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A Rare Cause of Obstructive Sleep Apnoea Syndrome – Morbus Madelung. Case Reports


Authors: K. Klobučníková 1;  I. Mucska 2;  B. Kollár 1
Authors‘ workplace: UN Bratislava I. neurologická klinika LF UK v Bratislave 1;  UN Bratislava Pľúcna ambulancia a ambulancia pre poruchy dýchania počas spánku 2
Published in: Cesk Slov Neurol N 2013; 76/109(2): 235-240
Category: Case Report

Overview

Madelung’s disease, or benign symmetric lipomatosis, is a rare condition characterized by diffuse deposition of adipose tissue in the neck, shoulders and upper trunk. It is one of the benign lipomatous diseases. Patients suffer for clinical and cosmetic problems. Possible respiratory discomfort resulting from compression of the upper airways by adipose tissue may lead to obstructive sleep apnoea syndrome. The authors present two cases of a 66-year-old-male and a 50-year-old-male with Madelung’s disease and sleep apnoea syndrome. Adequate diagnosis and indication of noninvasive ventilation (bilevel possitive airway pressure) led to improved sleep respiration in both patients. The authors wish to point out the rare cause of obstructive sleep apnoea syndrome. Since the management of Madelung’s disease is essentially symptomatic, with surgical removal of adipose tissue, indicating noninvasive ventilation in patients with sleep apnoea syndrome may markedly improve their quality of life.

Key words:
Madelung’s disease – sleep – sleep apnoe syndrome


Sources

1. Brodie BC. Clinical lectures on surgery, delivered at St. George’s hospital. Philadelphia: Lea and Blanchard 1846.

2. Boozan JA, Maves MD, Schuller DE. Surgical management of massive bening symmetric lipomatosis. Laryngoscope 1992; 102(1): 94–99.

3. Biasi D, Caramaschi P, Scarletto A, Baracchino F, Botto M, Pacor ML et al. Symmetric multiple lipomatosis with Charcot‘s joint and neuropathic ulcer. Description of a clinical case. Minerva Med 1993; 84(3): 135–139.

4. Chan ES, Ahuja AT, King AD, Lau WY. Head and neck cancers associated with Madelung‘s disease. Ann Surg Oncol 1999; 6(4): 395–397.

5. Nielsen S, Levine J, Clay R. Adipose tissue metabolism in benign symmetric lipomatosis. J Clin Endorinol Metab 2001; 86(6): 717–720.

6. Novotná I. Epilepsie a metabolický syndróm X. Neurológia pre prax 2011; 12(3): 185–186.

7. Hržičová J, Jančo S, Kollár B, Ondriáš F, Turčáni P. Dercumova choroba (lipomatosis dolorosa) – zriedkavo diagnostikované ochorenie: kazuistika. Cesk Slov Neurol N 2007; 70/103(6): 707–709.

8. Gámez J, Playán A, Andreu AL, Bruno C, Navarro C, Cervera C, Arbós MA et al. Familiar multiple symmetric lipomatosis associated with the A8344G mutation of mitochondrial DNA. Neurology 1998; 51(1): 258–260.

9. Dercum FX. Three cases of hitherto unclassified affliction resembling in its grosser aspects of obesity, but associated with special nervous system – Adiposis dolorosa. Am J Med Sci 1892; 104(6): 521–535.

10. Camperon R, Mankin H, Louis DN, Hirano M, Maccolin M. Familiar occurence of adiposis dolorosa. J Am Acad Dermatol 2001; 44(1): 132–136.

11. Worthan NC, Tomlison IP. Decrum’s disease. Skinmed 2005; 4(3): 157–162.

12. Chuang Ch, Cheng Z, Chang P, Lin Ch. Madelung’s disease, case report. J Chin Med Assoc 2004; 67(11): 591–594.

13. Rechtschaffen A, Kales A. Manual of standardized terminology, techniques and scoring system for sleep stages of human subjects. Washington: NIH Publication 204, 1968: 2–11.

14. Johns MW. A new method for measuring daytime sleepiness: The Epworth sleepiness scale. Sleep 1991; 14(6): 540–545.

15. Cascardon MA, Dement WC. The multiple sleep latency test: what does measure? Sleep 1982; 5 (Suppl 2): 67–72.

16. Mucska I. Diagnostika a liečba spánkových porúch dýchania. Neurológia 2011; 6(1): 19–23.

17. Nevšímalová S, Šonka K et al. Poruchy spánku a bdění. 2nd ed. Praha: Galén 2007.

18. The International Classification of Sleep Disorders. 2nd ed. Westchester: American Academy of Sleep Medicine 2005.

19. Martin JM, Agusti A, Villar I, Forner M, Nieto D, Carrizo SJ et al. Association between treated and untreated obstructive sleep apnea and risk of hypertension. JAMA 2012; 307(20): 2169–2176.

Labels
Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 2

2013 Issue 2

Most read in this issue
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