The symptoms of CSF Leaks (cerebrospinal fluid leaks) mimic those symptoms of POTS. However, a CSF leak is a more serious medical condition requiring prompt diagnosis and treatment. CSF Leaks can occur anywhere along the length of the brain-spinal cord pathway. CSF (cerebrospinal fluid) constantly produced in the brain circulates around and bathes the brain and spinal cord serving to cushion and protect these critical nervous organs. A continuous thick sac-like membrane called dura covers the brain and spinal cord and tightly encloses the cerebrospinal fluid space.
When there is a tear or hole in the protective dura sac, CSF leaks or escapes from the dura sac. Leakage of the CSF via a defect results in decreased volume of cerebrospinal fluid that bathes and sustains the brain and spinal cord. In individuals with low CSF volume and loss of brain buoyancy due to the leak, standing results in the brain descending within the skull. Sinking of the brain to a smaller region of the skull causes compression on the lower brain and pressure-related symptoms that can mimic POTS.
The most common symptoms shared by patients with both POTS and CSF Leak include headaches, nausea, dizziness, visual disturbances, fatigue and “brain fog.” Both patients generally have worsening of symptoms when sitting or standing upright, but the severity of the disability may vary from mild to incapacitating. Characteristically, CSF Leak headaches and associated symptoms come on more quickly upon assuming an upright posture, but that is not always true. Additional symptoms more unique to CSF Leaks include pain face, neck, arms and shoulder regions, facial and arm numbness, changes in smell, taste and hearing and difficulty with balance during walking. Recognition and diagnosis of patients with CSF Leaks, similar to recognition and diagnosis of those with POTS, is difficult and often delayed. Low awareness of both illnesses contributes to the lack of their recognition for months or years.
Most commonly, CSF Leaks occur following head, neck or spinal surgery, falls, sports injuries or motor vehicle accidents. Those leaks related to trauma at the base of the skull are often associated with leakage of clear CSF from the nose or ear. These patients report drainage from the nose, salty or metallic taste in mouth, or sense of drainage down back of throat or ear. Leakage of CSF from other locations along the brain-spinal cord pathway occur “silently,” and require extensive imaging procedures to localize. Those leaks associated with lumbar punctures (spinal taps) and spinal anesthesia may be associated with a temporary headache, but usually close spontaneously.
More recently, spontaneous cases of CSF Leak occurring without an apparent precipitating event or following an insignificant activity like straining, bending or lifting have been recognized among patients with connective tissue disorders. Patients with inherited Ehlers-Danlos syndrome and Marfan syndrome manifest defective and weakened connective tissues generally, and are prone to have co-existing POTS. Thus, in addition to increased repetitive stress injuries, joint pains, dislocations and arthritis, a spontaneous tear of the spinal dura sac with a leak can occasionally occur. In fact, patients with POTS or POTS-like symptoms and weakened connective tissues are those most frequently discovered to have spontaneous tears of the dura and CSF leakage. Those with a CSF dura defect are at greater risk to acquire a life-threatening infection of the brain, meningitis.
If you have POTS symptoms and additionally have symptoms suggestive of a CSF Leak, it would be best to schedule an appointment with a neurologist as early as feasible for an evaluation.