Headache in cerebrospinal fluid volume depletion syndrome: a case report
a Department of Neurology, Otorhinolaryngology, Ophthalmology and Psychiatry, University of Palermo, Italy
b Department of Medical Biotechnologies and Forensic Medicine, University of Palermo, Italy
Reprint requests to: Prof. Giuseppe Salemi
Dipartimento di Neurologia, Otorinolaringoiatria,
Oculistica e Psichiatria
Accepted for publication: June 12, 2005
Cerebrospinal fluid (CSF) volume depletion syndrome is due to leakage of cerebrospinal fluid through lesions of the dural sac at the level of the cranial base or of the spine.
When past medical history is negative for recent trauma or surgery, the term spontaneous intracranial hypotension (SIH) is used. SIH is characterized clinically by orthostatic headache, neck pain, nausea, emesis, horizontal diplopia, tinnitus, plugged ear, hearing difficulties, blurring of vision, facial numbness, and upper limb radicular symptoms. In SIH, brain and cervical MR scans show a diffuse pachymeningeal gadolinium enhancement that ends at the site of CSF leakage. The application of epidural blood patches has been proposed as an effective therapy for SIH.
Here we describe a case of SIH with very unusual headache features; the patient reported a paradoxical pattern of postural headache provoked by clinostatic position. The CSF leakage was identified at the convexity of the skull and headache disappeared following treatment with fluid, analgesics and steroids.
KEY WORDS: cerebrospinal fluid volume depletion syndrome, paradoxical postural headache, spontaneous intracranial hypotension.
Cerebrospinal fluid (CSF) volume depletion syndrome is due to leakage of CSF through lesions in the dural sac at cranial base or spine level. According to the revised IHS classification (1), it can arise after lumbar puncture, because of the formation of a fistula, or spontaneously when there is no history of lumbar puncture or other causes of CSF fistula (1). CSF volume depletion syndrome is clinically characterized by orthostatic headache, neck pain, nausea, emesis, horizontal diplopia, tinnitus, plugged ear, hearing difficulties, blurring of vision, facial numbness, and upper limb radicular symptoms (2). Headache typically occurs within 15 minutes of sitting or standing and improves within 15 minutes of lying down.
When past medical history is negative for recent trauma or surgery, the term spontaneous intracranial hypotension (SIH) is used. Spontaneous intracranial hypotension (SIH) may evolve into chronic daily headache.
Overdraining CSF shunts following spine or cranial base surgery is a well-recognized cause of CSF volume depletion syndrome (3,4). Spontaneous CSF leakage largely occurs at the level of the spine and only rarely at the cranial base (5,6).
Imaging studies in the course of CSF volume depletion syndrome have led to the identification of a growing number of patients and to awareness of a broader clinical spectrum of the disorder (7-10).
Recently, the application of epidural blood patches was proposed as an effective therapy for SIH (11,12).
We describe the case of a man affected by CSF volume depletion syndrome secondary to a mild skull trauma that occurred three months before the onset of a headache with atypical features.
man was hospitalized in January 2002. Two weeks before
admission he had begun to complain of a severe frontal headache that occurred
some minutes after he assumed a recumbent position and was associated
with tinnitus, dizziness, and a painful feeling of stiff neck. The pain spontaneously resolved after several minutes as the
patient assumed the orthostatic position. The patient
had no previous history of lumbar puncture, or neurological diseases. Three months before the appearance of this headache, on standing up
suddenly, he had knocked the left frontal region of his head against the
bonnet of a car; he had sustained no wounds and there had occurred no
modification of consciousness. Neurological examination and
fundus oculi were normal. On
brain MR imaging, T1-weighted scans showed a diffuse pachymeningeal gadolinium
enhancement and the presence of two small hypointense fluid collections in the
left frontal and parietal regions, which at the parietal level, interrupted the
diffuse pachymeningeal gadolinium enhancement (Fig.s 1A and 2A, black
arrowheads). A diffuse subdural fluid accumulation, appearing as a hyperintense
signal, was present on T2-weighted scans (Fig.3A, white arrowheads). A decrease in
size of the lateral ventricles (Fig.1A,
white arrow), pituitary enlargement, and a decrease in size of the
perichiasmatic cistern could also be observed. CSF
examination in recumbent position revealed an opening pressure of
After Schaltenbrand’s first description of SIH in 1938, orthostatic headache was considered the typical symptom of CSF volume depletion syndrome (2). In recent times the use of MR imaging has made it possible to highlight other peculiarities of CSF volume depletion syndrome, such as diffuse pachymeningeal gadolinium enhancement, descent of the cerebellar tonsils, decrease in the size of the prepontine and perichiasmatic cisterns, flattening of the optic chiasm, subdural fluid collections, decrease in the size of the ventricles, enlargement of the pituitary gland, and engorgement of the cerebral venous sinuses (7-10,13). The search for these peculiar instrumental findings led to an increase in the number of patients diagnosed with CSF volume depletion syndrome and to recognition of a broader clinical and imaging spectrum of the disorder, including patients with CSF pressure consistently within normal limits (5,9), ones without abnormal pachymeningeal enhancement on MRI, and ones with documented CSF leaks, low CSF pressures, and diffuse pachymeningeal gadolinium enhancement, but no headache (14). Recently, Mokri described the case of a patient who developed a CSF leak from the site of a previous craniotomy. In this patient, the MR picture was typical of CSF volume depletion syndrome, but the headache occurred when he was lying on his back (4). Mokri subsequently described two other patients who experienced headache when lying down and presented CSF volume depletion (15). In the first patient, a paradoxical postural headache appeared a month after an episode of otitis, the MR picture was typical of CSF volume depletion syndrome, but no site of CSF leakage could be detected. A paradoxical postural headache also arose in the second patient after a cold and MR myelography following intrathecal injection of gadolinium revealed the site of the CSF leakage at T4. These reports broaden the spectrum of CSF volume depletion syndrome.
Both the clinical features and the MR scan modifications observed in our patient were similar to those described by Mokri in the subject who had undergone craniotomy (4). However, unlike that patient (4), our patient did not report a close temporal relationship between the headache and skull trauma.
that cause paradoxical postural headache in CSF volume depletion syndrome
remain controversial and speculative (15-18). They are
doubtless associated with the modification of CSF pressure that occurs when a
human being changes his position in space. In the
horizontal position, CSF lumbar, cisternal, and probably vertex pressure are
equal (approximately 60-
Paradoxical postural headache associated with a fissure at calvarial level is probably related to leakage of CSF from the cleft in recumbency, a position in which vertex pressure is positive (4). Its appearance with recumbency was seen to be delayed for several minutes, the time needed for CSF leakage to reach a level able to cause headache-inducing CSF hypovolaemia. Conversely, in an upright position, the drop in intracranial and vertex pressure would lead to cessation of the CSF leakage and after an interval, during which sufficient CSF is replaced, also to cessation of the headache.
However, this proposed mechanism cannot be applied to paradoxical postural headache associated with CSF leakage at spinal level.
It has been proposed that in the latter condition a dysfunction of autoregulation might lead, in the horizontal position, to engorgement of the cerebral venous sinuses and thus to increased cerebrovascular volume. In the upright position, a drop in intracranial and vertex pressure would reduce this engorgement and after an interval of time, cause the headache to cease (15).
Unfortunately, our patient refused radioisotope cisternography and any explanation concerning the mechanisms underlying his headache remains speculative. However, brain MR on T1-weighted scans showed, in this patient, an interruption of the diffuse pachymeningeal gadolinium enhancement at the parietal level. We can hypothesize that this interruption was due to the presence of a cleft in the arachnoid sheath. The pressure forces generated by the head trauma in the left frontal region reported in the patient’s previous history might have caused the opening of this fissure in the arachnoid sheath allowing CSF to leak through when the patient was recumbent. It is possible that, at the time of the frontal trauma, the arachnoid sheath at parietal level was only damaged, and that, over the following three months, the CSF progressively accumulated and provoked a lesion at the level of the already damaged sheath: this would explain the long interval between skull trauma and headache onset.
In conclusion, the case of our patient suggests that, with respect to the last IHS classification (1), there is a need for a more complete characterization of CSF volume depletion syndromes, and for detailed history taking, to detect previous skull trauma in chronic headache.
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