Spontaneous Intracranial Hypotension: Case Study and Review of the Literature

Abstract

Spontaneous intracranial hypotension (SIH) is a pathology characterized by orthostatic headaches, diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI), and low to normal cerebrospinal fluid (CSF) pressures.We present the case of a 46-year-old male with refractory postural headaches, found to have a diffuse CSF leak throughout the cervicothoracic (C1-T12) spine. His neurological status declined rapidly to a Glasgow Coma Scale (GCS) of eight, necessitating bilateral subdural drain placement. Despite an overall brisk neurologic recovery, the patient remained unable to speak for nearly a week after the return of the remainder of his function. This raised the concern for possible cerebellar mutism.We review the multiple modalities used in this patient's treatment and explore possible explanations for the failure of initial therapy. The placement of bilateral subdural drains was a temporizing measure to treat the patient's neurologic decline, but it was likely the epidural blood patch with prolonged bedrest that hastened the patient's recovery. His speech function also returned with time and repeated therapy.

Intracranial subdural hemorrhage following closed neural tube defect repair: illustrative case

“…1,4,6,[18][19][20][21] Additionally, the downward tension on dural venous sinuses, which are pain-sensitive, can contribute to headaches. 4 Spontaneous CSF leaks that lead to intracranial pathology can be found in a certain subset of patients who have various connective tissue disorders; 11,22 however, our patient demonstrated no past medical history concerning for any of these disorders.…”

“…[1][2][3][4][5][6][7] Patients commonly present with symptoms of orthostatic headaches, neck stiffness, nausea, vomiting, altered mentation, and possible seizures. 3,[8][9][10][11][12][13] Typically, symptoms present from 1 day to 1 week after the procedure, 9,10 with 90% of headaches occurring within 3 days of the dural puncture or tear. 1,10,14 These headaches are commonly described as occurring in frontal and occipital regions.…”

Extreme Intracranial Hypotension With Brain Herniation Treated With Repeat Bolus Intrathecal Infusions

“…Intravenous caffeine or theophylline has also been employed to relieve headaches [9]. More severe cases may require an EBP, such as the one described by Podkovik et al where a patient with diffuse CSF leak throughout the cervicothoracic spine with a neurological decline was treated with bilateral subdural drain placement as a temporizing measure until definitive treatment with EBP was administered [10]. In our reported case, conservative management was largely supplanted for invasive therapy given the complicated nature of the presentation, with rapid neurologic deterioration supported by imaging.…”

Corticosteroids for spontaneous intracranial hypotension: a case-report and critical review focusing on pathophysiology and treatment

“…SIH is not as rare as previously considered [4]. Solid community-based epidemiological data are lacking [5], and the condition may be often underdiagnosed especially in local primary healthcare centers, given its relatively broad differential diagnosis [1,6].…”

“…Meningeal structural fragility could play a role [2], and minor traumatic events like weight lifting or intense coughing may precipitate the leakage [11,15]. Extradural pathologies like a congenital osseous spur or acquired degenerative disc disorder are some other potential causes [4,11]. Abnormal elastin and fibrillin leading to increased dural permeability may underlie SIH development in connective tissue disorders [2,16].…”