2. Definition
Definition
Phakos, oma, osis.
Van der Hoeve in 1921
Neuro-cutaneous
syndromes.
Exception
Sturge Weber syndrome
(no neoplastic component)
Von Hippel Lindau disease
(no cutaneous manifestation)
Yakovlev and Guthrie in 1931
Dysplasia & / or
TS & NF
neoplasms of tissues of (have endodermal &
ectodermal origin
mesodermal lesions).
Yakovlev and Guthrie in 1931
40. DD of tuberous sclerosis
DD of
subependymal
nodules
Non clacified
Subependymal heterotopia
Calcified
TORCH
DD of cortical tubers
Focal cortical dysplasia
DD of SGCA
Foramen of Monro masses
41. DD of subpendymal nodules
Subependymal nodules
Subependymal hetertopia
Rounded or oval with their Oval with their long axis
long axis perpendicular the parallel to the ventricular
ventricular wall.
wall.
Variable.
Iso-intense to grey matter
on all pulse sequences
May be calcified.
May be enhancing.
Never calcified.
No enhancement
87. NF
Types of neurofibroma
• Localized (involve a short segment).
• Plexiform (involve a long segment & its
branches).
• Diffuse (infiltrate subcutaneous fat).
• A short segment means (a nerve can be traced
entering & exiting from the mass).
• A long segment means ( a nerve can not be
traced entering or exiting from the mass).
106. Diagnostic criteria of NF1
Two of the following must be present to diagnose as NF1:
• 6 or more cafe-au-lait macules .
• 2 or more neurofibromas of any type or 1 plexiform
neurofibroma,
• freckling in the axillary or inguinal regions
• optic glioma,
• 2 or more Lisch nodules,
• distinctive bony lesion (i.e. sphenoid dysplasia, thinning of
long bone cortex with or without pseudoarthrosis),
• a first degree relative diagnosed with NF1.
147. Diagnostic Criteria for NF-2
• Definite NF2
Bilateral vestibular schwannomas (VS)
• Probable NF2:
Family history of NF2 (first degree family relative) +
Unilateral VS or any two of the following:
– Meningioma,
– Glioma,
– Schwannoma,
– Juvenile posterior subcapsular lenticular opacity,
– Juvenile cortical cataract
158. CP angle
Meningioma
CP angle
Schwannoma
Intra-canalicular extension, with
widening of the internal auditory
.canal
Wide dural base
Centered upon the porus
.acousticus
Obtuse angle along the
anterior & posterior borders
Acute angle along the anterior &
posterior borders
.Dural tail enhancement
Hyperostosis
.Microhemorrhage on T2* Wis