Symptoms

Symptoms of a spinal CSF leak: From common to rare

Cerebrospinal fluid (CSF) bathes and supports the brain and spinal cord. When the dura mater (a connective tissue that holds CSF in around the spinal cord) has a tear, the result is a loss of CSF volume, known as intracranial hypotension. This causes a loss of buoyancy of the brain that is worse when upright. Distension of intracranial veins and traction on pain-sensitive structures in the head are thought to be causes of the head pain that is usually worse with upright posture—one of the most common symptoms of spinal CSF leak.

The most common and classic symptom suggestive of a spinal CSF leak is head pain that is positional, such as a headache that is worse when upright and improved when positioned horizontally. This is also sometimes referred to as an orthostatic headache, as orthostatic means “relating to or caused by an upright posture.” It is not unusual for this head pain to become less positional over time, or for the positional aspect to resolve entirely. Occasionally, the head pain is never positional at all, and very rarely people can experience a reverse pattern, with a worse headache while recumbent. Note that not all patients with positional head pain have a spinal CSF leak and not all headaches related to spinal CSF leaks are positional.

The head pain is often located at the back of the head but can be frontal, bitemporal (both sides of the head), or all over the head. Head pain severity varies enormously, from mild to severe, and may not correlate well with findings on imaging. Many people affected by spinal CSF leak are quite disabled by their inability to be functional while upright.

Although head pain is the most common symptom, not every person with spinal CSF leak will experience it. And in fact there are a number of non-headache signs and symptoms. Recognizing the headache pattern and other symptoms is important in leading physicians to suspect the diagnosis of intracranial hypotension secondary to a spinal CSF leak.

Common Symptoms

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Headache that is worse when upright and better when horizontal (but other patterns do occur)

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Nausea and vomiting

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Neck pain or stiffness

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Change in hearing (muffled, underwater, tinnitus)

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Sense of imbalance

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Photophobia (sensitivity to light)

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Phonophobia (sensitivity to sound)

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Interscapular (between shoulder blades) pain

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Pain or numbness of arms

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Changes in cognition (“brain fog”)

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Dizziness or vertigo

Less common symptoms

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Visual changes (blurring, double vision, visual field defects)

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Facial numbness or pain

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Changes in taste

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Pain or numbness at various nerve root levels below the arms

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Fatigue

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Galactorrhea (fluid discharge from nipples)

Rare signs or complications

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Quadriplegia

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Dementia

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Parkinsonism, other movement disorders

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Ataxia (unsteady gait)

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Stupor / coma

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Cerebral venous thrombosis

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Reversible cerebral vasoconstriction syndrome (RCVS)

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Posterior reversible encephalopathy syndrome (PRES)

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Stroke

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Death

Key points about symptoms of intracranial hypotension due to spinal CSF leak:

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Not all patients with a positional headache have a spinal CSF leak

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Headache may be trivial or absent, with other signs and symptoms being more prominent

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The positional aspect of headache often lessens with time and may be absent from the onset

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The severity of symptoms and associated disability are often under-appreciated