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30 Headache Attributed toPituitary Apoplexy
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The headache pathogenesis in an apoplexy can be multifactorial: (a) with the
vascular changes taking place in the sella, a sudden surge in the sellar pressure,
distending its meningeal walls is a possible mechanism—this hypothesis is reinforced by the fact that sellar decompression is usually able to improve this symptom, and (b) the hemorrhagic changes happening within the sella may reach the
subarachnoidal space. Both hypotheses uphold in the case presented: intraoperative
ndings consistent with elevated intrasellar pressure were seen in our patient, as
well as meningeal signs at admission and a certain amount of blood in the basal
cisterns was deemed present in the initial CT.Although transop intrasellar pressure
recordings have been reported, this method still awaits further validation [5] to
become routinely used.
30.4 Differential Diagnosis
Because pituitary apoplexy syndrome may share a commonality of symptoms and
signs with other common neurological emergencies such as subarachnoid hemorrhage, bacterial meningitis or stroke [3], this diagnosis may be delayed or missed.
Most apoplexies seem spontaneous, but causal roles have been identied in up to
40% of cases [3]. These range from the most frequent—hypertension—through a
wide array of contributing factors, including dopamine agonist therapy (bromocriptine and cabergoline) [1], estrogen treatment, radiation therapy of pituitary tumors
[1], head trauma, thrombocytopenia, lymphocytic leukemia, sickle cell anemia [4],
pregnancy, arteriography, lumbar puncture, myelography, pneumoencephalography,
recent surgery, use of anticoagulants, increased intracranial pressure, and pituitary
function tests [1, 18], to the least common—major surgery [3]; causal factors abound.
Factors implied on the pathogenesis of apoplexy include (a) the close relationship between the portal vessels and the diaphragm sellae, (b) the critical size of a
tumor inside a semi-rigid sella and (c) the tumor metabolic activity versus its vascularization. Some other factors seem to be implied in specic cases. Vascular endothelial growth factor messenger RNA may be increased, particularly in
nonfunctioning pituitary adenomas [4].
All these factors for pituitary apoplexy can be arranged into four groups: (a)
vascular ux reduction (as in surgical procedures, radiotherapy and after spinal
anesthesia), (b) acute increase in blood ow (physical activity and systemic hypertension), (c) pituitary stimulation (as in pituitary function tests), and (d) coagulation
disturbances (thrombocytopenia and anticoagulation) [4].
Considering the association of apoplexy and pituitary function tests [18], one
should remember that the thyrotropin-releasing hormone (TRH) effect in elevating
serum norepinephrine may trigger vasospasm. It can also act directly activating the
tumor cells, resulting in higher metabolic demand. Both effects being therefore
implicated in apoplectic events.
In apoplexy related to dopamine agonists therapy (bromocriptine and cabergoline) [1], the resulting apoptosis of lactotroph cells reduces metabolic demands and
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C. Martins et al.
angiogenesis is inhibited, but it is the imbalance between the two that leads to
infarctions with hemorrhage or hemorrhage only [1]. In bromocriptine long-term
usage, it is the rapid shrinkage of tumor cells and perivascular accumulation of uid
the implicated factors. The disruption of tissue textures seems to be the factor causing hemorrhage within the lesion, but the hypotension induced effect caused by this
drug has also been suggested as causal factor in the circulatory disturbances within
the capillaries of the region [18]. Cystic degeneration within the prolactinoma
which is being treated with a dopamine antagonist, particularly cabergoline, signals
previous intratumoral hemorrhage and is considered a predisposition to the development clinical pituitary apoplexy [1].
30.5 Diagnosis
Unenhanced tomogram (CT) is often the rst study performed. Although it is fairly
sensitive to demonstrate the sellar lesion, usually disclosing a variably hyperdense
mass in the sella [6] and suprasellar regions, it is not infrequent that it fails in presenting a small hemorrhage or infarction [18]; therefore, it is the combination of an
acute syndrome and a sellar tumor on CT that underscores the diagnosis of pituitary
apoplexy and treatment decisions can be made with CT in centers where MRI is not
readily available.
MRI is considered the exam of choice and can identify intrinsic hemorrhage,
necrosis and provide data on adjacent structures [6]. The ndings will depend on
the size of tumor, extent of hemorrhage/necrosis, and timing of examination,
because—like hemorrhage elsewhere in the brain—the MRI appearance depends
on the stage of hemoglobin degradation. Gadolinium IV administration discloses
areas of necrosis or infarct that—differently from normal pituitary tissue—stays
hypointense.
Endocrinologic assessment involves establishing a hormonal serum prole
(serum prolactin, testosterone/estrogen, luteinizing hormone, follicle-stimulating
hormone, free thyroxine, thyroid stimulating hormone, growth hormone, insulinlike growth factor-1 and cortisol).
Preop evaluation includes blood count, kidney and liver function, electrolytes,
glucose, and coagulation prole.
Early register of the visual elds should be obtained whenever possible but
should not postpone treatment. This rationale is based on the observation that signicant improvement has been observed in patients rendered blind by the apoplexy
if early decompression is undertaken [3]. Campimetry studies performed on the
days following surgery can be used as set-point for visual eld follow-up, because
although improvement is seen in the immediate postoperative period, it often continues for several weeks afterwards [3].
30 Headache Attributed toPituitary Apoplexy
287
30.6 Treatment
Although alleviating the headache is undoubtedly a focus in the management of
these patients, it is not the main priority:
Pituitary apoplexy is a life-threatening complication in which reaching clinical
stability, relief of the elevated intracranial pressure, and decompression of the optical apparatus takes clear precedence and should not be delayed longer than the time
to stabilize the patient. It is also true that because of the pathogenesis involved in
this type of headache (Table30.1), prioritizing the goal just presented also relieves
the headache.
It has been a rule of thumb in the eld that if the apoplectic patient has no visual
decits or is making neurological improvements, a conservative treatment may be
established. Surgical intervention, by an experienced surgical team, is required
whenever the patient presents with visual decits or deteriorates within the frame of
a previously established conservative treatment. Visual eld defects and ocular
palsy seem to benet the most from surgical treatment, but there is also a tendency
for a protective effect of surgery on visual acuity and pituitary function [19].
Intractable headaches were also listed as indications for surgery in 7.7% of pituitary
apoplexy patients [4], but are usually seconded by other indications for surgery.
A lot has been debated about the timing of surgery in apoplectic patients [3, 20].
Urgent decompressive transsphenoidal surgery [4] is the denomination given for the
surgical treatment performed within hours (in a semi-elective regimen, as reported
in our patient), on the same day or a maximum of 24hours after the ictus. It is our
experience, as well as others [4] that timing of surgery and extent of preop decits
are important factors inuencing the nal outcome of these patients, and considering headache, early surgery results in prompt control or shortening of the analgesic
requirements. A semi-elective regimen takes advantage of a harmonic structure
existing within a neurosurgical department in which—while the patient is being
stabilized and initially evaluated by the on-call neurosurgical and ICU teams—the
group with experience in sellar surgery and handling of the specic equipment is
being deployed, in a sequential fashion, to benet the patient and provide prompt
and qualied care as have happened in our case-example.
Surgery (transcranial, transsphenoidal microscopic or endoscopic) resulted in
improvement of visual function in 73–80% of cases [18, 21], but the best results are
to be expected the sooner visual decompression is performed, particularly in patients
presenting with blindness [21]. The goals of the procedure should be debulking of
the lesion and decompression of intracranial structures, while avoiding complications that may keep the patient in the ICU, as postoperative stula (and the following meningitis) or diabetes insipidus.
Transient diabetes insipidus is noted in the postoperative period in up to 16% of
patients with pituitary apoplexy [3]. In our department, besides the usual monitoring of serum indicators, urinary output and density have been carefully followed,
sometimes only to witness an interval of insufciency that is but a few hours-long
and swiftly recovered in the cases in which the pituitary gland and its stalk are
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C. Martins et al.
anatomically preserved, suggesting that in fact transient diabetes insipidus can be
much more frequent than reported in literature. However, diabetes insipidus is a
dreaded complication to be dealt with when it is permanent, taking a considerable
tool on the life of the patient, family, and on the health system.
30.7 Conclusion
Patients with pituitary apoplexy should have endocrine, biochemical assessment of
pituitary function, formal assessment of visual acuity and elds four to eight weeks
after the event. MRIs are recommended three to six months after apoplexy and
annually for ve years, then two-yearly.
Residual tumor should be followed. Tumor regression after apoplexy is a wellknown phenomenon with a clear pathophysiology but is denitely not a done deal.
The fate of such a tumor after apoplexy lies on its molecular signature, size of residue, and extent of hemorrhage or infarction [8] and recurrences are also recognized.
In such cases, redo surgery or radiotherapy might be indicated [3], depending on the
type of lesion.
References
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3. Rajasekaran S, Vanderpump M, Baldeweg S, Drake W, Reddy N, Lanyon M, etal. UK guidelines for the management of pituitary apoplexy pituitary apoplexy guidelines development
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4. Gondim JA, de Albuquerque LAF, Almeida JP, Bulcao T, Gomes E, Schops M, et al.
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wneu.2017.08.131.
5. Jahangiri A, Wagner JR, Chin AT, Han SW, Tran MT, Miller LM, etal. Incidence of headache
as a presenting complaint in over 1000 patients with sellar lesions and factors predicting postoperative improvement. Clin Neurol Neurosurg. 2015;132:16–20. https://doi.org/10.1016/j.
clineuro.2015.02.006.
6. Chapman PR, Singhal A, Gaddamanugu S, Prattipati V.Neuroimaging of the pituitary gland:
practical anatomy and pathology. Radiol Clin North Am. 2020;58:1115–33. https://doi.
org/10.1016/j.rcl.2020.07.009.
7. Martinez-Perez R, Kortz MW, Carroll BW, Duran D, Neill JS, Luzardo GD, etal. Coronavirus
disease 2019 and pituitary apoplexy: a single-Center case series and review of the literature.
World Neurosurg. 2021;152:e678–87. https://doi.org/10.1016/j.wneu.2021.06.004.
8. Almeida JP, Sanchez MM, Karekezi C, Warsi N, Fernández-Gajardo R, Panwar J, etal. Pituitary
apoplexy: results of surgical and conservative management clinical series and review of the
literature. World Neurosurg. 2019;130:e988–99. https://doi.org/10.1016/j.wneu.2019.07.055.
https://doi.org/10.1111/j.1365- 2265.2010
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9. Oyesiku NM, Tindall GT. Pituitary apoplexy: syndromes and surgical management. Clin
Neurol Neurosurg. 1997;99:S243–4.
10. Rosso M, Ramaswamy S, Sucharew H, Vagal A, Anziska Y, Levine SR.Isolated third cranial
nerve palsy in pituitary apoplexy: case report and systematic review. J Stroke Cerebrovasc Dis.
2021:30. https://doi.org/10.1016/j.jstrokecerebrovasdis.2021.105969.
11. Campero A, Martins C, Yasuda A, Rhoton AL Jr. Microsurgical anatomy of the diaphragma
sellae and its role in directing the pattern of growth of pituitary adenomas. Neurosurgery.
2008;62 https://doi.org/10.1227/01.neu.0000317321.79106.37.
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16. Zaidi HA, Cote DJ, Castlen JP, Burke WT, Liu YH, Smith TR, etal. Time course of resolution of hyperprolactinemia after Transsphenoidal surgery among patients presenting with
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wneu.2016.09.066.
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or incidental ndings? Endocr Abstr. 2017; https://doi.org/10.1530/endoabs.50.p301.
18. Masago A, Ueda Y, Umemura S.Pituitary apoplexy after pituitary function test: a report oftwo
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19. Tu M, Lu Q, Zhu P, Zheng W. Surgical versus non-surgical treatment for pituitary apoplexy: a systematic review and meta-analysis. J Neurol Sci. 2016;370:258–62. https://doi.
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20. Sahyouni R, Goshtasbi K, Choi E, Mahboubi H, Le R, Khahera AS, etal. Vision outcomes in
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21. Lubbe DE, Mankahla N, Carrara H, Semple P.Surgical intervention for binocular blindness in
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Part V
Headache Attributed to Non-vascular
Intracranial Disorder
Chapter 31
Headache Attributed toLow Cerebrospinal
Fluid (CSF) Pressure
MarcioNattanPortesSouza andDiogoGuilhermeLeãoEdelmuth
31.1 Introduction
Spontaneous intracranial hypotension (SIH) is an underdiagnosed secondary headache condition caused by spontaneous cerebrospinal uid (CSF) leakage, resulting
in a spectrum of diverse symptoms and radiological ndings. The most common
clinical manifestation is orthostatic headache, and the rst step of investigation is
the brain magnetic resonance imaging (MRI). Advances in imaging and understanding of the underlying mechanisms, including cerebrospinal uid (CSF)–venous stulas (CVF), have improved diagnostic accuracy; however, management remains
challenging, particularly in patients with complex presentations or comorbidities.
This chapter explores the pathophysiology, clinical presentation, diagnostic
approaches, and treatment strategies for SIH, grounded in a real-world case that
highlights the importance of specialized care, multidisciplinary evaluation, and targeted imaging techniques. Through this lens, we aim to provide clinicians with
practical insights and an evidence-based framework to recognize and manage this
condition effectively across a range of clinical settings.
31.2 Pathophysiology
Currently, three different etiologies of SIH are recognized: (1) CSF leak due to a
nerve root sleeve diverticulum, (2) CSF leak due to an osteophyte spur, and (3)
CSF–venous stula (CVF)(1). In all types, there is a CSF leak from the subarachnoid space to either the epidural space or the intravenous compartment. Despite the
CSF volume loss, intracranial pressure measured by lumbar puncture is usually
M. N. P. Souza (*) · D. G. L. Edelmuth
Hospital Israelita Albert Einstein, Hospital das Clinicas FMUSP, São Paulo, Brazil
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_31
293© The Author(s), under exclusive license to Springer Nature
294
M. N. P. Souza and D. G. L. Edelmuth
normal, due to compensatory mechanisms including an increase in intracranial
venous volume, which can be identied in some cases by prominent intracranial
venous engorgement [1–3]. One important concept is that skull-base CSF leaks
typically do not present with the typical symptoms of SIH or neuroimaging signs.
Therefore, in cases of SIH presentation, the target of investigation and treatment
should be at the spinal levels [4].
31.3 Case Presentation
A 72-year-old man with a history of chronic kidney disease following unilateral
nephrectomy, splenomegaly, and prior malignancies (including bladder, renal,
intestinal, and prostate cancer) presented with a sudden onset of daily headache,
predominantly orthostatic, worsening in the upright position and alleviating with
recumbency. He had no previous history of headaches. Brain MRI at the time
revealed bilateral subdural hematomas and diffuse pachymeningeal thickening,
ndings suggestive of spontaneous intracranial hypotension (SIH) (Fig.31.1).
Spine MRI did not present any longitudinal epidural collection (Fig.31.2).
After the rst week, there was spontaneous improvement in symptoms. A few
days later, following a minor motor vehicle accident, the patient experienced clinical deterioration characterized by exacerbation of headaches, postural instability,
gait disturbances, and altered level of consciousness. Brain computed tomography
(CT) showed enlargement of the subdural hematomas, requiring urgent neurosurgical drainage (Fig.31.3).
Fig. 31.1 Axial uid-attenuated inversion recovery (FLAIR) image shows diffuse pachymeningeal thickening and bilateral subdural effusions/hematomas
31 Headache Attributed toLow Cerebrospinal Fluid (CSF) Pressure
Fig. 31.2 Spine MRI sagittal T1 showing absence of longitudinal epidural collection
295
Fig. 31.3 Brain CT showing left hematoma with shift of the midline suggestive of hypertensive
hematoma
296
Fig. 31.4 Axial FLAIR
showing bilateral subdural
hematoma
M. N. P. Souza and D. G. L. Edelmuth
The postoperative course was complicated by delayed surgical site infection and
subsequent bacterial meningitis, necessitating further surgical interventions, including craniectomy and drainage repositioning. Although the headaches initially
improved after these procedures, the patient reported persistent asthenia and discrete proximal weakness in the upper limbs, and brain MRI continued to show ndings suggestive of SIH (Fig.31.4).
An epidural blood patch (EBP) was considered at that time. Still, due to the
comorbidities and the absence of an epidural collection at the rst spine image, a
second opinion was sought in a specialized center. Considering the typical presentation at the beginning of the evolution, the typical ndings of the rst Brain MRI, the
absence of an identiable epidural CSF collection and the persistence of signs of
SIH in the last Brain MRI after surgery, the possibility of a CSF–venous stula was
considered.
A rst computed tomography (CT) myelography was performed with contrast
injection in the right lateral decubitus position and did not reveal any denite cerebrospinal uid–venous stula (Fig.31.5). However, subtle linear contrast enhancement was observed near the left T10–T11 neural foramen (at the emergence of the
left T10 nerve root), with delayed washout, possibly representing veins of the intervertebral venous plexus.
After two days, a second CT myelography was performed in the left lateral decubitus position, and it revealed a CSF–venous stula at the level of T10–T11
(Fig.31.6).