The diagnosis of intracranial hypotension due to spinal cerebrospinal fluid (CSF) leak can be challenging for physicians and other healthcare professionals. Even though the most frequent symptom is a positional headache – worse after minutes to hours upright and improved with lying flat, intracranial hypotension has many faces or presentations. Intracranial hypotension can be mistaken for a long list of other disorders. An incorrect diagnosis can lead to incorrect or even harmful testing and treatments and therefore poor outcomes for patients.
Most commonly, the headache associated with intracranial hypotension is mistaken for other causes of headache:
• migraine headache
• tension headache
• sinus headache
• cervicogenic headache (pain from neck problems)
• occipital neuralgia (pain arising from occipital nerves at back of head)
• meningitis
• thunderclap headache
Brain imaging findings are often mistaken for:
• Pituitary adenoma (tumor on pituitary gland)
• Chiari I – the cerebellar tonsils are often low-lying in intracranial hypotension, but this is NOT congenital Chiari I and is reversible with treatment of the underlying intracranial hypotension. This is often termed Pseudo-Chiari.
Clinical presentations may mimic:
• Meniere’s disease – hearing loss and vertigo can be mistaken as Meniere’s
• Postural orthostatic tachycardia syndrome (POTS)
• Parkinson’s disease – unsteady gait and tremor are not uncommon
• Frontotemporal dementia
• Anxiety or depression
In addition, many of the manifestations and complications may not be recognized as being due to intracranial hypotension. Some of these are:
• subdural hematomas (blood pressing on brain inside skull)
• trigeminal neuralgia (facial pain)
• hyperprolactinemia (high levels of the hormone prolactin)
• syringomyelia (fluid cavity inside spinal cord)
• quadriplegia (arm and leg numbness + weakness)
• stroke
• coma
• non-aneurysmal subarachnoid hemorrhage (brain bleeding not associated with an aneurysm)
• reversible cerebral vasoconstriction syndrome (RCVS – manifestations include sudden severe headache, stroke or mini-stroke, seizures, etc.)
• posterior reversible encephalopathy syndrome (PRES – manifestations include headache, confusion, seizures, visual loss)
This printable document, Spinal CSF Leak Diagnosis outlines information about the diagnosis of intracranial hypotension due to spinal CSF leak. Share this with your health care providers since greater awareness will assist more cases of correct diagnosis and treatment. Better outcomes depend on it.