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56 2 Diseases Mainly Affecting the Brain and its Coverings

Intracranial Hypertension and Brain Tumors


Definition:
An elevation of intracranial pressure (intracranial hypertension) may be
caused by various types of intracranial process, particularly brain tumors.
In addition to the focal signs produced by the underlying process itself, in-
tracranial hypertension manifests itself initially by headache, then by pro-
gressive impairment of consciousness and vegetative signs such as vomit-
ing, bradycardia and arterial hypertension. The objective findings include
papilledema, oculomotor palsy, pupillary abnormalities, disturbances of
respiration and other vital functions, and, finally, coma, when the elevated
intracranial pressure has led to herniation through the tentorial notch or
the foramen magnum (p. 223).

Signs of Intracranial orrhages, coronary hypoperfusion,


Hypertension and Pseudotumor paresthesias in the hands and feet,
Cerebri headache, and cerebral edema. Im-
mediate descent and treatment for
The main signs of intracranial hyper- intracranial hypertension (see below)
tension are listed in Table 2.10. are essential for recovery.

| Altitude Sickness
Too rapid ascent may be associated
with pulmonary edema, retinal hem-

Table 2.10 Clinical manifestations of intracranial hypertension

Subjective Headache (diffuse and persistent, most severe in the morning), vo-
miting (fasting, projectile), apathy
Signs of im- Confusion, respiratory disturbance, bradycardia, hypertension, cere-
pending bellar fits (opisthotonus and extensor spasms of arms and legs), di-
herniation lated pupils
Ocular findings Papilledema (may appear within hours), enlarged blind spot, at-
tacks of amblyopia, oculomotor palsy, occasionally abducens palsy
Skull radiograph Increased digitate markings, enlarged sella turcica with demineral-
ized dorsum sellae, diastasis of sutures in children
CT/MRI Slit ventricles (when elevation of ICP is due to cerebral edema), pe-
riventricular signal change, possible causative lesion (e.g., tumor,
hemorrhage)
EEG Diffusely abnormal, nonspecific
Lumbar Contraindicated when a dangerous elevation of ICP is suspected!
puncture Pressure over 200 mmH2O; may be normal, however, if CSF flow is
blocked at the occipitocervical or spinal level

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Table 2.11 Causes of intracranial hypertension

Category Specific entities Clinical features Remarks

Intracranial mass Brain tumor, subdural hema- Focal neurological and neuropsychologi-
toma, intracerebral hematoma cal deficits, headache
Infection Encephalitis, meningitis Fever, meningism E.g., neurobrucellosis,
syphilis
Traumatic brain injury Contusion, brain edema, intrace- Progression of brain edema, focal
rebral hematoma seizures
Impairment of Intraventricular tumors, aque- Headache (possibly ictal), vomiting; in Intermittent elevation of
cerebrospinal fluid ductal stenosis, malresorptive malresorptive hydrocephalus spasticity cerebrospinal fluid pres-
resorption hydrocephalus of legs, urinary incontinence, psycho- sure, prior subarachnoid

Intracranial Hypertension and Brain Tumors


organic syndrome hemorrhage or meningitis
Elevation of cerebro- E.g. in polyradiculitis, spinal Lumbar puncture yields cerebrospinal
spinal fluid protein tumors (esp. schwannoma) fluid with elevated protein
Toxic processes Lead poisoning, insecticide Psycho-organic syndrome, anemia, lead
poisoning line (on gums)
Iatrogenic Steroids, oral contraceptives,
tetracycline
Altitude sickness On rapid ascent Headache, pulmonary edema, retinal Descend immediately!
hemorrhage, angina pectoris
Pseudotumor cerebri Usually affects obese young women; A diagnosis of exclusion;
slit ventricles, often papilledema see Table 2.12
Empty sella syndrome CT shows an apparently empty sella May be associated with
turcica containing air visual disturbances

57
58 2 Diseases Mainly Affecting the Brain and its Coverings

| Pseudotumor Cerebri (Benign syphilis and vitamin A deficiency


Intracranial Hypertension) (246a).
Cases of intracranial hypertension Spontaneous recovery is the rule, and
that are not due to any of the entities recurrence is rare. 8 % of these pa-
listed above or to any other discern- tients suffer permanent visual im-
ible organic intracranial process are pairment as a consequence of papill-
designated as pseudotumor cerebri or edema; the risk is particularly high in
benign intracranial hypertension. patients concomitantly suffering
This diagnosis of exclusion is most from arterial hypertension. Gradual
commonly made in obese young shrinkage of the blind spot on visual
women. The neurological examina- field testing is an excellent indicator
tion and neuroimaging studies are of a positive response to treatment.
normal except for papilledema, an
enlarged blind spot, and (occasion- Treatment
ally) very narrow cerebral ventricles Serial therapeutic lumbar punc-
(slit ventricles). The cerebrospinal tures. If cerebral edema is present,
fluid is under elevated pressure but of high-dose dexamethasone or pred-
normal composition. The clinical fea- nisone should be given intrave-
tures are summarized in Table 2.12. nously at first, and then orally.
Causative factors are found in only Mannitol or furosemide, 40 mg i.v.
about three-quarters of cases of intra- b.i.d. or t.i.d., may also be given. A
cranial hypertension not associated commonly used treatment is with
with an intracranial space-occupying the carbonic anhydrase inhibitor
lesion (see above). Approximately acetazolamide, 250750 mg/day.
one-quarter are associated with otitis Obese patients should be put on a
media. Other causes include neuro- reducing diet.

Table 2.12 Characteristics of pseudotu-


| Empty Sella Syndrome
mor cerebri The subarachnoid space may extend
into the sella turcica in the presence
Usually diffuse headache or absence of intracranial hyperten-
Young women sion when the diaphragma sellae in-
adequately separates the intrasellar
Often obese and suprasellar compartments. CT re-
Relatively common in pregnancy veals a seemingly empty sella with
Hounsfield values approximating
Vomiting (moderately common) those of air. In cases of pseudotumor
Dizziness and tinnitus (common) cerebri, an empty sella is associated
with a higher frequency of visual
Nystagmus (moderately common) disturbances.
Papilledema (common)
| Brain Tumors
Enlarged blind spot (97c, 221b, 520a, 851a)
Slit ventricles on CT Epidemiology
One in 10,00020,000 people will de-
Elevated cerebrospinal fluid pressure
velop a brain tumor. Perhaps an addi-

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Intracranial Hypertension and Brain Tumors 59

tional one-quarter of this number | General Clinical Manifestations


harbor an asymptomatic cerebral me- of Brain Tumors
tastasis of a malignant tumor else- General Characteristics
where in the body. Primary brain tu- The general characteristics of brain
mors are much more common among tumors are listed below and summa-
psychiatric inpatients (prevalence rized in Table 2.13.
1 %) than in the general population. In > The symptoms and signs are
an epidemiologic study performed at steadily progressive. (N.b., so may
the Mayo Clinic over a period of be those of inflammatory and
40 years, the overall incidence of (more rarely) vascular processes,
brain tumors was 19.1 per 100,000 while a sudden hemorrhage into a
persons per year (778a). tumor may produce sudden, apo-
plectiform manifestations.)
Causes > Headache is common (steady, dif-
Brain tumors are thought to result fuse, often both day and night with
from the interaction of a nonlocalized diminution over the course of the
humoral factor with a local factor re- day); it may be an early symptom
lated to embryonic histogenesis, in ca. one-third of cases.
which is responsible for the typical > Signs of intracranial hypertension
locations of various particular types may be present (see Table 2.10).
of tumor, and for the overall in- > Mental changes may include irrita-
creased occurrence of brain tumors in bility, fatigability, memory impair-
the vicinity of the neural groove. Ge- ment, and general and focal
netic factors also play a role; thus, the psycho-organic syndromes.
loss of tumor suppressor genes > Epileptic seizures are more com-
through mutation seems to play a monly generalized than focal, and
role in the generation of glioblastoma are the first symptom in ca. one-
multiforme. Trauma contributes to fourth of all cases.
the generation of brain tumors ex-
ceedingly rarely, if at all.

Table 2.13 General characteristics of brain tumors

Symptoms and signs: Course Objective findings

Headache (early symptom in one- Steady progression Focal neurologic deficits


third) (exception: acute
onset due to hemor-
rhage into tumor)
General signs of intracranial hy- Neuropsychological and
pertension (vomiting, bradycardia psychopathological abnor-
not always present) malities
Epileptic seizures (initial symptom Cranial nerve palsies
in one-fourth) (sometimes)
Neuropsychological changes papilledema (common)
(apathy, irritability, memory impair-
ment)

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60 2 Diseases Mainly Affecting the Brain and its Coverings

Focal signs and symptoms. These shows an elevation of protein and cell
usually appear sooner or later in the count, particularly when the tumor
course of the illness, accompanied by abuts the ependymal or pial surface.
the general manifestations described Acoustic neuromas are characteristi-
above. They may provide a clue to the cally accompanied by an elevation of
localization of the tumor. cerebrospinal fluid protein. Tumor
cells are sometimes noted on cytolog-
Falsely localizing symptoms and ical examination of the cellular sedi-
signs. These may result from shifting ment.
of intracranial structures due to mass
effect. Plain skull roentgenogram. Plain
| Diagnostic Evaluation skull roentgenograms may reveal evi-
dence of intracranial hypertension:
Imaging Studies
increase of the digitate markings, en-
MRI and CT. MRI and CT are essential larged sella turcica, demineralized
procedures in the diagnostic evalua- dorsum sellae (note the distinction
tion of brain tumors. An MRI should between this finding and that of an
be performed when the CT is negative intrasellar tumor!), diastasis of the
despite a high degree of clinical sus- sutures in young children, or, in pos-
picion (e.g., of an infiltrative, low- terior fossa tumors, an increased ver-
grade astrocytoma), or when the clin- tical height of the posterior fossa
ical findings suggest a lesion in the with thinning of the occipital bone.
posterior fossa. It should never be for- The pineal calcification or falcine cal-
gotten that a negative CT does not cification (if present) may be dis-
rule out a brain tumor, particularly an placed to the side opposite the tumor.
infiltrative, low-grade glioma. Thus, it The skull may be locally sclerotic
may be necessary to repeat an ini- (meningioma) or destroyed (metasta-
tially negative study, particularly sis). Meningiomas may also be associ-
when the symptoms and signs are ated with abnormally deep vascular
progressive. grooves, reflecting the meningeal
blood supply to the tumor. Some tu-
Arteriography. This technique has
mors may be calcified (meningioma,
vastly declined in importance since
oligodendroglioma, craniopharyngi-
the introduction of CT and MRI, but
oma, choroid plexus papilloma, li-
may still be required as a comple-
poma of the corpus callosum), but
mentary preoperative study in some
this finding is not pathognomonic for
cases. In yet other cases, a decision to
tumor, as various kinds of inflamma-
remove the tumor surgically or to ad-
tion may also be calcified (e.g., tuber-
minister radiation therapy can only
culoma). Large intracranial aneu-
be made after a diagnostic stereotac-
rysms may display sickle-shaped cal-
tic biopsy.
cifications.
EEG. EEG no longer plays a role in the
diagnostic evaluation of brain tu- ECG. Potentially misleading ECG ab-
mors. normalities (QT prolongation, sinus
tachy- or bradycardia, pathological U
Cerebrospinal fluid examination. The wave, and ST elevation or depression)
cerebrospinal fluid is normal or else are found in association with 40 %

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Intracranial Hypertension and Brain Tumors 61

of tumors involving the temporal lobe tumor suppressor genes primarily af-
(529). fect protein phosphorylation and
phosphatase activity.
| Individual Types of Brain
Tumor Grade IV astrocytoma (glioblastoma
The most important types of brain tu- multiforme). Grade IV astrocytomas
mor and their frequency in a neuro- grow very rapidly and are highly ma-
surgical patient base are listed in lignant. Histologically, they are char-
Table 2.14. acterized by necrosis and vascular
proliferation. They are the most com-
| Astrocytic Tumors (Gliomas) mon primary brain tumor, appearing
The loss of tumor suppressor genes is mainly in the fifth and sixth decades
a contributing factor in the genera- of life, and they grow by infiltrating
tion of astrocytic tumors (e.g., LOH 10 into the brain substance. They usually
in glioblastoma). Mutations of the arise in a cerebral hemisphere but
may cross the corpus callosum to in-
Table 2.14 Frequency of different types
volve both sides (butterfly glioma)
of brain tumors in a group of neurosurgical
patients or grow downward into the basal
ganglia (Fig. 2.9). They account for
Tumor Percent approximately 90 % of all cerebral gli-
omas in adults. The patients generally
Gliomas:
come to medical attention because of
> Glioblastoma multiforme 20 neurologic deficits within weeks or,
(= astrocytoma, grade IV) at most, a few months of their first
> Astrocytoma, grades IIII 10 symptom. Apart from general tumor
symptoms and signs, focal deficits
> Ependymoma 6 such as hemiparesis and dysphasia
> Medulloblastoma 4 are usually present. Even after gross
total neurosurgical resection of the
> Oligodendroglioma 5 tumor, patients generally die of tu-
Meningioma 15 mor recurrence within a few months
to, at most, 2 years. A combination of
Pituitary adenoma 7 surgery, radiotherapy, and chemo-
Schwannoma (= neurinoma) 7 therapy improves the prognosis only
slightly. Wafers containing the cyto-
Metastasis 6 static agent BCNU bound to a poly-
Craniopharyngioma, dermoid, 4 mer are sometimes surgically im-
epidermoid, teratoma planted in the tumor cavity when a
recurrent tumor is resected, although
Angioma 4 this has been found to prolong sur-
Sarcoma 4 vival by no more than a few weeks
(136). Temozolomide has also been
Unclassifiable 5 recommended recently for chemo-
Other, rarer tumors (pinealoma, 3 therapy of recurrent tumors. The only
chondroma, granuloma, etc.) beneficial prognostic factor for a
grade IV astrocytoma is a younger age
Total 100
of onset of the disease.

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62 2 Diseases Mainly Affecting the Brain and its Coverings

a b
Fig. 2.9a, b Grade IV astrocytoma (glioblastoma multiforme). The T1- and T2-
weighted spin-echo images (a and b, respectively) reveal a polycystic tumor surrounded by
marked edema. The peripheral portion of the tumor is strongly contrast-enhancing (a).

Grade IIII astrocytoma (972a). These circumscribed fibrillary astrocytomas


astrocytomas of the cerebrum arise that have been macroscopically to-
most commonly in the fourth decade tally resected may not recur till years
of life. They generally grow slowly afterward (792a), and permanent sur-
and are sometimes well circum- gical cures are occasionally seen. In
scribed, but often infiltrate into the many cases, however, the tumor
white matter of the frontal and tem- eventually transforms itself into a
poral lobes. Pilocytic astrocytoma is a glioblastoma. Sometimes the tumor
subtype of grade I astrocytoma, while appears to be multifocal, with a
astrocytomas of grades I and II are highly heterogeneous degree of ma-
sometimes termed low-grade astro- lignity; in such cases, one may speak
cytomas; grade II tumors differ from of gliomatosis of the brain.
grade I tumors in the degree of cellu-
lar abnormality. An increased mitotic Cerebellar astrocytoma (pilocytic as-
rate is found in grade III tumors (so- trocytoma of the cerebellum). These
called anaplastic astrocytoma). Well- tumors have a much better prognosis
differentiated tumors may reside in than supratentorial astrocytomas.
the cerebral white matter for years They usually arise between the ages
while they slowly grow and produce of 5 and 15 and account for
progressive clinical manifestations. 25 % of all brain tumors of childhood
The most common of these are cogni- and adolescence. They are well-
tive changes, slowly progressive circumscribed, often cystic tumors
hemiparesis, ataxia, papilledema, that usually reside in the cerebellar
headache, and epileptic seizures. The hemispheres but may also be found
tumor is often initially detectable in the vermis or pons. They give rise
only on MRI, and not on CT. Well- to slowly progressive cerebellar signs

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Intracranial Hypertension and Brain Tumors 63

such as ataxia, dysequilibrium, nys- yields a 10-year survival rate of nearly


tagmus, and sometimes intracranial 70 %.
hypertension as an early manifesta-
tion. Neurosurgical resection may be Medulloblastoma. This malignant tu-
difficult because of the location of the mor of childhood and adolescence ac-
tumor, but a total resection, if counts for 20 % of brain tumors in this
achieved, can be curative. age group. It is found in the inferior
portion of the vermis in nine out of 10
Ependymoma. These tumors, too, cases but may also arise in the cere-
generally affect children and adoles- bellar hemispheres or the pons. It
cents, and only rarely adults. They grows by infiltration and often fills
arise from ependymal cells that lie the fourth ventricle, thereby ob-
abnormally deep within the tissue structing cerebrospinal fluid flow and
substance, rather than at the ependy- sending drop metastases caudally
mal surface. The arrangement of tu- along the cerebrospinal fluid pathway
mor cells into perivascular pseudoro- into the spine. These may lead to clin-
settes is the histologic hallmark of ical findings relating to the spinal
this tumor. It is found in the vicinity cord or cauda equina. In general, me-
of the ventricular system, more often dulloblastomas cause symptoms and
below the tentorium than above it; signs similar to those of cerebellar as-
the commonest site is the area trocytoma (see above). Much less
around the fourth ventricle. Intrame- commonly, a medulloblastoma may
dullary ependymomas are most com- appear in adulthood, practically al-
monly found in the conus medullaris ways in the posterior fossa. Even after
of the spinal cord (cf. Fig. 3.4e, f). appropriate treatment, these tumors
Ependymomas of the posterior fossa usually recur and go on to metasta-
produce focal cerebellar signs as well size to other locations in the central
as obstructive hydrocephalus, which nervous system and other organs
often dominates the clinical picture. (756). Macroscopically radical resec-
Unusual (persistent) headache in a tion combined with radiation ther-
child should arouse suspicion of an apy, to which this tumor has a high
ependymoma. Such tumors also rate of response, still cannot prevent
manifest themselves on occasion by recurrences, which frequently appear
focal signs of the pons or medulla, within months or years, in both older
e.g., hemifacial spasm (p. 678) (780a). and younger patients. In one study,
They are relatively benign, and the 58 patients with a mean age of
patient may survive many years after 17 years underwent surgical resection
the initial resection; the older the followed by radiation therapy (331).
child at first manifestation of the tu- The rates of survival and of
mor, the better the prognosis. Radical recurrence-free survival were 50 %/
resection may be impossible if the tu- 46 % at 5 years, and 32 %/32 % at
mor involves the floor of the fourth 10 years. Positive prognostic factors
ventricle. Surgery should always be included radical resection, radiation
followed by radiation therapy, which dose above 50 Gy, and radiation of the
should encompass not only the brain, entire neuraxis. Further chemother-
but also the entire spinal cord. The apy for the treatment of recurrence
combination of surgery and radiation yielded a 2-year survival rate of 46 %,

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64 2 Diseases Mainly Affecting the Brain and its Coverings

while the survival rate after tumor re- is comparable to that of supratento-
currence without further chemother- rial astrocytomas of grades IIII. Oli-
apy was zero. godendrogliomas are responsive to
PCV chemotherapy, which is recom-
Oligodendroglioma. These tumors mended as an adjuvant treatment.
most commonly appear between the A study of 81 patients revealed a me-
ages of 35 and 45. They grow by dis- dian survival time of 7.9 years for
placement or infiltration of brain tis- those who were treated with a com-
sue in the cerebral hemispheres or bination of surgery and local radia-
basal ganglia or, particularly in youn- tion therapy at a dose of at least 50
ger patients, in the thalamus. More Gy. 31 % of these patients survived
than half of all oligodendrogliomas 10 years or more (867).
are located in the frontal lobes. They
grow very slowly, and, in the pre-CT Brainstem glioma. The different his-
era, there was usually an interval of tological types of glioma occurring in
several years between the appear- the brainstem may be considered to-
ance of the first symptom and the di- gether because of the typical clinical
agnosis. Epileptic seizures occur in features associated with this location.
70 % of patients and are the initial They generally arise in the first two
manifestation in 50 %. Focal neuro- decades of life and cause progressive
logic deficits develop over the ensu- symptoms and signs through involve-
ing months or years. Gross total re- ment of the pons and medulla ob-
section is almost invariably followed longata, as summarized in Table
by recurrence, but sometimes at a la- 2.15.
tency of 35 years, so that the median These tumors are often difficult to
postoperative survival time of 5 years distinguish from other brainstem tu-
mors and vascular malformations,
Table 2.15 Signs and symptoms of a brainstem encephalitis, or the initial
brainstem tumor manifestations of multiple sclerosis.
Nuclear cranial nerve palsies with: Surgical resection is only rarely pos-
sible and usually incomplete. The
> Dysphagia benefit of chemotherapy is uncertain.
> Trigeminal sensory and motor defi- Ventricular shunting may be neces-
cits sary if hydrocephalus develops. The
> Peripheral facial nerve palsy
median survival time is 1 year.
Hemifacial spasm
Optic and chiasmatic glioma. These
Oculomotor disturbances tumors are found nearly exclusively
Long tract manifestations: in children and adolescents, and are
> Pyramidal tract signs twice as common in girls. Fourteen
> Hemiparesis percent of cases are associated with
> Ataxia neurofibromatosis. Their clinical
> (possibly) dissociated sensory deficit manifestations are listed in Ta-
> All of the above may be bilateral ble 2.16. The diagnosis is made by CT
or MRI, by means of which these tu-
Intracranial hypertension, if the mors can be distinguished from pitui-
aqueduct is occluded
tary tumors, intraorbital meningi-

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Intracranial Hypertension and Brain Tumors 65

oma, medial sphenoid wing meningi- Table 2.16 Symptoms of optic and chias-
oma, and the lesions of Hand- matic glioma
Schller-Christian disease. The com-
Almost exclusively in children and ad-
bination of surgery and radiation
olescents
therapy yields a long-term survival
rate of 85 % for anterior tumors and Visual disturbances
50 % for posterior tumors. Visual field defects

Hypothalamic tumors. Astrocytoma is Exophthalmos


the most common histological type. Late diencephalic manifestations:
These tumors lie in the rostral portion
of the floor of the third ventricle and > Polyuria
usually arise in the first 2 years of life. > Obesity
Their clinical hallmark is a progres-
sive emaciation that leads to ca- > Infantilism
chexia, despite normal food intake > Arousal disturbances
and otherwise normal behavior (Rus-
sell syndrome). This picture is to be
distinguished from anorexia nervosa, mas can be treated by observation
which generally strikes older girls. (301), particularly in elderly patients
Colloid cysts are also found in the (709b). Larger tumors and those
third ventricle and may lead to an ob- displaying hyperintensity on T2-
struction of cerebrospinal fluid flow weighted images are more likely to
in one or both foramina of Monro, progress (709b). In general, meningi-
and thereby to uni- or bilateral hy- omas should be neurosurgically re-
drocephalus of the lateral ventricles. sected.
They are treated neurosurgically. Although meningiomas may, in prin-
ciple, arise from the dura mater at
| Meningioma any site, they are preferentially found
This is a benign tumor that arises at a number of classic sites, which are
from the dura mater and grows listed in Table 2.17, together with
slowly over the years, compressing some of the particularities associated
the adjacent brain tissue. Malignant with tumors at each site.
transformation is rare, but there is a Figure 2.10 shows an MR image of a
statistical association with other, ma- meningioma.
lignant tumors, especially breast can- The adjacent bony structures are
cer. Meningioma is the most common thickened and demonstrate radially
intracranial tumor of mesodermal or- arranged bony spicules. Exostoses
igin and most often becomes clini- may also be present. Flat en plaque
cally evident between the ages of 40 meningiomas may develop largely
and 50. It may also be encountered as within the bone of the skull and give
an incidental finding at autopsy or on rise to a picture resembling fibrous
CT or MRI studies of the brain. In such dysplasia (287). Supratentorial me-
cases, follow-up studies have shown ningiomas in any location are fre-
highly variable annual growth rates, quently associated with epileptic sei-
ranging from 1 % to 21 %; thus, slowly zures, which may be their initial
growing, asymptomatic meningio- symptom.

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Table 2.17 Preferential locations of meningioma, and associated clinical features

66
Site Most common initial Course Special features
manifestations

2
Olfactory groove Anosmia Seizures, headache, personality The frontal branch of the

Diseases Mainly Affecting the Brain and its Coverings


change with frontal features, temporal artery may be en-
possible involvement of optic larged
nerve
Convexity Seizures Hemiparesis
Parasagittal and falx Lower extremity paresis, Seizures Rarely causes paraparesis
(sometimes) bilateral Babinski
sign
Sphenoid wing Visual disturbances Exophthalmos, hemiparesis Lateral tumors may be externally
(when medially located, adjacent evident as temporal hyperostosis
to optic nerve)
Tuberculum sellae Visual disturbances, pale optic Progressive visual field defect
disks
Cerebellopontine angle Deafness, vertigo Facial and trigeminal nerve Differential diagnosis:
deficits, brainstem compression acoustic neuroma
Foramen magnum Spastic quadriparesis, dysphagia, Lower cranial nerve deficits Diagnosis difficult, clinical find-
dysarthria ings may be misleading
Intraventricular Intermittent headaches and Progressive hydrocephalus Often found in trigone
vomiting
Intraspinal Progressive paraparesis Paraplegia
Intracranial Hypertension and Brain Tumors 67

a b

Fig. 2.10ac Meningioma. An 85-year-


old man who had had a slowly progressive
right hemiparesis for 2 years.
a, b The T1- and T2-weighted spin-echo
images (a and b, respectively) reveal a
large left frontal convexity meningi-
oma compressing the underlying brain
tissue. Abnormal signal in compressed
cortex (arrowhead); brain edema (T1-
hypointensity and T2-hyperintensity).
c Marked contrast enhancement, typical
for meningioma.

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68 2 Diseases Mainly Affecting the Brain and its Coverings

| Pituitary Adenoma | Schwannoma (Neurinoma)


Only about 10 % of these tumors are Schwannomas are benign neoplasms
associated with sellar enlargement arising from the Schwann cells. They
and other signs of an intracranial most commonly affect the eighth cra-
space-occupying lesion. Adenomas nial nerve, where they are familiarly
mainly occur in patients between the but inaccurately known as acoustic
ages of 30 and 50. Practically all cases neuromas. These usually appear in
exhibit endocrine disturbances: the fourth and fifth decades of life
> The rare growth-hormone secreting and give rise to the clinical syndrome
tumors (in older nomenclature, eo- of the tumor of the cerebellopontine
sinophilic adenomas) cause acro- angle, including progressive unilat-
megaly. eral deafness, tinnitus, dysequilib-
> Nonsecreting tumors (chromophobe rium, (later) trigeminal nerve deficits,
adenomas) cause panhypopituita- facial palsy, and possibly (in ad-
rism, with thin, wrinkled skin and vanced stages) cerebellar deficits, py-
secondary thyroid and sex hor- ramidal tract signs, and signs of intra-
mone deficiencies. cranial hypertension.
> Prolactinomas (serum prolactin Acoustic neuroma is sometimes a
G 100 ng/mL) cause galactorrhea manifestation of von Recklinghau-
and secondary amenorrhea in sens neurofibromatosis (p. 86) and is
women, and impotence in men. then often bilateral.
The syndrome of the tumor of the ce-
Larger pituitary adenomas cause vi- rebellopontine angle is more rarely
sual field defects (usually bitemporal produced by other processes, e.g.,
hemianopsia or bitemporal upper meningioma or epidermoid.
quadrantanopsia) and sellar enlarge- Acoustic neuroma is always associ-
ment, which may be balloon-like or ated with an elevation of the cerebro-
plate-like in radiological views. The spinal fluid protein concentration.
degree of improvement of visual acu- The diagnosis is established by CT or
ity and visual fields after surgical re- MRI (Fig. 2.11). Small, intracanalicular
section is highest in patients with tumors are well seen on MRI but only
milder impairment preoperatively, with difficulty on CT; they may be
shorter duration of visual symptoms, seen more easily on CT after the in-
and slower tumor progression. troduction of positive or negative
ACTH-secreting tumors (basophil ade- contrast (air) into the subarachnoid
nomas) are the responsible lesion in space, which outlines the tumor in
Cushings disease. They cause symp- the internal auditory canal. Microsur-
toms not through mass effect, but gical resection can usually be per-
rather through secondary adrenocor- formed without injury to the facial
tical hypersecretion of cortisol, which and trigeminal nerves. Stereotactic
leads to truncal obesity, hyperten- radiosurgery (Gamma Knife) is rap-
sion, osteoporosis, and hyperglyce- idly becoming the treatment of
mia as well as abdominal striae, hir- choice for smaller tumors.
sutism, and (in women) secondary
amenorrhea. Craniopharyngiomas | Brain Metastases
may arise in the pituitary fossa and Some 1015 % of cancer patients
are described below on p. 69. eventually develop clinically evident

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Intracranial Hypertension and Brain Tumors 69

tentorial location, gross total resec-


tion, and advanced age. Only about
30 % of cases are suitable for surgical
treatment (71a). In one study, of 122
patients from whom apparently soli-
tary brain metastases were removed,
only 5 % were alive 4 years later. In the
overall group of 122 patients with ap-
parently solitary brain metastases,
the longest survival was attained by
those who underwent gross total re-
section of the metastasis followed by
whole-brain radiation therapy in the
absence of other systemic evidence of
disease. The median survival time in
Fig. 2.11 Acoustic neuroma. this group was 1.3 years; 41 % were
T1-weighted spin-echo MR image after the alive at 2 years, and 21 % at 5 years
administration of intravenous contrast. Ob- (888). Whole-brain radiation therapy
serve the marked displacement of the pons
led to an improvement of symptoms
to the opposite side and the compression
of the fourth ventricle (small arrow). The
in 80 % of patients (71a).
beak-like projection of the tumor into the For carcinomatous meningitis, see p.
internal auditory canal (arrowhead) and 410; for paraneoplastic encephalopa-
the marked contrast enhancement are typ- thy, see p. 321.
ical features of acoustic neuroma.
| Tumors of Maldevelopmental
Origin
brain metastases. The commonest Craniopharyngioma. This tumor en-
brain metastases arise from carci- ters into the differential diagnosis of a
noma of the lung (in men) and breast pituitary tumor. It is diagnosed most
(in women), followed by melanoma commonly in children and adoles-
and renal cell carcinoma. In at least cents, with peak incidence in the sec-
three-quarters of cases, brain metas- ond decade of life, but older patients
tases are already multiple by the time may also present with initial symp-
of clinical presentation; this fraction toms of craniopharyngioma, particu-
is even higher for melanoma, while larly visual disturbances. Endocrine
metastatic renal cell carcinoma is disturbances are almost always pre-
more often solitary. Sometimes, a sent except in the less common cases
cancer outside the central nervous of purely suprasellar tumor. Cranio-
system may initially present as a pharyngioma is far more likely than
brain metastasis; this occurs most pituitary adenoma to extend into the
commonly in lung cancer. According diencephalon and third ventricle and
to the literature, brain metastases ac- produce the corresponding clinical
count for 420 % of all brain tumors. signs (hydrocephalus, behavioral dis-
The mean life expectancy after diag- turbances, diabetes insipidus, etc.).
nosis of one or more brain metastases Craniopharyngiomas are often calci-
is only 12 months. Positive prognos- fied and contain cholesterol. Al-
tic factors for survival include supra- though they are histologically benign,

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70 2 Diseases Mainly Affecting the Brain and its Coverings

Fig. 2.12ac Cavernoma (cavernous an-


gioma).
A 59-year-old woman with temporal lobe
epilepsy of increasing severity, despite anti-
convulsant treatment. A neuroradiological
evaluation after the first seizure (10 years
previously) was normal. This follow-up
study reveals a cavernoma combined with
anomalous venous drainage. This finding
suggests the possibility of treatment by
neurosurgical resection of the cavernoma.
a The coronal, T2-weighted spin-echo im-
age reveals a cavernoma in the left supe-
rior temporal gyrus. The hemosiderin
ring around the lesion, seen as loss of
a signal, is typical.
b The hemosiderin ring is even more
clearly seen in this axial gradient-echo
image, because of signal loss due to a
susceptibility effect.
c Venous phase angiogram. An anoma-
lous venous drainage with caput medu-
sae is seen (arrow). Anomalous venous
drainage is frequently associated with
cavernoma. The neurosurgeon need
only resect the cavernoma.

their clinical management can be


problematic, because they are often
not radically resectable for technical
reasons.

Other tumors of maldevelopmental


origin. Epidermoid and dermoid tu-
mors are derived from tissues not
normally found within the central
nervous system. Epidermoid tumors
generally present between the ages of
25 and 45 and are most often located
at the skull base and in the cerebello-
pontine angle. Dermoid tumors, on
c the other hand, are tumors of child-

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Intracranial Hypertension and Brain Tumors 71

hood and are most often located in to bleed with a frequency estimated
the parapituitary, parapontine, and at 0.7 % per year, and somewhat
orbitomaxillary regions. They often higher in women (674a, 792). The risk
present with visual disturbances of a symptomatic hemorrhage is par-
(from involvement of the optic chi- ticularly high when there has been a
asm), lower cranial nerve palsies, previous hemorrhage, but substan-
brainstem compression, seizures, and tially lower when seizures are the
(occasionally) behavioral abnormali- only presenting feature or when the
ties and signs of intracranial hyper- lesion is an incidental radiological
tension. Both of these types of tumor finding (16).
grow very slowly and compress, Tumors of the pineal region are rarely
rather than invade, the adjacent ner- derived from cells intrinsic to the pi-
vous tissue. They are only rarely neal gland (pineocytoma, pineoblas-
found in the spinal canal. Radical re- toma), and belong somewhat more
moval is associated with a good prog- commonly to the glioma series (as-
nosis. trocytoma, ependymoma), but are
Cavernomas (cavernous angiomas) most often of maldevelopmental ori-
(564, 792, 879, 905) are a variety of gin (germinoma, teratoma, choriocar-
hamartoma composed of abnormal cinoma, embryonal cell carcinoma,
blood vessels (Fig. 2.12). Pathological endodermal sinus tumor). Any of
examination reveals a well- these tumors may compress the aq-
circumscribed mass of blood vessels ueduct and cause obstructive hydro-
without intervening nervous tissue, cephalus. Physical findings include
containing foci of calcification. Caver- paresis of upward gaze (sometimes a
nomas may be found anywhere in the late sign) and wide pupils that con-
brain, brainstem, or spinal cord, but tract on convergence, but not in re-
are most commonly found subcorti- sponse to light (all of these findings
cally in the cerebral hemispheres. together constitute the Parinaud syn-
They are multiple in nearly one- drome). Germ cell tumors of the pi-
fourth of all cases (674a) and may oc- neal region occur predominantly in
cur in families (679c). Approximately young men.
two-thirds cause epileptic seizures
(188c), and 16 % cause clinically ap- | Other Brain Tumors
parent hemorrhage (674a). A caver- The following tumors are rare and
noma gene is found at the CCM 1 lo- will be mentioned only briefly.
cus on chromosome 7 (367b). The
commonest presenting manifesta- Malignant lymphoma. Malignant
tions are seizures, headache, and in- lymphoma may occur as a solitary or
tracranial hemorrhage. In CT images, multiple lesion anywhere in the cere-
they appear as hyperdense regions brum or cerebellum. These tumors
with contrast enhancement; calcifi- grow rapidly and infiltrate the sur-
cation may also be seen. MRI com- rounding tissue. They are seen more
monly reveals hemosiderin deposits commonly, but by no means exclu-
reflecting previous, often clinically si- sively, in immunocompromised per-
lent hemorrhage. The treatment con- sons, including AIDS patients. Their
sists of microsurgical removal and is prevalence is approximately five per
advisable because these lesions tend 10 million population, and they ac-

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72 2 Diseases Mainly Affecting the Brain and its Coverings

count for only about 1 % of all brain cysticercosis of the nervous system
tumors. They are clinically mani- affects up to 4 % of persons harboring
fested by general signs of intracranial the pork tapeworm Taenia solium in
hypertension, seizures, and/or focal regions in which this infection is en-
neurological and neuropsychological demic. Neurocysticercosis evokes
abnormalities. Simultaneous involve- symptoms by mass effect in approxi-
ment of the cranial nerves and eyes is mately half of affected individuals.
not uncommon. CT reveals one or
more foci of tumor in the vicinity of
Differential Diagnosis of Brain
the cerebral ventricles; these are ini-
tially hyperdense and enhance Tumors
strongly on the administration of Other intracranial processes may pro-
contrast. They are solitary in only duce clinical manifestations resem-
one-third of cases (135). The treat- bling those of brain tumor, either by
ment consists of whole-brain radia- mass effect or by other mechanisms.
tion therapy at a dose of 50 Gy and Table 2.18 provides an overview of
sometimes chemotherapy as well the differential diagnosis of brain tu-
(448). Treatment is effective, but not mors.
curative (135). Brain lymphoma only
rarely represents metastasis of extra-
cerebral disease. Treatment of Intracranial
Hypertension and Cerebral
Intraventricular tumors. These tu- Edema
mors may intermittently obstruct the
flow of cerebrospinal fluid. See the The available means of treating in-
above discussion of intraventricular tracranial hypertension and cere-
meningioma (p. 65) and colloid cyst of bral edema are most effective
the third ventricle (p. 819). when these are due to brain tumor,
but can also be used in cases of
Choroid plexus papilloma. These tu- pseudotumor cerebri (p. 58), intra-
mors are most common in the first cranial hemorrhage, and traumatic
decade of life, particularly in the first brain injury. Dexamethasone (De-
2 years. They are most commonly lo- cadron) is given at an initial dose of
cated in the fourth ventricle, grow by 4 mg every 6 hours intravenously,
compression rather than invasion, later by mouth. 50150 mg of solu-
may become calcified, give rise to ble prednisone may be given in-
drop metastases, and are accessible to stead. A 20 % solution of mannitol
radical surgical resection (706). can be given in an initial dose of
100 mL over 3060 minutes, and
Syphilitic gummata and tuberculo- thereafter up to eight times daily,
mas. These intracranial masses are as needed. Furosemide (Lasix) can
uncommon in Europe and North be given as a diuretic in a dose of
America. Suspicion of either should 40 mg intravenously up to three
prompt a search for other manifesta- times a day. If either of the latter
tions of the underlying disease. two medications are given to som-
nolent or comatose patients, a uri-
Hydatid cysts and cysticercosis. Hy-
nary catheter should be inserted.
datid cysts are quite rare lesions, but
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Mumenthaler, Neurology 2004 Thieme

Table 2.18 General differential diagnosis of brain tumors

Category Example Specific features Important for differential diagnosis

Space-occupying lesion Chronic subdural Headache (always), fluctuating mental Prior head trauma (almost always)
other than tumor hematoma changes, few neurological deficits
Brain abscess Fever, high erythrocyte sedimentation Febrile, acutely ill
rate, rapid progression
Arachnoid cyst Most common in young males, on the Skull asymmetry (common); often
left side, fronto-temporal complicated by subdural hematoma
after traumatic brain injury
Encephalitis Herpes encephalitis Involves temporal lobe Fever, CSF pleocytosis and xantho-
chromia, positive CT or MRI

Intracranial Hypertension and Brain Tumors


Brainstem encephalitis Brainstem signs, bilateral long tract Positive MRI
signs
Multiple sclerosis Progressive hemiparesis over a few days Absence of general signs of intra-
cranial hypertension; positive MRI
Vascular processes Stroke in progress Rapidly progressive hemiparesis without Vascular risk factors, possible demon-
signs of intracranial hypertension stration of carotid stenosis by Doppler
ultrasound
Intracerebral hemorrhage Headache (always), rapid progression, CT, MRI
decline in level of consciousness

73
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Mumenthaler, Neurology 2004 Thieme

Table 2.18 General differential diagnosis of brain tumors (Cont.)

74
Category Example Specific features Important for differential diagnosis

2
Arteriovenous malforma- Seizures, (possibly) progressive focal CT, MRI

Diseases Mainly Affecting the Brain and its Coverings


tion manifestations, may bleed acutely
(Giant) aneurysm Cranial nerve deficits, (rarely) hemi- CT, MRI may show sickle-shaped
paresis calcification in wall of aneurysm
Other Mills paralysis Hemiparesis slowly ascending from CT, MRI (diagnosis of exclusion)
leg to arm (years)
Alzheimers disease and In rare cases, there may only be a focal CT, MRI
Picks disease progressive deficit (e.g., dysphasia) for
several years
Pseudotumor cerebri, Manifestations of intracranial hyper- Young, obese women; CT shows
benign intracranial tension without focal deficit but possibly symmetrical narrowing of the lateral
hypertension with papilledema ventricles