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Neurocutaneous melanosis concurrent with a posterior cranial fossa cyst (Dandy–Walker complex) and an intradural arachnoid cyst of the spinal canal

https://doi.org/10.14412/2074-2711-2020-2-79-85

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Abstract

The paper describes a female patient with neurocutaneous melanosis (NCM) concurrent with Dandy–Walker malformation and a spinal arachnoid cyst, who has been treated in the A.Ya. Kozhevnikov Clinic of Nervous System Diseases. Over the course of one year, the patient had progressive neurological symptoms as central lower paraparesis and coordination disorders; her medical treatment was ineffective. Neuroimaging revealed Dandy–Walker malformation and a spinal arachnoid cyst. Surgical intervention (fenestration of the upper and lower poles of the arachnoid cyst at the CVI-VII and TIX-X level) was performed. After one month, reoperation (commissure resection in the arachnoid cyst cavity at the TV-VI level; implantation of a cystoperitoneal shunt) was made because of the absence of a clinical result. Reversal of neurological disorders was noted postoperatively.
The described clinical case is unique, since NCM is an extremely rare abnormality, especially when concurrent with  Dandy–Walker malformation and an arachnoid cyst. In addition, it is of interest that the manifestation of neurological symptoms and verification of the diagnosis occurred just in adulthood, although NCM is most commonly diagnosed in children.
The present case shows that an operation aimed at improving cerebrospinal fluid dynamics in patients with arachnoid cysts and Dandy–Walker complex in the presence of NCM can lead to a significant reversal of neurological symptoms and improvement in the quality of life despite gross brain structural changes detected by magnetic resonance imaging.

About the Authors

G. Yu. Evzikov
Department of Nervous System Diseases and Neurosurgery; Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia


M. G. Bashlachev
Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia



E. V. Shashkova
Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia



K. A. Belozerskikh
Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia



F. V. Grebenev
Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia



References

1. Belopasov VV, Soprunova IV, Tsotsonava ZhM, Azhkamalov SI. Rokitansky's Disease (Neurocutaneous Melanosis). Neirokhirurgiya i nevrologiya detskogo vozrasta. 2012;(2-3): 36-43. (In Russ.).

2. Kadonaga JN, Barkovich AJ, Edwards MS, Frieden IJ. Neurocutaneous melanosis in association with the Dandy–Walker complex. Pediatr Dermatol. 1992 Mar;9(1):37-43.

3. Ramaswamy V, Delaney H, Haque S, et al. Spectrum of central nervous system abnormalities in neurocutaneous melanocytosis. Dev Med Child Neurol. 2012 Jun;54(6):563-8. doi: 10.1111/j.1469-8749.2012.04275.x. Epub 2012 Apr 2.

4. DeDavid M, Orlow SJ, Provost N, et al. Neurocutaneous melanosis: clinical features of large congenital melanocytic nevi in patients with manifest central nervous system melanosis. J Am Acad Dermatol. 1996 Oct;35(4):529-38.

5. Kinsler VA, Thomas AC, Ishida M, et al. Multiple congenital melanocytic nevi and neurocutaneous melanosis are caused by postzygotic mutations in codon 61 of NRAS. J Invest Dermatol. 2013 Sep;133(9):2229-36. doi: 10.1038/jid.2013.70. Epub 2013 Feb 7.

6. Salgado CM, Basu D, Nikiforova M, et al. BRAF mutations are also associated with neurocutaneous melanocytosis and large/giant congenital melanocytic nevi. Pediatr Dev Pathol. 2015 Jan-Feb;18(1):1-9. doi: 10.2350/14-10-1566-OA.1. Epub 2014 Dec 9.

7. Kü sters-Vandevelde HV, Willemsen AE, Groenen PJ, et al. Experimental treatment of NRAS-mutated neurocutaneous melanocytosis with MEK162, a MEK-inhibitor. Acta

8. Neuropathol Commun. 2014 Apr 8;2:41. doi: 10.1186/2051-5960-2-41.

9. Pedersen M, Kü sters-Vandevelde HVN, Viros A, et al. Primary melanoma of the CNS in children is driven by congenital expression of oncogenic NRAS in melanocytes. Cancer Discov. 2013 Apr;3(4):458-469. doi: 10.1158/2159-8290.CD-12-0464. Epub 2013 Jan 9.

10. Shah KN. The risk of melanoma and neurocutaneous melanosis associated with congenital melanocytic nevi. In: Seminars in cutaneous medicine and surgery. WB Saunders; 2010.

11. Doroshenko MB, Utyashev IA, Demidov LV, Aliev MD. linical and biological features of giant congenital nevi in children. Pediatriya. Zhurnal im. G.N. Speranskogo. 2016;(4): 50-6. (In Russ.).

12. Marnet D, Vinchon M, Mostofi K, et al. Neurocutaneous melanosis and the Dandy–Walker complex: an uncommon but not so insignificant association. Childs Nerv Syst. 2009 Dec;25(12):1533-9. doi: 10.1007/s00381-009-0976-6. Epub 2009 Aug 27.

13. Foster RD, Williams ML, Barkovich AJ, et al. Giant congenital melanocytic nevi: the significance of neurocutaneous melanosis in neurologically asymptomatic children. Plast Reconstr Surg. 2001 Apr 1;107(4):933-41.

14. Lovett A, Maari C, Decarie JC, Marcoux D, et al. Large congenital melanocytic nevi and neurocutaneous melanocytosis: one pediatric center's experience. J Am Acad Dermatol. 2009 Nov;61(5):766-74. doi: 10.1016/j.jaad.2008.11.022. Epub 2009 Sep 18.

15. Agero AL, Benvenuto-Andrade C, Dusza SW, et al. Asymptomatic neurocutaneous melanocytosis in patients with large congenital melanocytic nevi: a study of cases from an Internet-based registry. J Am Acad Dermatol. 2005 Dec;53(6):959-65. Epub 2005 Oct 7.

16. Takayama H, Nagashima Y, Hara M, et al. Immunohistochemical detection of the c-met proto-oncogene product in the congenital melanocytic nevus of an infant with neurocutaneous melanosis. J Am Acad Dermatol. 2001 Mar;44(3):538-40.

17. Di Rocco F, Sabatino G, Koutzoglou M, et al. Neurocutaneous melanosis. Childs Nerv Syst. 2004 Jan;20(1):23-8. Epub 2003 Oct 24.

18. Barkovich A, Frieden I, Williams M. MR of neurocutaneous melanosis. AJNR Am J Neuroradiol. 1994 May;15(5):859-67.

19. Chaloupka J, Wolf R, Varma P. Neurocutaneous melanosis with the Dandy– Walker malformation: a possible rare pathoetiologic association. Neuroradiology. 1996 Jul; 38(5):486-9.

20. Kadonaga JN, Frieden IJ. Neurocutaneous melanosis: definition and review of the literature. J Am Acad Dermatol. 1991 May; 24(5 Pt 1):747-55.

21. Makkar HS, Frieden IJ. Neurocutaneous melanosis. Semin Cutan Med Surg. 2004 Jun; 23(2):138-44.

22. Schaffer JV, McNiff JM, Bolognia JL. Cerebral mass due to neurocutaneous melanosis: eight years later. Pediatr Dermatol. 2001 Sep-Oct;18(5):369-77.

23. Azzoni A, Argentieri R, Raja M. Neurocutaneous melanosis and psychosis: a case report. Psychiatry Clin Neurosci. 2001 Apr;55(2):93-5.

24. Ahmed I, Tope WD, Young TL, et al. Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis. J Am Acad Dermatol. 2002 Aug;47(2 Suppl): S196-200.

25. Holmes G, Wines N, Ryman W. CASE REPORT Giant congenital melanocytic naevus and symptomatic thoracic arachnoid cyst. Australas J Dermatol. 2001 May;42(2):124-8.

26. Nabors MW, Pait TG, Byrd EB, et al. Updated assessment and current classification of spinal meningeal cysts. J Neurosurg. 1988 Mar;68(3):366-77.

27. Barkovich AJ, Kjos BO, Norman D, Edwards MS. Revised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging. AJR Am J Roentgenol. 1989 Dec;153(6):1289-300.

28. Tortori-Donati P, Fondelli MP, Rossi A, Carini S. Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Childs Nerv Syst. 1996 Jun;12(6):303-8.

29. Spennato P, Mirone G, Nastro A, et al. Hydrocephalus in Dandy–Walker malformation. Childs Nerv Syst. 2011 Oct;27(10):1665-81. doi: 10.1007/s00381-011-1544-4. Epub 2011 Sep 17.

30. Correa GG, Amaral LF, Vedolin LM. Neuroimaging of Dandy–Walker malformation: new concepts. Top Magn Reson Imaging. 2011 Dec;22(6):303-12. doi: 10.1097/RMR.0b013e3182a2ca77.

31. Cho IY, Hwang SK, Kim SH. Dandy–Walker malformation associated with neurocutaneous melanosis. J Korean Neurosurg Soc. 2011 Nov;50(5):475-7. doi: 10.3340/jkns.2011.50.5.475. Epub 2011 Nov 30.

32. Alexiou GA, Sfakianos G, Prodromou N. Dandy–Walker malformation: analysis of 19 cases. J Child Neurol. 2010 Feb;25(2):188-91. doi: 10.1177/0883073809338410. Epub 2009 Oct 15.

33. Ugur HC, Torun F, Yilmaz E, Kanpolat Y. Trigeminal neuralgia in a patient with Dandy–Walker malformation. J Clin Neurosci. 2005 Sep;12(7):815-7.

34. Prakash R, Singh LK, Kour J, et al. Psychiatric comorbidities in Dandy–Walker variant disorder. J Neuropsychiatry Clin Neurosci. 2009 Fall;21(4):477-9. doi: 10.1176/appi.neuropsych.21.4.477-a.

35. Alikhan A, Ibrahimi OA, Eisen DB. Congenital melanocytic nevi: where are we now? Part I. Clinical presentation, epidemiology, pathogenesis, histology, malignant transformation, and neurocutaneous melanosis. J Am Acad Dermatol. 2012 Oct;67(4):495.e1-17; quiz 512-4. doi: 10.1016/j.jaad.2012.06.023.

36. Bett BJ. Large or multiple congenital melanocytic nevi: occurrence of neurocutaneous melanocytosis in 1008 persons. J Am Acad Dermatol. 2006 May;54(5):767-77. Epub 2006 Feb 2.

37. Ruiz-Maldonado R, del Rosario BaronaMazuera M, Hidalgo-Galvan LR, et al. Giant congenital melanocytic nevi, neurocutaneous melanosis and neurological alterations. Dermatology. 1997;195(2):125-8.

38. Kinsler VA, Paine SM, Anderson GW, et al. Neuropathology of neurocutaneous melanosis: histological foci of melanotic neurones and glia may be undetectable on MRI. Acta Neuropathol. 2012 Mar;123(3):453-6. doi: 10.1007/s00401-012-0945-0.

39. Chang IC, Chou MC, Bell WR, Lin ZI. Spinal cord compression caused by extradural arachnoid cysts. Clinical examples and review. Pediatr Neurosurg. 2004 Mar-Apr;40(2):70-4.

40. Lee HJ, Cho DY. Symptomatic spinal intradural arachnoid cysts in the pediatric age group: description of three new cases and review of the literature. Pediatr Neurosurg. 2001 Oct;35(4):181-7.

41. Arneja JS, Gosain AK. Giant congenital melanocytic nevi of the trunk and an algorithm for treatment. J Craniofac Surg. 2005 Sep; 16(5):886-93.

42. Arneja JS, Gosain AK. Giant congenital melanocytic nevi. Plast Reconstr Surg. 2007 Aug;120(2):26e-40e.

43. Arneja JS, Gosain AK. Giant congenital melanocytic nevi. Plast Reconstr Surg. 2009 Jul;124(1 Suppl):1e-13e. doi: 10.1097/PRS.0b013e3181ab11be.


For citation:


Evzikov G.Y., Bashlachev M.G., Shashkova E.V., Belozerskikh K.A., Grebenev F.V. Neurocutaneous melanosis concurrent with a posterior cranial fossa cyst (Dandy–Walker complex) and an intradural arachnoid cyst of the spinal canal. Neurology, Neuropsychiatry, Psychosomatics. 2020;12(2):79-85. (In Russ.) https://doi.org/10.14412/2074-2711-2020-2-79-85

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ISSN 2074-2711 (Print)
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