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Neurofibromatosis type 2

آخر تحديث 9 Dec 2022

يتوافق مع الإرشادات التحريرية

Neurofibromatosis type 2 is a disorder characterised by the growth of noncancerous tumours in the nervous system. The most common tumours associated with neurofibromatosis type 2 are called vestibular schwannomas. These growths develop along the nerve that carries information from the inner ear to the brain (the auditory nerve). Tumours that form on the membrane that covers the brain and spinal cord (meninges) are also common in neurofibromatosis type 2. These tumours are called meningiomas. Tumours can also occur on other nerves or tissues in the brain or spinal cord in people with this condition.

الوصف

Neurofibromatosis type 2 is a disorder characterised by the growth of noncancerous tumours in the nervous system. The most common tumours associated with neurofibromatosis type 2 are called vestibular schwannomas. These growths develop along the nerve that carries information from the inner ear to the brain (the auditory nerve). Tumours that form on the membrane that covers the brain and spinal cord (meninges) are also common in neurofibromatosis type 2. These tumours are called meningiomas. Tumours can also occur on other nerves or tissues in the brain or spinal cord in people with this condition.

The signs and symptoms of neurofibromatosis type 2 usually appear during adolescence or in a person's early twenties, although they can begin at any age. The most frequent early symptoms of vestibular schwannomas are hearing loss, ringing in the ears (tinnitus), and problems with balance. Less commonly, vestibular schwannomas cause facial weakness or paralysis. In most cases, these tumours occur in both ears (bilaterally) by age 30. If tumours develop elsewhere in the nervous system, signs and symptoms vary according to their location. Complications of tumour growth can include changes in vision, numbness or weakness in the arms or legs, and fluid buildup in the brain. Some people with neurofibromatosis type 2 also develop clouding of the lens (cataracts) in one or both eyes, often beginning in childhood.

Inheritance patterns

The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.

Related genes

Other names

BANF

Bilateral acoustic neurofibromatosis

Central neurofibromatosis

Familial acoustic neuromas

Neurofibromatosis 2

Neurofibromatosis type 2 merlin

Neurofibromatosis type II

NF2

Schwannoma, acoustic, bilateral

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  • 9 Dec 2022 | أحدث إصدار
  • 1 Mar 2007 | نُشر في الأصل

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