Assistant Professor Johns Hopkins University School of Medicine Baltimore, Maryland, United States
Case Diagnosis: Spontaneous intracranial hypotension
Case Description: We present an unusual case of spontaneous intracranial hypotension (SIH) in a p</span>atient who developed a disorder of consciousness (DoC) in the inpatientrehabilitationsettingafterthoracic laminectomy and fusion for dural defect repair. Although imaging showed brain sagging and dural enhancement, the diagnosis of SIH was delayed due to the patient’s nonspecific symptomatology.He wassubsequently treated with conservative measures including Trendelenburg positioning and bed rest. His mentationultimately returned to baseline after several weeks.
Discussions: SIH is a potentially fatal neurologic condition caused by low intracranial pressure secondary to cerebral spinal fluid (CSF) volume disequilibrium. The hallmark of SIH is orthostatic headache. However, other symptoms associated with SIH may be nonspecific and variable, often leading to misdiagnosis and delayed treatment.Workup may involve CSF pressure measurementand MRI of the brain and spine.Imaging findingsinclude pachymeningeal enhancement, brain sagging, and evidence of CSF leak.Due to a dearth of evidence in the efficacy of therapeutic options, there is no first-line treatment for SIH. Conservative therapy used includes bed rest, Trendelenburg positioning, hydration, and oral caffeine. Epidural blood patch and surgery are sometimes performed to treat the underlying CSF leak.
Conclusions: SIH is potentially fatal and often misdiagnosed, which underscores the importance of studying patients with this disorder. Clinical presentations vary widely, with some patients presenting without the characteristic orthostatic headache. Chroniccases may demonstratedementia-like syndromes whileacute cases maymanifest asrapid change in mental status, as observed inour patient. AcuteSIH is a serious condition that canpresent in the inpatient rehabilitation setting and should beconsideredin patients who develop rapid mental status change and possess risk factors for CSF leak, such as a history of spinal surgery.Understanding the pathophysiology of this elusive disordermay facilitate early identification and timely treatment.