1. Neurology Case Conference 4
Matematico Matias Maulion Medenilla Medina, K. Medina, S. Mejino

2. Clinical Impression
MENINGIOMA

3. Meningioma Second most common primary tumor
15% of all primary intracranial tumors
Mostly benign tumor
Origin: arachnoidal (meningothelial cap) cells
particular from the arachnoid villi
Women:Men (2:1)
Peak Age Incidence: 60th & 70th decades
Vascular Endothelial Growth Factor
Highly vascular, prominent surrounding edema
Meningioma is the second most common primary tumor 15% among the types of incranial tumors.
Meningioma is most benign tumor as illustrateD by Matthew Bailie in his Morbid Anatomy (1787)

4. Etiology of Meningioma
Familial
Truncating mutations in the neurofibromatosis 2 gene (merlin) on chromosome 22q
Radiation Therapy
Previous Head Trauma
Estrogen and Progesterone receptors
Most frequent genetic defects of meningiomas are truncating (inactivating) mutations in the neurofibromatosis 2 gene (merlin) on chromosome 22q. These are present in the great majority of certain meningiomas (e.g., fibroblastic and transitional types), but not others. Merlin deletions probably also play a role in those instances in which there is a loss of the long arm of chromosome 22. In meningiomas of both the sporadic and neurofibromatosis (NF2)-associated types, other genetic defects are found, including deletions on chromosomes 1p, 6q, 9p, 10q, 14q, and 18q.
Radiation therapy to the scalp or cranium appear at an earlier age in such individuals (Rubinstein et al).
Estrogen and progesterone receptors - may relate to the increased incidence of the tumor in women, its tendency to enlarge during pregnancy, and an association with breast cancer.

5. WHO Classification of Meningioma
GRADE / INCIDENCE
HISTOLOGIC SUBTYPES
Benign
Grade I (90%)
Meningothelial (most common), Fibrous, Transitional, Psammomatous, Angioblastic (most aggressive)
Atypical
Grade II (7%)
Choroid, Clear Cell, Atypical
Anaplastic / Malignant
Grade III(2%)
Papillary, Rhabdoid, Anaplastic
World Health Organization (Lopes et al) have divided meningiomas into many subtypes depending on their mesenchymal variations, the character of the stroma, and their relative vascularity, but the value of such classifications is debatable.
Currently neuropathologists recognize a meningothelial (syncytial) form as being the most common. It is readily distinguished from other similar but non-meningothelial tumors such as hemangiopericytomas, fibroblastomas, and chondrosarcomas.

6. Histological Classification of Meningioma
HISTOLOGICAL LABEL
FEATURES
Fibroblastic
Narrow, long cells in sheets; less commonly, Whorls, Psammoma Bodies
Syncytial
Meningiothelial cells, Whorls, Psammoma Bodies
Transitional
Features of both Fibroblastic and Syncytial
Angioblastic
Intertwined, Complex, Thickened Blood Vessels, Reticulin Background, seldom contain Whorls, Psammoma Bodies
Currently neuropathologists recognize a meningothelial (syncytial) form as being the most common. It is readily distinguished from other similar but non-meningothelial tumors such as hemangiopericytomas, fibroblastomas, and chondrosarcomas

7. Clinical Manifestations of Meningioma
Asymptomatic, Incidental finding
Irritation of the underlying cortex  Seizures
Compression of the brain  Localized or Non-specific headaches, Focal or Generalized Cerebral Dysfunction
Compression of the cranial nerves  Focal Neurologic Deficits
Erosion, Invasion or Hyperostosis of Cranial bones
Narrowing, Occlusion of important cerebral arteries  Transient ischemic attack (TIA)–like episodes or as stroke
Asymptomatic, incidental finding - Small meningiomas, less than 2.0 cm in diameter, are often found at autopsy in middle-aged and elderly persons without having caused symptoms. Only when they exceed a certain size and indent the brain do they alter function. The size that must be reached before symptoms appear varies with the size of the space in which the tumor grows and the surrounding anatomic arrangements.
Compression: Localized or nonspecific headaches are common. Compression of the underlying brain can give rise to focal or more generalized cerebral dysfunction, as evinced by focal weakness, dysphasia, apathy, and/or somnolence.
Compression of the cranial nerves  Focal Neurologic Deficits(aphasia, hemianopsia, anosmia, proptosis)
The signs and symptoms secondary to meningiomas may appear or become exacerbated during pregnancy but usually abate or improve in the postpartum period.
Vascular: This presentation, although rare, should be considered. Meningiomas of the skull base may narrow and even occlude important cerebral arteries, possibly presenting either as transient ischemic attack (TIA)–like episodes or as stroke.
Physical
The physical findings mirror the aforementioned symptoms and include signs due to raised intracranial pressure, involvement of cranial nerves, compression of the underlying parenchyma, and involvement of bone and subcutaneous tissues by the meningioma.
Raised intracranial pressure leads to papilledema, decreased mentation and, ultimately, to brain herniation.
Involvement of the cranial nerves may lead to anosmia, visual field defects, optic atrophy, diplopia, decreased facial sensation, facial paresis, decreased hearing, deviation of the uvula, and hemiatrophy of the tongue.
Compression of the underlying parenchyma may give rise to pyramidal signs that are exemplified by pronator drift, hyperreflexia, positive Hoffman sign, and presence of the Babinski sign. Parietal-lobe syndrome may occur if the parietal lobes are compressed.
Compression of the dominant (usually left) parietal lobe may give rise to Gerstmann syndrome: agraphia, acalculia, right-left disorientation, and finger agnosia.
Compression of the nondominant (usually right) parietal lobe leads to tactile and visual extinction and neglect of the contralateral side.
Compression of the occipital lobes leads to a congruent homonymous hemianopsia
Spinal meningiomas may give rise to a Brown-Sequard syndrome (ie, contralateral decreased pain sensation, ipsilateral weakness, decrease in position sense), sphincteric weakness and, ultimately, complete quadriparesis or paraparesis.

8. Sites of Meningioma

9. Sites & Presentations of Meningioma
 90% Intracranial  10% Intraspinal
Parasagittal/Parafalcine
Urinary Incontinence, Demetia, Gradual Paraparesis, Seizures
Tentorial
May protrude within supratentorial and infratentorial compartments, producing symptoms by compressing specific structures within these 2 compartments
Lateral Convexity
Variable depending on structures compressed, including Slow Hemiparesis, Speech Abnormalities
Olfactory Groove
Anosmia, Visual Disturbances, Dementia, Foster-Kennedy Syndrome
Sphenoid Ridge
Extraocular Nerve Paresis, Exostoses, Proptosis, Seizures
Meningiomas in specific locations may give rise to the stereotyped symptoms listed in the Table. These stereotypical symptoms are not pathognomonic of meningiomas in these locations.
Since the clusters of arachnoidal cells penetrate the dura in largest number in the vicinity of venous sinuses, these are the sites of predilection for the tumor.
They may indent the brain and acquire a pia-arachnoid covering as part of their capsule, but they are clearly demarcated from the brain tissue (extra-axial) except in the unusual circumstance of a malignant invasive meningioma. Rarely, they arise from arachnoidal cells within the choroid plexus, forming an intraventricular meningioma.
90% Intracranial
Sylvian region, superior parasagittal surface of the frontal and parietal lobes, olfactory groove, lesser wing of the sphenoid bone, tuberculum sellae, superior surface of the cerebellum, cerebellopontine angle
Some meningiomas—such as those of the olfactory groove, sphenoid wing, and tuberculum sellae—express themselves by highly distinctive syndromes that are diagnostic in themselves.
Foster Kennedy syndrome (also known as Gowers-Paton-Kennedy syndrome, Kennedy's phenomenon or Kennedy's syndrome) refers to a constellation of findings associated with tumors of the frontal lobe. [1]
The syndrome is defined as the following changes:
optic atrophy
papilledema in the opposite eye
Central scotoma (loss of vision in the middle of the visual fields) in the same eye
anosmia (loss of smell) on the same side
This syndrome is due to optic nerve compression, olfactory nerve compression, and increased intracranial pressure (ICP) secondary to a mass (such as meningioma or plasmacytoma, usually a olfactory groove meningioma).[4][5] There are other symptoms present in some cases such as nausea and vomiting, memory loss and emotional lability
(i.e. frontal lobe signs).[5]

10. Sites & Presentations of Meningioma
Foramen magnum
Paraparesis, sphincteric troubles, tongue atrophy associated with fasciculation
Cerebello-pontine angle
Decreased hearing with possible facial weakness and facial numbness
Spinal cord
Localized spinal pain, Brown-Sequard (hemispinal cord) syndrome
Cavernous sinus
Multiple cranial nerve deficits (II, III, IV, V, VI), leading to decreased vision and diplopia with associated facial numbness
Subfrontal
Change in mentation, apathy or disinhibited behavior, urinary incontinence
Suprasellar
Hormonal Failure, Bitemporal Hemiapnosia, Optic Atrophy

11. Patient Correlation
NING, PWEDE PO PAINSERT NG SALIENT FEATURES DITO… 
67-year old woman
lifelong history of severe migraine
associated with vomiting and visual disturbances, presented with a sudden change in the severity and location of the headache and four episodes of apparent transient global amnesia with headache
urinary incontinence during one of the attacks
Fecal incontinent of during another attack
intellectual skills and memory had deteriorated dramatically in recent weeks
slowly developed spastic weakness of both legs
walked past her sons as if she did not recognize them, sat down at a different table and kept asking whether she paid for her meal

12. Focal seizures are often an early sign of meningiomas that overlie the cerebrum. The parasagittal frontoparietal meningioma may cause a slowly progressive spastic weakness or numbness of one leg and later of both legs, and incontinence in the late stages.
The sylvian tumors are manifest by a variety of motor, sensory, and aphasic disturbances in accord with their location, and by seizures

13. Human Homonculus

19. Parasagittal/Falcotentorial Meningioma
Patient Correlation
MENINGIOMA
Parasagittal/Falcotentorial Meningioma