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Launois Syndrome

Acromegalic Gigantism

Launois syndrome (acromegalic gigantism) is typically caused by a pituitary tumor that secretes excess growth hormone. This condition results in the enlargement of skeletal and soft tissue, leading to characteristic gigantism and acromegaly.


Presentation

Launois syndrome (LS), also known as acromegalic gigantism or pituitary gigantism, stems from the excessive production of growth hormone (GH) by the pituitary gland in the context of pituitary adenoma or hyperplasia, which sits in an enlarged sella turcica [1] [2]. At least 50% of LS cases are genetic in origin [3]. LS can occur as a mutation (for example in aryl hydrocarbon receptor interacting protein (AIP) gene), X-linked, familial or sporadic disorder.

There are two main phenotypic changes, namely gigantism, and acromegaly. Acromegaly typically occurs in adulthood, after the closure of the growth plates and sometimes in older children [4]. Gigantism typifies childhood onset of disease, when epiphyseal plates have not closed, leading to excessive growth of bones and thus an abnormally tall stature. Individuals with gigantism may experience less of the typical acromegaly features.

Abnormal growth is mediated by increased secretion of insulin-like growth factor I (IGF-I). Hyperprolactinemia is also common and may present as hypothyroidism, galactorrhea in females, and adrenal insufficiency. The former is associated with larger tumors and earlier onset of disease [5].

Acromegaly is insidious in onset, often occurring over years. In such cases, tumors are slow growing, leading to the average presentation in the fifth decade. Its clinical manifestations are a result of both skeletal and soft tissue growth, and these characteristically entail enlargement of the jaw, lips, nose and internal organs, for example, goiter. Furthermore, there is a growth of feet and hands, excessive sweating, increased body odor, arthropathy, joint stiffness and pain, and osteoarthritis-like features [6] [7].

Symptoms related to the compressive effects of the tumor include headaches, visual disturbances such as bitemporal hemianopsia, and signs of hypopituitarism. The latter happens when tumor proliferation damages the pituitary stalk and interrupts communication between the pituitary gland and the hypothalamus, thereby effectively severing the inhibitory mechanisms.

Additional findings of LS include obstructive sleep apnea, diabetes mellitus, heart disease, dyslipidemia and dysregulation of calcium metabolism and gastrointestinal (colon) malignancies.

Goiter
  • Its clinical manifestations are a result of both skeletal and soft tissue growth, and these characteristically entail enlargement of the jaw, lips, nose and internal organs, for example, goiter.[symptoma.com]
  • Differential diagnosis should include the various lesions of the subcutaneous cellular tissue, such as lipomas, angiodysplasias, neurofibromas, sarcomas, goiters, sialoadenitis, obesity and lymphatic tumors.[rbcp.org.br]
  • Also in our patient, a subtotal resection of the thyroid was done in 1991 due to a nodular goiter and thereafter thyroxin was substituted. However, there was no hypothyrosis.[dercums_data.tripod.com]
Accelerated Growth
Hypertension
  • The syndrome is often associated with features of metabolic syndrome such as diabetes mellitus, hyperuricemia, hyperlipidemia and hypertension and is associated with polyneuropathy which is an integral part of the disease.[ncbi.nlm.nih.gov]
  • The prevalence of atherogenic risk factors as hypertension (80%), type 2 diabetes (30%), OSAS (50%), smoking (30%) and alcohol abuse (80%) was high in the (obese) LBS group.[ncbi.nlm.nih.gov]
  • Due to the massive accumulation of adipose tissue, the patients frequently have features of the metabolic syndrome as hypertension, impaired fasting glucose or diabetes mellitus, hyperuricemia or hyperlipidemia.[ncbi.nlm.nih.gov]
  • The patient was treated for hypertension and healthier lifestyle, and diet methods were suggested to improve the metabolic syndrome. The patient was subsequently referred to a plastic surgeon for liposuction of inguinal lipoma.[ncbi.nlm.nih.gov]
  • Authors : Ojoga, Florina 1 ; Guşiţă, Veronica 2 Subjects: * CEREBROVASCULAR disease risk factors ; * HYPERTENSION risk factors ; * SMOKING Source: Romanian Medical Journal. 2010, Vol. 57 Issue 1, p31-33. 3p.[biblioteca.sagrado.edu]
Macroglossia
  • .: Symmetriclipomatosis of the tongue presenting as macroglossia: Report of two cases. J Craniomaxillofac Surg 1993;7:298-301.[dryoho.com]
Muscle Weakness
  • Woman with Leg Weakness and Atrophic Muscle Fibers on Biopsy 554 Case 89A A Woman with Muscle Weakness and a Skin Rash 559 Case 90 A Man with Muscle Pains 567 Case 91 An Older Woman with Progressive Muscle Weakness 572 Case 92 An HIVInfected Man with[books.google.com]
  • There is delayed puberty, muscle weakness, headache, perspiration; joint pain. At approximately 18 to 20 years of age abnormal growth starts and continues until 27 to 30 years of age. Possible, mental retardation, cephalgia, and visual defects.[whonamedit.com]
Increased Body Odor
  • Furthermore, there is a growth of feet and hands, excessive sweating, increased body odor, arthropathy, joint stiffness and pain, and osteoarthritis-like features.[symptoma.com]
Headache
  • Symptoms related to the compressive effects of the tumor include headaches, visual disturbances such as bitemporal hemianopsia, and signs of hypopituitarism.[symptoma.com]
  • There is delayed puberty, muscle weakness, headache, perspiration; joint pain. At approximately 18 to 20 years of age abnormal growth starts and continues until 27 to 30 years of age. Possible, mental retardation, cephalgia, and visual defects.[whonamedit.com]
  • If a tumor is present, there may be drowsiness and symptoms of increased intracranial pressure (for example, subtle locus of control changes and headache).[medical-dictionary.thefreedictionary.com]
  • Common side effects include shakiness, headache, fast heart rate, dizziness, serious side effects may include worsening bronchospasm, irregular heartbeat, and low blood potassium levels.[wikivisually.com]
  • Headache (tension headache, migraine, cervicogenic headache).[dercums_data.tripod.com]
Peripheral Neuropathy
  • The peripheral neuropathy has often been laid to alcoholism, but the neuropathy is likely an integral part of the syndrome. This disorder affects mainly men and is more frequent in the Mediterranean area.[medicinenet.com]
  • Neuropathy 405 Case 64 A Man with a Neuropathy Weight Loss and Lung Nodules 411 Case 65 A Woman with Difficulty Walking and Ataxia 415 Case 66 A Man with Progressive Neuropathy and Congestive Heart Failure 422 Case 67 An Elderly Woman with a Progressive[books.google.com]
  • MSL is associated with increased alcohol intake in up to 95% of cases and patients have also been known to suffer from other comorbidities such as peripheral neuropathy (80% of patients), macrocytic anemia, alcoholic fatty liver or cirrhosis, hyperuricemia[clinmedjournals.org]
  • ., and Donaghy, M. (1990) Familial multiple symmetric lipomatosis with peripheral neuropathy. Neurology 40, 1246-1250 17.[lipomadoc.org]
Bitemporal Hemianopsia
  • Symptoms related to the compressive effects of the tumor include headaches, visual disturbances such as bitemporal hemianopsia, and signs of hypopituitarism.[symptoma.com]
Paresthesia
  • The patient intermittently complained of tingling paresthesia at the fingertips accompanied by grip impairment. In addition, there was a predisposition to ecchymoses.[dercums_data.tripod.com]

Workup

Diagnosis of Launois syndrome relies on clinical evaluation and biochemical testing. Because of its slow progression, diagnosis can be made up to 10 years after disease onset. Biochemical parameters that are typically measured include:

  • Growth hormone: GH levels will be elevated. In some cases, the gonadotropin releasing hormone may also be overabundant, in instances where the secreting tumor is not located in the pituitary gland. GH measurements may be done after an oral glucose tolerance test (OGTT), where positive findings are GH in excess of 1ng/ml.
  • Insulin-like growth factor I: This will also be raised [8].
  • Prolactin: High prolactin levels are often reported.

Radiography of the skull and limbs is one of the imaging modalities carried out. Both computerized tomography (CT) and magnetic resonance imaging (MRI) scanning are useful in visualizing the tumor, however, MRI is more sensitive.

Screening for possible complications, such as diabetes and cardiovascular disease may involve specific tests such hemoglobin A1c (HbA1c), echocardiography and electrocardiography.

Enlarged Sella
  • Launois syndrome (LS), also known as acromegalic gigantism or pituitary gigantism, stems from the excessive production of growth hormone (GH) by the pituitary gland in the context of pituitary adenoma or hyperplasia, which sits in an enlarged sella turcica[symptoma.com]
Hypertriglyceridemia
  • Associated disorders include liver disease, hyperlipidemia including elevated high density lipoprotein (HDL) levels (in patients with a history of alcohol use), hypertriglyceridemia, hypothyroidism, diabetes mellitus, and neurological changes(12).[lipomadoc.org]

Treatment

  • The current treatment of the disease is described based on the authors' experience. Four cases treated in our department are retrospectively reviewed regarding comorbidities and type of surgery performed.[ncbi.nlm.nih.gov]
  • Therapeutic options are pharmacological treatment with salbutamol and surgical procedures such as lipectomy or liposuction.[ncbi.nlm.nih.gov]
  • The individual’s response to treatment dictates the prognosis of the condition Please find comprehensive information on Froelich's Syndrome regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment[dovemed.com]
  • Treatment depends on the primary cause of the condition, usually a tumor or infection involving the hypothalamus.[medical-dictionary.thefreedictionary.com]
  • Common regions involved include the head and neck area, shoulder, and upper arms Complete excision by surgery or liposuction is the preferred treatment option for Madelung Disease presenting significant symptoms.[dovemed.com]

Prognosis

  • , prevention, prognosis, and additional useful information HERE .[dovemed.com]
  • […] on Madelung Disease regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE .[dovemed.com]
  • Treatment and prognosis Treatment centers around surgical removal, including liposuction, lipectomy or injection lipolysis.[radiopaedia.org]

Etiology

  • Benign symmetric lipomatosis, also known as Madelung's disease or Launois-Bensaude syndrome, is a rare disease, the etiology of which is still unknown.[ncbi.nlm.nih.gov]
  • Export Citation: APA/MLA Format Download EndNote Download BibTex MeSH Terms Descriptor/Qualifier: Head and Neck Neoplasms* Hepatitis / complications Humans Hypersplenism / complications Jaundice / etiology Lipomatosis* Liver Cirrhosis / complications[biomedsearch.com]
  • Pages: 4 Number of Figures: 3 Number of Tables: 1 ISSN: 1018-8665 (Print) eISSN: 1421-9832 (Online) For additional information: Abstract Benign symmetric lipomatosis, also known as Madelung’s disease or Launois-Bensaude syndrome, is a rare disease, the etiology[karger.com]
  • Etiology and Pathophysiology The etiology and pathogenesis of lipomas is unclear. A pathogenetic link between soft tissue trauma and the formation of lipomas has been suggested.[unboundmedicine.com]
  • . -------------------------------------------------------------------------------- Figure 1 Launois- Bensaude Syndrome or Madelung' s Disease, is rare disorder of unknown etiology.[proz.com]

Epidemiology

  • Romanian journal of morphology and embryology Revue roumaine de morphologie … , 2017 3 2017 EPIDEMIOLOGY AND PATHOLOGY OF PAROTID TUMORS–FIVE-YEAR RETROSPECTIVE STUDY LB Ungureanu, D Ciobanu, M Danciu, V Costan, C Ungureanu, A Nicolau, ...[scholar.google.ro]
  • Must be differentiated from other tumors, particularly liposarcomas because treatment protocols differ Epidemiology Lipomas can occur at any age but most commonly occur in middle-aged adults, typically in the 40- to 60-year-old age group, and more likely[unboundmedicine.com]
Sex distribution
Age distribution

Pathophysiology

  • She suffered from benign symmetric lipomatosis (BSL), also called Launois-Bensaude syndrome (LBS), which is a rare disorder of unknown origin and poorly understood pathophysiology.[ncbi.nlm.nih.gov]
  • Coupling between the beta-adrenergic receptor and the adenylatecytase-pathophysiological implications. Acta Med Scand. 1983;672:17–20. Google Scholar 15. Klein JA. Tumescent technique for local anesthesia improves safety in large-volume liposuction.[link.springer.com]
  • Possible risk factors include obesity, alcohol abuse, liver disease, glucose intolerance, and soft tissue trauma (see “Etiology and Pathophysiology”).[unboundmedicine.com]
  • Although there might be a genetic predisposition, most cases are sporadic with exact risk factors and pathophysiology unknown [ 5 ].[clinmedjournals.org]

Prevention

  • […] individual’s response to treatment dictates the prognosis of the condition Please find comprehensive information on Froelich's Syndrome regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention[dovemed.com]
  • […] signs and symptoms and the underlying cause of the condition among other factors Please find comprehensive information on Madelung Disease regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention[dovemed.com]
  • […] an estimated incidence rate of 1 in 25,000, and affects males up to 30 times more frequently than females. [2] Cause [ edit ] The cause of the disease remains unknown, but its incidence strongly correlates with alcohol abuse ; abstinence from alcohol prevents[en.wikipedia.org]

References

Article

  1. Chahal HS, Stals K, Unterländer M, et al. AIP mutation in pituitary adenomas in the 18 th century and today. N Engl J Med. 2011;364(1):43-50.
  2. de Herder WW. Acromegaly and gigantism in the medical literature.Case descriptions in the era before and the early years after the initial publication of Pierre Marie (1886). Pituitary. 2009;12(3):236-244.
  3. Rostomyan L, Daly AF, Petrossians P, et al. Clinical and genetic characterization of pituitary gigantism: an international collaborative study in 208 patients. Endocr Relat Cancer. 2015;22(5):745–757.
  4. Gagel RF, McCutcheon IE. Images in clinical medicine.Pituitary gigantism. N Engl J Med. 1999;340(7):524.
  5. Wang M, Mou C, Jiang M, et al. The characteristics of acromegalic patients with hyperprolactinemia and the differences in patients with merely GH-secreting adenomas: clinical analysis of 279 cases. Eur J Endocrinol. 2012;166(5):797-802.
  6. Melmed S. Acromegaly. N Engl J Med. 2006;355(24):2558-2573.
  7. Killinger Z, Payer J, Lazúrová I, et al. Arthropathy in acromegaly. Rheum Dis Clin North Am. 2010;36(4):713-720.
  8. Giustina A, Chanson P, Bronstein MD, et al. A consensus on criteria for cure of acromegaly. J Clin Endocrinol Metab. 2010;95(7):3141-3148.

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Last updated: 2019-07-11 21:40