Spontaneous intracranial hypotension: two steroid-responsive cases
More details
Hide details
Publication date: 2018-05-22
Pol J Radiol, 2018; 83: 229-233
Spontaneous intracranial hypotension (SIH) is characterised by orthostatic headache, low cerebrospinal fluid pressure and diffuse pachymeningeal enhancement after intravenous gadolinium contrast administration. Magnetic resonance imaging (MRI) often plays a crucial role for correct diagnosis.

Case description:
We described two similar cases of SIH, whose clinical and imaging features are typical for this pathology. At MRI brain scan, both patients showed diffuse and intense pachymeningeal enhancement and moderate venous distension and epidural vein engorgement. The two patients were treated with bed rest and oral steroid therapy, with complete and long-lasting symptomatic relief.

Orthostatic nature of headache is the most indicative clinical feature suggesting SIH; contrast-enhanced MRI provides definite imaging diagnostic findings. Conservative treatment coupled to steroid therapy is often sufficient to obtain complete disappearance of symptoms.

Paldino M, Mogilner AY, Tenner MS. Intracranial hypotension syndrome: a comprehensive review. Neurosurg Focus 2003; 15: ECP2.
Jacobs MB, Wasserstein PH. Spontaneous intracranial hypotension. An uncommon and underrecognized cause of headache. West J Med 1991; 155: 178-180.
Schievink WI, Dodick DW, Mokri B, et al. diagnostic criteria for headache due to spontaneous intracranial hypotension: a perspective. Headache 2011; 51: 1442-1444.
Mokri B. Spontaneous CSF leaks: low CSF volume syndromes. Neurol Clin 2014; 32: 397-422.
Savoiardo M, Minati L, Farina L, et al. Spontaneous intracranial hypotension with deep brain swelling. Brain 2007; 130 (Pt 7): 1884-1893.
Mokri B. The Monro-Kellie hypothesis: applications in CSF volume depletion. Neurology 2001; 56: 1746-1748.
Nosik W. Intracranial hypotension secondary to lumbar nerve sleeve tear. J Am Med Assoc 1955; 157: 1110-1111.
Goto S, Ohshima T, Yamamoto T, et al. Successful steroid treatment of coma induced by severe spontaneous intracranial hypotension. Nagoya J Med Sci 2016; 78: 229-236.
Raskin NH. Lumbar puncture headache: a review. Headache 1990; 30: 197-200.
Schievink W, Meyer FB, Atkinson JLD, Mokri B. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg 1996; 84: 598-605.
Rando T, Fishman R. Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 1992; 42 (3 Pt 1): 481-487.
Rabin BM, Roychowdhury S, Meyer JR, et al. Spontaneous intracranial hypotension: spinal MR findings. AJNR Am J Neuroradiol 1998; 19: 1034-1039.
Dillon WP, Fishman RA. Some lessons learned about the diagnosis and treatment of spontaneous intracranial hypotension. AJNR Am J Neuroradiol 1998; 19: 1001-1002.
Medina JH, Abrams K, Falcone S, Bhatia RG. Spinal imaging findings in spontaneous intracranial hypotension. AJNR Am J Neuroradiol 2010; 195: 459-464.
Arnett BC. Tonsillar ectopia and headaches. Neurol Clin 2004; 22: 229-236.
Gentile S, Giudice RL, Martino PD, et al. Headache attributed to spontaneous low CSF pressure: report of three cases responsive to corticosteroids. Eur J Neurol 2004; 11: 849-851.
Hoffmann J, Goadsby PJ. Update on intracranial hypertension and hypotension. Curr Opin Neurol 2013; 26: 240-247.
Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia – evolution of a concept. Mayo Clin Proc 1999; 74: 1113-1123.
Mokri B. Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks. Headache 2013; 53: 1034-1053.
Spears RC. Low-pressure/spinal fluid leak headache. Curr Pain Headache Rep 2014; 18: 425.
Ho JM, Law H, Yuen S, Yam K. Overshunting-associated myelopathy: report of 2 cases. Neurosurg Focus 2016; 41: E16.
Howard BM, Sribnick EA, Dhall SS. Over-shunting associated myelopathy. J Clin Neurosci 2014; 21: 2242-2244.
Moayeri NN. Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension. J Neurosurgery 1998; 88: 912-918.
Schievink WI, Chu RM, Maya MM, et al. Spinal manifestations of spontaneous intracranial hypotension. J Neurosurg Spine 2013; 18: 96-101.
Antony J, Hacking C, Jeffree RL. Pachymeningeal enhancement – a comprehensive review of literature. Neurosurg Rev 2015; 38: 649-659.
Yoo M, Lee C, Kim K, et al. A case of intradural-extramedullary form of primary spinal cysticercosis misdiagnosed as an arachnoid cyst. J Korean Neurosurg Soc 2014; 55: 226-229.
Elefante A, Caranci F, Del Basso De Carro ML, et al. Paravertebral high cervical chordoma. A case report. Neuroradiol J 2013; 26: 227-232.
Jeong SK, Seong HY, Roh SW. Extra-intradural spinal meningioma: a case report. Korean J Spine 2014; 11: 202-204.
Maroldi R. Metastatic disease of the brain: extra-axial metastases (skull, dura, leptomeningeal) and tumour spread. Eur Radiol 2005; 15: 617-626.
De Divitiis O, Elefante A, De Divitiis E. Historic background of spinal disorders. World Neurosurg 2013; 79: 91-94.
Journals System - logo
Scroll to top