Issue 6,
pp. 1050-1056
(1988)
•https://doi.org/10.1364/AO.27.001050

Rod densitometry in congenital stationary night blindness

Johan E. E. Keunen, Gerard J. van Meel, and Dirk van Norren

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Johan E. E. Keunen, Gerard J. van Meel, and Dirk van Norren, "Rod densitometry in congenital stationary night blindness," Appl. Opt. 27, 1050-1056 (1988)

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Abstract

Rod densities were measured in ten patients with congenital stationary night blindness (CSNB) using the Utrecht densitometer. In five patients rod density was within the range observed in a group of control subjects. One of these patients reached a normal rod two-way density after a prolonged regeneration time. In five patients with different hereditary patterns of CSNB we found decreased amounts of rhodopsin. These observations deviate from the findings on four CSNB patients hitherto described in the literature, where normal concentrations and kinetics of rhodopsin were found. We observed no relationship between the age of CSNB patients and mode of rhodopsin kinetics. No inconsistencies between rod densitometry and electroretinography were revealed. The different electroretinography patterns were not helpful in predicting the densitometric responses in CSNB. The present results included, five of fourteen CSNB patients ever studied with the technique of retinal densitometry show abnormal rhodopsin kinetics. We propose a schema of CSNB subtyping based on the different rhodopsin kinetics.

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Patient no. sex, age (yr)	Visual acuity, L.E.	Mode of inheritance	Rod two-way Density L.E.^a	Densitometric rod time constant (s) L.E.^a	Ampl. scot. a-wave (medium flash) L.E.	Ampl. Scot. b-wave (μV) (medium flash) L.E.^d	Implicit time scot. b-wave (medium flash) L.E.	Oscillatory potentials L.E.	Arden ratio R.E.– L.E.	Psychoph. rod threshold elevation at 30 min in dark (log units) L.E.
1,M,15	0.35(S-11.50C-2.25 × 175)	X-linked recessive	0.18	302	Normal	120	Normal	Absent	1.60–1.40	1.5
2,M,14	0.35(S-5.50C-1.50 × 15)	X-linked recessive	0.18	246	Normal	150	Normal	Absent	^c	3.0
3,M,35	0.75(S-11.50)	X-linked recessive	0.08	^b	Normal	110	Normal	Normal	2.04–1.80	2.3
4,M,24	0.5(S-16.00C-2.00 × 15)	X-linked recessive	0.10	272	Normal	60	Normal	Normal	2.58–2.77	3.0
5,M,21	0.75(S-4.00)	X-linked recessive	0.07	^b	Normal	50	Normal	Absent	1.80–1.77	3.0
6,M,37	0.10(S-9.00)	X-linked recessive	0.07	^b	Normal	100	Normal	Abnormal	^c	2.5
7,M,25	1.5(plano)	Autosomal dominant	0.11	195	Normal	80	Normal	Absent	2.15–1.84	1.2
8,M,27	1.0(S-9.50C-0.75 × 90)	Autosomal dominant	0.06	^b	Normal	50	Normal	Absent	2.03–2.03	3.0
9,M,19	1.5(S-4.50)	Autosomal dominant	0.14	259	Normal	80	Normal	Absent	1.61–1.61	2.4
10,M,17	1.50(plano)	Autosomal dominant	0.14	188	Normal	300	Normal	Normal	^c	2.0

Types of rhodopsin kinetics	Observed scotopic ERG patterns	Observed modes of inheritance^a	Number of cases	Origin of study
Type Ia
Normal rhodopsin concentration Normal regeneration time	Normal scotopic a-wave and subnormal scotopic b-wave;	AD	1	K (patient 10)
	Normal scotopic a-wave and decreased scotopic b-wave;	XR, AD, AR	4	C,K (patient 2,7,9)
	Normal scotopic a-wave and absent scotopic b-wave;	Not given	1	A
	Absent scotopic a-wave and absent scotopic b-wave	AD	1	C
Type Ib
Normal rhodopsin concentration Prolonged regeneration time	Normal scotopic a-wave and decreased scotopic b-wave	XR	1	K (patient 1)
Type II
Decreased rhodopsin concentration Normal regneration time	Normal scotopic a-wave and decreased scotopic b-wave	XR, AD	5	K (patient 3,4,5,6,8)

Patient no. sex, age (yr)	Visual acuity, L.E.	Mode of inheritance	Rod two-way Density L.E.^a	Densitometric rod time constant (s) L.E.^a	Ampl. scot. a-wave (medium flash) L.E.	Ampl. Scot. b-wave (μV) (medium flash) L.E.^d	Implicit time scot. b-wave (medium flash) L.E.	Oscillatory potentials L.E.	Arden ratio R.E.– L.E.	Psychoph. rod threshold elevation at 30 min in dark (log units) L.E.
1,M,15	0.35(S-11.50C-2.25 × 175)	X-linked recessive	0.18	302	Normal	120	Normal	Absent	1.60–1.40	1.5
2,M,14	0.35(S-5.50C-1.50 × 15)	X-linked recessive	0.18	246	Normal	150	Normal	Absent	^c	3.0
3,M,35	0.75(S-11.50)	X-linked recessive	0.08	^b	Normal	110	Normal	Normal	2.04–1.80	2.3
4,M,24	0.5(S-16.00C-2.00 × 15)	X-linked recessive	0.10	272	Normal	60	Normal	Normal	2.58–2.77	3.0
5,M,21	0.75(S-4.00)	X-linked recessive	0.07	^b	Normal	50	Normal	Absent	1.80–1.77	3.0
6,M,37	0.10(S-9.00)	X-linked recessive	0.07	^b	Normal	100	Normal	Abnormal	^c	2.5
7,M,25	1.5(plano)	Autosomal dominant	0.11	195	Normal	80	Normal	Absent	2.15–1.84	1.2
8,M,27	1.0(S-9.50C-0.75 × 90)	Autosomal dominant	0.06	^b	Normal	50	Normal	Absent	2.03–2.03	3.0
9,M,19	1.5(S-4.50)	Autosomal dominant	0.14	259	Normal	80	Normal	Absent	1.61–1.61	2.4
10,M,17	1.50(plano)	Autosomal dominant	0.14	188	Normal	300	Normal	Normal	^c	2.0

Types of rhodopsin kinetics	Observed scotopic ERG patterns	Observed modes of inheritance^a	Number of cases	Origin of study
Type Ia
Normal rhodopsin concentration Normal regeneration time	Normal scotopic a-wave and subnormal scotopic b-wave;	AD	1	K (patient 10)
	Normal scotopic a-wave and decreased scotopic b-wave;	XR, AD, AR	4	C,K (patient 2,7,9)
	Normal scotopic a-wave and absent scotopic b-wave;	Not given	1	A
	Absent scotopic a-wave and absent scotopic b-wave	AD	1	C
Type Ib
Normal rhodopsin concentration Prolonged regeneration time	Normal scotopic a-wave and decreased scotopic b-wave	XR	1	K (patient 1)
Type II
Decreased rhodopsin concentration Normal regneration time	Normal scotopic a-wave and decreased scotopic b-wave	XR, AD	5	K (patient 3,4,5,6,8)

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Applied Optics