A case of spontaneous intracranial hypotension in a 45-year-old male with headache, behavior changes and altered mental status

Abstract

Spontaneous intracranial hypotension is a rare disease that results from low cerebrospinal fluid (CSF) volume caused by leakage of CSF from the spine in the absence of lumbar puncture, spine surgery, or intervention. The most common presentation is the headache that is usually but not invariably orthostatic. The underlying pathology is a CSF leak resulting from dural weakness involving the nerve root sleeves, ventral dural tears associated with calcified disc herniations, or CSF venous fistula. In severe cases, neuropsychiatric symptoms and changes in mental status may develop. Some case reports also mention gait disturbances, slurred speech, and urinary incontinence. The constellation of neuropsychiatric symptoms similar to behavior variant frontotemporal dementia in the presence of "brain sag" on MRI is known as frontotemporal brain sagging syndrome, first described by Wicklund et al. (4). The disease presents a diagnostic challenge to the primary care physicians, who are the first to see these patients. Brain and spine imaging is key to diagnoses but requires a high index of suspicion, as very rarely are all classic findings of intracranial hypotension present in the same patient. Here we discuss a case of spontaneous intracranial hypotension in a 45-year-old male patient who presented with headache, drowsiness, incoherent speech, behavior symptoms, and altered mental status.

Keywords: CSF leak; Dural tear; Frontotemporal brain sagging syndrome; Orthostatic headache; Sagging midbrain; Spontaneous intracranial hypotension.

Fig. 1

(A) Sagittal CT head depicts central brain descent, slumping of the midbrain and downward displacement of the pons. (B) Sagittal T1WI demonstrates the same findings in greater detail. There is sagging of the midbrain, downward displacement of pons, prominent pituitary and retroclival subdural hemorrhage.

Fig. 2

(A) Axial T1WI depicts near complete effacement of the suprasellar cistern, ambient cisterns and partial effacement of the quadrigeminal plate cistern. There is bilateral uncal herniation. (B) Coronal FLAIR depicts hyperintense bilateral frontal subdural hematomas and uncal herniation.

Fig. 3

(A) Sagittal T1WI acquired 6 days following bilateral craniotomies to drain the subdural hematomas depicts exacerbation of the central brain descent with slumping of the midbrain, downward displacement of pons and cerebellar tonsils, loss of pons-midbrain angle. The pituitary is prominent and there is downward displacement of the optic chiasm. (B) Axial T1WI acquired post-operatively depicts bilateral uncal herniation and complete effacement of the suprasellar and quadrigeminal plate cistern. (C) Coronal FLAIR image acquired post-operatively depicts decrease in size of the left subdural hematoma and slight increase in the right subdural hematoma. (D) Axial post contrast T1WI depicts bilateral, diffuse and smooth pachymeningeal enhancement. (E) MRV of the brain depicts prominence of the dural venous sinuses, cortical and deep cerebral veins in the absence of occlusion or stenosis.

Fig. 4

(A) Sagittal STIR image of the thoracic spine depicts dorsal epidural collection spanning the thoracic spine. (B) Sagittal T2WI of the cervical spine depicts ventral epidural collection spanning from C6 to T2 and dorsal epidural collection extending from the inferior endplate of C6 to T4.

Fig. 5

(A) Axial CT image of the cervical spine post myelography depicts hyperdense ventral epidural collection. Note that the collection is hyperdense compared to the intrathecal contrast due to pooling of contrast in a confined space. (B) Axial CT image of the thoracic spine depicts a calcified disc protrusion at T3-T4 which indents the thecal sac and abuts the ventral spinal cord. (C) Sagittal CT of the thoracic spine depicts a solitary calcified disc protrusion at T3-T4. (D) Axial CT image of the thoracic spine post myelography depicts extravasation of contrast into the right retrocrural soft tissues at T10-T11. (E) Axial CT image of the thoracic spine post myelography depicts contrast extending into the extra-pleural space at the left lung apex.

Fig. 6

Sagittal CT head 2 months after repair of dural defect shows normal appearing brainstem with restoration of the pons-midbrain angle, the prepontine and suprasellar cisterns and normal sized pituitary.