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7.0:
Headache associated with nonvascular intracranial disorder
7.1:
High CSF Pressure
Raskin
(1988) points out that the frequency and severity of headaches that
accompany intracranial lesions correlate poorly with the presence or
degree of intracranial hypertension (Northfield, 1938; Pickering, 1939);
the latter therefore, is unlikely to be an important mechanism of
headaches in these settings. Whether intracranial hypertension per se
can result in headache at all is controversial. Kunkle et al (1943)
found, in four subjects, that elevation of the cerebrospinal fluid
pressure to between 680 and 850 mm H2O for 2
minutes did not result in headache, and concluded that intracranial
hypertension does not produce headache. However, Fay (1940) performed a
similar experiment and found thresholds for headache production that
varied greatly among subjects. Headache is often the sole symptom of the
acute increase in intracranial pressure that occurs with shunt
malfunction in treated hydrocephalic patients (Dahlerup et al, 1985). It
may be that the rate at which the CSF pressure increases is critical to
production of headache.
The
syndrome of benign intracranial hypertension (pseudotumor cerebri) is
associated with headache in nearly all patients (Ahlskog and O’Neill,
1982; Corbett, 1983). The lateral ventricles are not enlarged in this
disorder, so traction and displacement of periventricular pain-sensitive
structures are not likely to be a mechanism of headache, as they might
be in obstructive hydrocephalus. Headache is sometimes, but
inconsistently, relieved by lumbar puncture in this disorder, despite
the restoration of CSF pressure to normal levels. Intracranial
hypertension in animal results in compensatory dilatation of pial
arteries (Forbes and Wolff, 1928), vasomotor instability, and loss of
cerebral vasomotor regulation (Langfitt et al, 1965). The magnitude and
persistence of these vasomotor responses may be important to the
mechanism of headache. Studies of cerebral hemodynamics in patients with
benign intracranial hypertension have shown that cerebral blood volume
is increased but have led to differing conclusions regarding the
impairment of autoregulation in this condition (Raichle et al, 1978;
Mathew et al, 1975). The mechanism underlying the increase intracranial
pressure appears to be an increased flow resistance in the arachnoid
villi and/or an increase in dural sinus pressure (Black and Conner,
1986; Aisenberg and Rottenberg, 1980). How this occurs remains unknown.
Many patients who have a pseudotumor cerebri may have undiscovered
venous sinus thrombosis. Many of the patients do not have arteriography
and the etiology is not discovered unless arteriography or contrast
enhanced MRI is used. I have recently seen two patients in whom original
MRI were misinterpreted as normal in patients with pseudotumor. Both
these patients had thrombosis of the transverse sinus. Both were young
overweight women, but no coagulopathy was discovered. They were
successfully treated with anticoagulation as well as measures, such as
Diamox, to reduce intracranial pressure.
In
pseudotumor cerebri additional symptoms may include spinal and radicular
pain (Bortoluzzi et al, 1982) or facial pain (Hart and Carter, 1982),
and they may occasionally be the presenting complaints. Many patients
with pseudotumor continue to complain of headache long after papilledema
and increase intracranial pressure have subsided, which suggests that
the mechanism of headache in this condition depends on other factors.
Propranolol and ergonovine treatment has helped to quell headaches for
many of these patients (Raskin, unpublished observations).
7.2:
Low CSF pressure
Lumbar
puncture headache
This is
a frequent complication that curiously occurs more often when the
cerebrospinal fluid is normal than when it shows evidence of disease.
According to Davenport (1964), up to 30% of patients develop headache
following lumbar puncture. It apparently occurs from a fall in
intracranial pressure and continues during the period when the pressure
remains low as a result of leakage of fluid through the puncture wound
in the dura. The size of the needle used, the amount of fluid withdrawn,
and multiple punctures are factors that influence the development of
headache.
The
headache, which is best described as ``bursting,'' is often extremely
severe. The pain is usually worse in the occipital region and forehead,
but it may extend into the neck and shoulders. It is completely relieved
when the patient lies down with the feet at a higher level than the
head. The headache usually lasts for only about 2 or 3 days but may
persist for as long as 2, or even 3, weeks, and rarely for many months.
Cisternal
and ventricular punctures are rarely followed by severe headaches, but
when air, anesthetic, or radiographic contrast material (pantopaque or
metrizamide) are injected into the ventricles or particularly when they
are injected into the subarachnoid space by the lumbar route, severe and
prostrating headaches may occur.
The
frequency and severity of headaches following lumbar puncture may be
reduced by using a fine gauge needle, having the patient lie prone for 4
hours after lumbar puncture, and maintaining bed rest for 24 hours after
the procedure. Lying flat in bed and drinking large quantities of
fluids, particularly those with caffeine (tea, coffee, some soft drinks)
often give significant relief (Mathew, 1978).
Lumbar
puncture headache, ordinarily disabling only for a few days, has no
serious sequelae. Occasionally, however, lumbar puncture may produce
subarachnoid bleeding from laceration of vessels of an unexpected spinal
cord arteriovenous malformation. Subarachnoid hemorrhage may also occur
from rupture of a congenital aneurysm. In other cases where there is an
intracranial mass lesion, particularly when it is situated in the
posterior fossa, lumbar puncture may result in herniation of the medulla
into the foramen magnum and thus cause sudden death of the patient.
Dr.
Frank B. Walsh (Walsh and Hoyt, 1969) observed an individual who
suffered from severe vascular hypertension but did not complain of
headache except following lumbar puncture. On three successive taps, the
spinal fluid pressure was 575, 405, and 600 mm of water.
As
emphasized by Raskin (1988), headache following lumbar puncture usually
begins within 48 hours and is dramatically positional: it begins when
the patient stands or sits upright; there is relief upon reclining or
with abdominal compression. It is believed that the mechanism is the
loss of the CSF position to the brain so that when a patient is upright
there is probably dilatation and tension based on the brain’s
anchoring structures, the pain-sensitive dural sinuses, resulting in
pain.
Headache
due to cerebrospinal fluid fistula
Low CSF
or lumbar puncture headaches usually subside spontaneously in a day or
two and do not require aggressive therapy. However, some patients are
debilitated by persisting headache and these patients are candidates for
injection of saline or blood in the epidural space. Based on the
observation that headache seems to follow traumatic, bloody lumbar
punctures less often than atraumatic spinal taps, the epidural injection
of autologous blood was begun (Gormley, 1960). It has now become the
most predictable and rapidly effective measure for stopping post-lumbar
puncture headache (Olsen, 1987).
7.3:
Intracranial infection
Such
headaches are usually severe. They occur in any of the various types of
meningitis or as a result of intracranial bleeding where the blood is in
contact with the meninges. There is associated cervical rigidity,
tenderness of the scalp, and almost always a positive Kernig's sign. The
headache itself is continuous and nonlocalized. Examination of the
spinal fluid reveals an excess of white blood cells in inflammatory
processes and either blood or xanthochromia in cases of subarachnoid
hemorrhage.
The
pain of injury or inflammation, as well as the pain provoked by
extravascular blood in the subarachnoid space, appear to be caused by
the concerted action of substances that are contained in whole blood and
are formed from inactive plasma precursors. Serotonin and the kinins are
probably the most important of these agents.
Individuals
who suffer from fever that is associated with septicemia or infectious
diseases often complain of dull, deeply situated headaches and aching of
their eyeballs. The mechanism of such pain is not understood. Sutherland
and Wolff (1940) have compared it to headaches produced by injections of
histamine. Wolff (1955) noted cerebral vasodilation in experimental
animals that had been given typhoid vaccine. He concluded that headaches
associated with acute infections, sepsis, and bacteremia are primarily
caused by the distention of intracranial arteries.
7.4:
Intracranial sarcoidosis and other noninfectious inflammatory diseases
A
diffuse sometimes severe headache may accompany chronic glanulomatous
disorders involving the intracranial structures. One of the most
frequent is sarcoidosis usually producing its effect from invasion of
the basal structures and cranial nerves. Sarcoid involvement of the
cranial nerves and visual pathways is discussed elsewhere, however, one
of the presenting features may be a diffuse headache not associated with
increased intracranial pressure.
7.5:
Headache related to intrathecal injections
Local
pain at the sight of injection, if it is through the lumbar base, is not
uncommon, however, more frequently a diffuse headache may follow
injection of agents such as methotrexate used in the treatment of
meningeal carcinomatosis. There is usually an associated nausea and it
is uncertain whether this is part of a type of vascular migraine-like
phenomenon or due to the chemotherapeutic agent.
In the
IHS classification category 7.5.1 is a direct effect of the agent with
the following diagnostic criteria:
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A.
Headache follows intrathecal injection within 4 hours. |
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B.
Headache is diffuse and present also in the recumbent position. |
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C.
Headache clears completely within 14 days. (If it persists
consider post lumbar puncture) |
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If it
is due to a presumed chemical meningitis, the category is 7.52 with the
following diagnostic criteria:
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1)
Headache follows intrathecal injection within 5 to 72 hours |
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2)
Headache is diffuse and present also in the recumbent position |
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3)
Cerebrospinal fluid pleocytosis with negative culture |
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7.6:
Intracranial neoplasm (Brain tumor headache)
It is
important that increased intracranial pressure from tumors be considered
as a cause of headache. About 75% of patients with intracranial tumors
complain of headaches, and increased intracranial pressure is frequently
associated with such lesions. The headaches that are associated with
increased intracranial pressure are variously described as ``bursting,''
``boring,'' etc. Often they are paroxysmal at the onset and rather
frequently occur at night when they may last only for an hour or two.
The patient may be able to point to the area of maximum headache. As
time passes, the headaches last longer and become associated with
vomiting that may be of the projectile type. It is interesting that the
nature of the vomiting has received more attention than it merits, since
true projectile vomiting is rare. Sneezing, vomiting, straining at
stool, bending over, etc., are likely to increase the pain which may
also be exacerbated by changes in position.
Usually
headaches have little localizing value in cases of cerebral tumor,
although there are certainly exceptions to this generalization. In cases
of dural tumor, pain is often described as being present in the skull at
a place overlying the tumor that has impinged on the bone. In some such
cases, it is possible to palpate a protrusion of the skull, and we have
observed several patients in whom a meningioma was suspected from
palpation. In malignant tumors invading the bone or the meninges, there
may be localized pain.
Intermittent
headaches are occasionally caused by intraventricular tumors, primarily
cysts. According to Dandy (1932), the best examples of such tumors are
cysts obstructing the ventricular drainage posteriorly, but similar
obstructions occur in the third and lateral ventricles. Dandy remarked
upon the proof of the ball valve action of such tumors by the high
pressure that is present in the ventricles at the time of the height of
the headache and by the absence of increased pressure when headache is
absent.
Further
proof that headache is associated with increased intracranial pressure
is seen in individuals who have been subjected to subtemporal or
suboccipital decompression since, in most cases, the decompression
bulges when the headache is severe, and it collapses when the headache
has disappeared (Dandy, 1932). However, we have observed one patient in
whom, after subtemporal decompression for presumed pseudotumor cerebri,
the headaches continued. When they were most severe, the decompression
site was quite flat or even depressed. The patient was more comfortable
when the decompression was bulging. This observation correlates with the
findings of Kunkle et al. (1943) who found that in four subjects,
elevation of intracranial pressure to between 680 and 850 mm of water
for 2 minutes did not result in headache. Fay (1940) performed a similar
experiment and found that cerebro-spinal fluid thresholds for headache
production varied greatly among individuals. Headache arising as a
result of intracranial abscess is indistinguishable from that caused by
neoplasm except that it tends to be more constant.
Northfield
(1938) suggested that it is not the increased intracranial pressure that
is responsible for the associated headache, but rather the sudden
alteration of pressure, whether it be an elevation or a lowering.
Northfield cited clinical and experimental observations to support this
concept. Wolff (1955) concluded that the headache associated with either
increased or decreased intracranial pressure results from traction upon,
or displacement of, pain-sensitive intracranial structures and is
independent of generalized intracranial pressure changes per se.
The tumor or abscess may press upon the dura overlying the brain, on the
falx, or on the tentorium. It may result in traction or pressure on
blood vessels that are pain-sensitive or the lesion may put direct
pressure upon the sensory nerves. Hydrocephalus associated with cerebral
tumor accounts for diffuse pressure that may influence widely separated
pain-sensitive structures.
Northfield's
paper concerning headache in cases of cerebral tumor is instructive, and
his findings conform fairly well to the following observations of Wolff
(1955).
(1)
Although the headache of brain tumor is often referred from a distant
intracranial source, the area of pain approximately overlies the tumor
in about one-third of all patients. (2) The presence of headache in the
absence of papilledema in patients with brain tumor is of great
localizing value. In two-thirds of the patients with such headaches, the
area of pain immediately overlies, or is near, the tumor and, when it is
unilateral, it is always on the same side as the tumor. (3) Headache is
almost always present with tumors of the posterior fossa. (4) Headache
may be absent with any of the common types of supratentorial tumor. (5)
The headache associated with tumors of the posterior fossa is almost
always over the back of the head, although it may occur elsewhere. (6)
Headache is usually the first symptom of tumors of the posterior fossa
except for growths in the cerebellopontine angle. (7) Headache is the
first symptom in one-third of supratentorial tumors. (8) The headache of
tumors of the cerebellopontine angle is frequently, and sometimes
solely, postauricular. (9) Headache due to supratentorial tumors is
rarely in the back of the head unless it is associated with papilledema.
(10) When supratentorial tumor causes headache in the back of the head,
headache in the front of the head is usually also present. (11) Headache
which is both frontal and occipital indicates extensive displacement of
the brain and has little localizing value. (12) The headache of brain
tumor is usually intermittent, but when it is continuous, its value in
localization is greatly enhanced.
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