The Pituitary Gland in the Laurence-Moon Syndrome

Whitaker, Michael; Scheithauer, Bernd W.; Kovács, Kálmán; Randall, Raymond V.; Campbell, Robert J.; Okazaki, Haruo

doi:10.1016/s0025-6196(12)62445-8

Cited by 6 publications

(3 citation statements)

References 19 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The degree of mental deficiency, for example, has been reported to range from Full Scale IQs of 30 on the Wechsler-Bellevue (Bowen, Ferguson, Mosier, Lee, & Butler, 1965) to 80 on the Wechsler Adult Intelligence Scale-Revised (Wechsler, 1981;Rizzo et al, 1986). Similarly, histopathologic findings in the eyes of patients with LMS have ranged from widespread loss of the retinal pigment epithelium (membrane covering the retina) and the photoreceptive layer to normal retinal pigment epithelium and partial loss of the photoreceptor layer (Whitaker et al, 1987). Hypogenitalism, by comparison, has been reported in 86% of male patients with LMS (Klein & Ammann, 1969).…”

Section: Discussionmentioning

confidence: 99%

Laurence-Moon syndrome.

Nathanson¹

1998

View full text Add to dashboard Cite

In 1866, Laurence and Moon described four members of a single family with a combination of mental retardation, short stature, obesity, hypogenitalism (unusually small genitalia), and retinitis pigmentosa (constriction of the visual fields leading to progressive vision loss). Hutchinson (1900) later wrote about the same family, adding that spastic paraplegia (increased lower extremity muscle tone) was present in three of the four children. Bardet (1920) and Biedl(1922) independently wrote regarding a similar syndrome that included polydactyly (having more than five fingers or toes per hand or foot). Historically, these various clinical presentations have been recognized as the single disorder Laurence-Moon-Bardet-Biedl syndrome (LMBBS). Recently, however, many researchers have supported the nosologic splitting of LMBBS into the Laurence-Moon syndrome (LMS) and the Bardet-Biedl syndrome (BBS; Farag

show abstract

Section: Discussionmentioning

confidence: 99%

Laurence-Moon syndrome.

Nathanson¹

1998

View full text Add to dashboard Cite

show abstract

“…The common occurrence of hypogonadism among pa tients with Laurence-Moon-Biedl syndrome has been shown at autopsy by signs including small testes, atrophy of the thymus, and gynecomastia [5]. Documentation of normal hypothalamic-pituitary endocrine function and testicular morphologic features in other patients suggests that an evolving primary gonadal disorder is present [6], In contrast.…”

Section: Discussionmentioning

confidence: 99%

Hypothalamic Hamartoma Associated with Laurence-Moon-Biedl Syndrome

Prados

1991

Pediatr Neurosurg

View full text Add to dashboard Cite

show abstract

“…The hypo-gonadism has been variously attributed to unresponsiveness of target organs to gonadal hormones, or primary end-organ failure, or hypothalamic/ pituitary dysfunction. 100 However, hypothalamicpituitary function testing appears to be normal in most cases. 101 The syndrome may be transmitted by polygenic inheritance.…”

Section: Disorders With Associated Obesity and Menstrual Dysfunctionmentioning

confidence: 99%

The Impact of Obesity on Reproductive Function

Garner¹

1990

Semin Reprod Med

View full text Add to dashboard Cite

The Pituitary Gland in the Laurence-Moon Syndrome

Cited by 6 publications

References 19 publications

Laurence-Moon syndrome.

Laurence-Moon syndrome.

Hypothalamic Hamartoma Associated with Laurence-Moon-Biedl Syndrome

The Impact of Obesity on Reproductive Function

Contact Info

Product

Resources

About