The commonly missed diagnosis of intracranial ...

The commonly missed diagnosis of intracranial hypotension Ashlee N. Ruggeri-McKinley BSN, RN, Brock C. McKinley D.D.S, OMFS PII: DOI: Reference:

S2214-7519(15)30020-7 doi: 10.1016/j.inat.2016.01.002 INAT 94

To appear in:

Interdisciplinary Neurosurgery: Advanced Techniques and Case Management

Received date: Revised date: Accepted date:

13 October 2015 5 January 2016 17 January 2016

Please cite this article as: , The commonly missed diagnosis of intracranial hypotension, Interdisciplinary Neurosurgery: Advanced Techniques and Case Management (2016), doi: 10.1016/j.inat.2016.01.002

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ACCEPTED MANUSCRIPT The commonly missed diagnosis of intracranial hypotension Ashlee N. Ruggeri-McKinley, BSN, RN, Brock C. McKinley D.D.S, OMFS

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Oakland University, School of Nursing, 2200 N. Squirrel Road, Rochester, Michigan 48309

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Corresponding author: Telephone: (313) 363-1743. Fax: (586) 922-4040. E-mail address: [email protected]

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1. Abstract

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We report a 28 year old female who presented with a subacute onset of a severe throbbing and stabbing headache after a morning spin class 9 months ago. We confirmed the diagnosis of spontaneous intracranial hypotension cause by a cerebrospinal fluid leak. The headache finally resolved after a 55ml blood patch. Affecting an estimated 5/100,000 patients, spontaneous intracranial hypotension is considered rare in medical literature. Many patients with spontaneous intracranial hypotension are incapacitated for years and even decades. The misdiagnosis of intracranial hypotension can have serious consequences and lead to unnecessary testing and treatment. Healthcare professionals need to be aware of this diagnosis when evaluating a patient with acute head pain. Considering that physical exams are usually normal, clinicians must focus on the patient history and physical. Clues in the patient interviewing process can lead to an immediate and accurate diagnosis.

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The commonly missed diagnosis of intracranial hypotension

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Ashlee N. Ruggeri-McKinley, BSN, RN, Brock C. McKinley D.D.S, OMFS

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1. Introduction

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Intracranial pressure is determined by the production, flow and absorption of cerebral spinal fluid in the brain. Low levels of cerebral spinal fluid, also known as intracranial hypotension, is a direct result of leaks located in the vertebral column or skull. [2,5]. The most common manifestation of spontaneous intracranial hypotension is a headache that often occurs within seconds to hours of a patient assuming an upright position. The postural headache is commonly relived by lying down or returning to a reverse trendeleburng position, usually within 30 minutes. The headache may be described as throbbing or non- throbbing and will most often be localized to the occipital and/ or the frontotemporal regions. Rarely, is the headache unilateral. Patients often describe the pain as an “anvil sitting on top of the head” or a “pulling sensation from the head down to the neck.” [4]. Often, the vast majority of the patients are able to recall the exact day and time the headache started. Other clinical features include but are not limited to pain/stiff feeling in the neck, photophobia, interscapular pain, tinnitus, hypacusia and nausea or vomiting. This type of headache is usually present during the afternoon or the second half of the day. [1,4]. In many cases, this physiological event is associated after a lumbar puncture. However, when intracranial hypotension is present without a previous lumbar puncture, the case is described as spontaneous [2,5]. The following case report represents a misdiagnosed patient with spontaneous intracranial hypotension secondary to multiple spinal leaks. 2. Case Report A 28 year old female presented with subacute onset of a severe throbbing and stabbing headache after a morning spin class 9 months ago. These headaches have persisted every day since. The patient describes the pain as beginning in the occipital area of the skull, bilaterally, and extending up through the neck to the front of the forehead, making a rams horn shape. The headaches have historically presented themselves in the afternoon, progressing as the day went on. However, it wasn’t unusual for the pain to begin immediately in the morning. The pain was consistently worse when standing upright. The patient admits to spending as much time as possible

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in a supine position. She only stands up to shower or to go to class. The patient reported that her headaches were relived by the consumption of caffeine or laying down in a supine position. On a numeric pain rating scale from 0-10, the pain is a 5 at it’s best and a 10 at it’s worst. She also complains of photophobia, nausea, interscapular pain, tinnitus, hypacusia and urge incontinence of urine. Neurological examination showed neck stiffness and bilateral occipital tenderness. Brain MRI showed evidence of idiopathic intracranial hypotension with descent of the cerebellar tonsils, prominent enhancement of the pituitary gland, dysmorphic appearance of the corpus callosum and patchy dural enhancement with loss of the pontomedullary angulation. Thoracic and lumbar MRI showed heavily T2weighted images with streaky areas of high signal, consistent with fluid extending through the neural foramina, likely along the nerve root sheaths, at multiple levels including T7-T8 on the left, T9-T10 bilaterally, T10-T11 bilaterally and L1-L2 on the left with the most prominent being at T10-T11 on the right. A large volume blood patch was performed on the patient. A total of 55 ml of autologous blood was injected into the epidural space with the use of fluoroscopy. The patient reported instant relief of all head pain immediately after the procedure. A two month follow up MRI of the thoracic and lumbar spine showed no evidence of CSF leak.

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3. Discussion

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We report a 28 year old female with spontaneous intracranial hypotension resulting from unknown cause. The headache finally resolved after a 55ml blood patch. Affecting an estimated 5/100,000 patients, spontaneous intracranial hypotension is considered rare in medical literature. Many patients with spontaneous intracranial hypotension are incapacitated for years and even decades. They find themselves unable to engage in any useful activity while being upright. Due to the common knowledge of this being a rare condition, these patients are often misdiagnosed. Many are left with treatment plans that are not effective and inappropriate. Sometimes, these treatments may even make the condition worse. Spontaneous intracranial hypotension, although considered a rare phenomenon, is still essential as a differential diagnosis for a patient presenting with a positional headache [3]. The patient has been to numerous different physicians and naturopathic doctors with little success. The patient had been previously diagnosed with muscle strain, exercise induced migraines, occipital neuralgia, tension headaches, temporomandibualr joint disorder, and new daily persistent headache. The patient expressed concerned multiple times throughout her medical journey that she is experiencing spontaneous intracranial hypotension, but she feels that her current and past medical professional teams have not validated her concern. The patient reports that she “knows what a migraine feels like, and this is not a migraine.” Literature suggests that the diagnosis of spontaneous intracranial hypotension is often missed and results in a delay of proper treatment. The misdiagnosis of intracranial hypotension can have serious consequences and lead to unnecessary testing and treatment. Many patients may follow through with repeated lumbar punctures, meningeal biopsy and numerous courses of drug therapy before the correct diagnosis is established. However, once the diagnosis is established, treatments provide relatively

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immediate relief and have a high success rate [3,6]. Healthcare professionals need to be aware of this diagnosis when evaluating a patient with acute head pain. Considering that physical exams are usually normal, clinicians must focus on the patient history and physical. Clues in the patient interviewing process can lead to an immediate and accurate diagnosis.

References

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[1] Mokri B. Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks. Headache 2013;53:1034-1053.

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[2] Schievink WI, Deline CR. Headache secondary to intracranial hypotension. Springer 2014;18:457.

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[3] Schievink WI. Misdiagnosis of spontaneous intracranial hypotension. JAMA 2003;60: 1713-1718.

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[4] Schievink WI. Spontaneous spinal cerebrospinal fluid leaks. Cephalalgia 2008;28:1347-1356.

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[5] Spears RC. Low-pressure/spinal fluid leak headache. Springer 2014;18:425

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[6] Zaatreh M, Finkel A. Spontaneous intracranial hypotension. Southern Medical Journal 2002;95:111342-1346.

ACCEPTED MANUSCRIPT The commonly missed diagnosis of intracranial hypotension Ashlee N. Ruggeri-McKinley, BSN, RN, Brock C. McKinley D.D.S, OMFS

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Spontaneous intracranial hypotension, although considered a rare phenomenon, is still essential as a differential diagnosis for a patient presenting with a positional headache The misdiagnosis of intracranial hypotension can have serious consequences and lead to unnecessary testing and treatment. However, once the diagnosis is established, treatments provide relatively immediate relief and have a high success rate. The most common manifestation of spontaneous intracranial hypotension is a headache that often occurs within seconds to hours of a patient assuming an upright position. The postural headache is commonly relived by lying down or returning to a reverse trendeleburng position, usually within 30 minutes. The headache may be described as throbbing or non- throbbing and will most often be localized to the occipital and/ or the frontotemporal regions. Rarely, is the headache unilateral. Patients often describe the pain as an “anvil sitting on top of the head” or a “pulling sensation from the head down to the neck.”

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1. Highlights