SUPPLEMENTARY MATERIAL
Case 1. A 60-years-old woman presented at age 57 complaining of increased severity and frequency
of headaches. She had been suffering from headache for many years, but in the past 2 years they
had become daily, continuous, with a clear orthostatic component. An MRI obtained at another
institution was interpreted as demonstrating a malformation of posterior fossa structures. On
admission to our Institute (May 2005), additional complaints were profound weakness, difficulty
swallowing liquids, dizziness, hearing distortion and neck pain. Neurological examination
demonstrated psychomotor slowing, frontal signs, dysphagia for liquids, ataxia. MRI showed
diencephalo-mesencephalic swelling with brain sagging; descent of the iter to the aqueduct below
the incisural line was 20 mm; descent of cerebellar tonsils below Chamberlain’s line: 15 mm. No
subdural haematomas were found. Thin dural enhancement over the convexities and along the
clivus was present. Pituitary gland enlargement, consistent with hyperemia, was present. MRI of the
spine showed numerous dilated perineural cysts along the spinal roots, no CSF extradural
collections, and mild engorgement of the spinal venous plexuses. Diagnosis of SIH was made and
an epidural blood patch was performed. Improvement ensued in less than 72 hours: headache and
slowing of speech disappeared, ataxia and dysphagia greatly improved. Brain MRI abnormalities
were reduced at 2-month control and further improvement was observed at 8 months.
Case 2. A 56-year-old woman had been complaining of continuous headache exacerbated by
orthostatism, dizziness, and pain along the spine for 10 months. An MRI had allegedly
demonstrated a posterior fossa malformation. She was admitted to our Institute because nausea and
vomiting aggravated her clinical condition; headache was no longer relieved by horizontal
decubitus. On admission, she was drowsy, slow in speech and mentation, and appeared profoundly
weak. MRI showed diencephalo-mesencephalic swelling with brain sagging; descent of the iter to
the aqueduct: 22 mm; descent of cerebellar tonsils below Chamberlain’s line: 5 mm. No subdural
1
collections. On postcontrast study, no dural enhancement was present except, thin, along the clivus.
Large dural venous sinuses and enlarged pituitary gland were also seen. On spinal examination,
engorged peridural venous plexuses, no extradural CSF collections and an irregularly dilated dural
sleeve at T1-T2 on the left side were found. The patient underwent surgical ligature and covering of
the left T1 root with excellent clinical result and return to normal conditions for 3 months. Brain
MRI obtained 2 months after operation, however, did not show substantial changes. One month
after recurrence of clinical deterioration, the patient had a lumbar blood patch, followed by
immediate clinical improvement which lasted 4 months. After this period, gradual recurrence of
clinical symptoms occurred and 6 months later the patient was submitted to another blood patch
(May 2006), whose beneficial effects are still present after 6 months, in spite of no significant
improvement of the brain sagging and swelling on MRI.
Case 3. A 60-year-old woman had been suffering from headaches for years. At the end of 2004, the
headaches changed pattern, becoming nearly continuous and more severe, with a distinct orthostatic
pattern: elevation of the head caused headache in less than 15 minutes. Profound weakness,
episodes of urinary incontinence, dizziness and double vision were additional complaints. A few
months later, she was submitted to MRI and diagnosed to have SIH. On admission to our Institute
in November 2005, the patient was profoundly weak and emotionally unstable. Urinary
incontinence was present. Neurological examination showed frontal signs, left 6th nerve palsy,
ataxia, positive Romberg sign. MRI showed diencephalo-mesencephalic swelling with brain
sagging; descent of the iter to the aqueduct: 22 mm; descent of cerebellar tonsils below
Chamberlain’s line: 5 mm. No subdural collections and no obvious venous engorgement were
present. Minimal dural enhancement was found on postcontrast examination. On spinal MRI,
periradicular cysts at nearly all levels with a Christmas tree-like aspect were demonstrated. Epidural
blood patch led to a complete clinical recovery for 5 months. Worsening with headache, weakness
2
and incontinence was again relieved by repeated blood patch. Improvement was maintained at last
follow-up, 2 months after the second blood patch.
Case 4. A 42-year-old man had onset of posterior pulsating headache that rapidly became clearly
orthostatic in December 2005. His neurological examination was normal when, 3 months later, he
had an MRI that showed very thin subdural collections and diencephalo-mesencephalic swelling
with brain sagging; descent of the iter to the aqueduct: 19 mm; no descent of cerebellar tonsils
below Chamberlain’s line. Dural enhancement over the convexities and along the clivus were
present. Spinal MRI showed dilatation of venous plexuses and a thin anterior extradural CSF
collection from C6 to T3. No suspected points of CSF leakage were found. The patient, who has
been treated with bed rest, hydration and steroids with good clinical improvement, is currently in
follow-up.
Case 5. This 63-year-old woman had onset of orthostatic headache in 2001, 5 years prior to
admission at our Institute, shortly after a mild head trauma. She became depressed, then developed
mild disequilibrium, hearing changes and periods of somnolence and was submitted to a series of
examinations receiving different diagnoses. MRI examinations demonstrated posterior fossa
abnormalities with a midbrain deformity, which were interpreted as a possible midbrain tumour,
and subsequently a brainstem malformation, and a Chiari 1 malformation. In 2003, on admission to
another hospital, she was found depressed, with remarkable slowing in cognitive and motor
performances, and ataxic. On MRI, a severe brain sagging at the tentorial incisura was described,
with very thin third ventricle, elongation of the anteroposterior diameter of the midbrain with
posterior dislocation of the tectum and obliteration of the anterior mesencephalic and pontine
cisterns. The tonsils were at the level of the foramen magnum. The findings were still considered
indicative of a malformative aspect of the brainstem. In 2004, a minimal dural enhancement was
noted and diagnosis of SIH was suggested. Extensive search for a CSF leakage, including
3
radioisotope myelocisternogram, was negative. The patient improved after bed rest and hydration
and nothing else was done. The patient slowly developed parkinsonian features treated with
levodopa with some improvement. She also complained of urinary urgency with occasional
incontinence. In July 2006, the patient was again admitted to another hospital because of
reappearance of orthostatic headache, mild ataxia, dysarthria, and tremor in the right hand.
Diagnosis of malformation was again considered. The patient had some subjective and objective
improvement without treatment except for bed rest and was discharged home. In October 2006 she
presented at our Institute with the same clinical picture; MRI demonstrated severe brain sagging
with deep BS. Descent of the iter to the aqueduct below the incisural line was 19 mm. The
cerebellar tonsils were not herniated. No subdural effusions were present but large sulci and
subarachnoid spaces along the convexities could be seen. Further examinations to search for the
dural leakage have been proposed in order to proceed with adequate treatment, but so far the patient
has postponed additional investigations and treatment.
4