J Neurol (2009) 256:10021003 DOI 10.

1007/s00415-009-5026-5

LETTER TO THE EDITORS

Spontaneous intracranial hypotension manifesting as an amnestic syndrome

Michael J. Devine Amrish Mehta Sean ORiordan

Received: 13 August 2008 / Revised: 9 November 2008 / Accepted: 14 November 2008 / Published online: 5 March 2009 Springer-Verlag 2009

Sirs, Spontaneous intracranial hypotension (SIH) typically presents with orthostatic headache, although more dramatic presentations are reported [1, 2, 4, 5, 7]. We describe a case of SIH presenting with anterograde amnesia. A 46-year-old right-handed man presented with a 10day history of short-term memory impairment. On the second evening of his illness, he was unable to remember where he had parked his car. He had improved the following morning, albeit incompletely. This diurnal variation to his symptoms persisted. A few days later he began to lose his way around his local neighbourhood. His wife needed to constantly repeat information, which he could register, but not recall. He was mildly unsteady on his feet, had recurrent hiccups and two episodes of urinary incontinence. He consumed little alcohol. For the preceding 9 months, he had complained of intermittent aural fullness and mufed hearing. For 6 months he had reported neck pain radiating to the right arm. For several months he had complained of recurrent occipital headaches, comprising a steady, continuous pain, without migrainous features, which worsened 4 weeks prior to admission. The headaches were invariably less severe in the morning, worse from early afternoon to evening, and would improve after 30 min of recumbency. There was no benet from analgesics (non-steroidal and
M. J. Devine S. ORiordan (&) Department of Neurology, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UK e-mail: sean.oriordan@imperial.nhs.uk A. Mehta Department of Neuroradiology, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UK

codeine), nor caffeine. For 1 week prior to presentation, the headaches became more continuous throughout the day. There was no clear precipitant at onset (e.g., trauma, heavy lifting, cough or constipation). On examination, he was alert but disorientated in time and place. He could not recollect events of the preceding week. Mini Mental State Examination (MMSE) score was 20/30 (impaired orientation, recall and attention). Right pupil was mildly dilated and optic discs were swollen and hyperemic. There was mild postural arm tremor and ngernose ataxia bilaterally. Plantar response was exor on the right, equivocal on the left. Tandem gait was mildly impaired. Examination was otherwise normal. There were no stigmata of connective tissue disorders. Serum biochemistry, haematology, inammatory markers and autoimmune prole were unremarkable. CT brain showed generalised cerebral swelling and shallow subdural collections, precluding lumbar puncture. Cranial MRI demonstrated appearances consistent with intracranial hypotension (Fig. 1ac). Subsequent MRI of whole spine with gadolinium was normal. He was restricted to bed rest in the Trendelenburg position, and oral dexamethasone was administered. Within 24 h, orientation, memory and attention began to improve. After several days intravenous caffeine 500 mg was also administered. Epidural blood patching was not performed because of the risk of dural puncture and the dramatic response to conservative measures. Cognition returned to normal (MMSE 30/30, Addenbrookes Cognitive Examination normal) over 5 days. Anisocoria abated and optic discs normalised. MRI at 10 days showed marked improvement. He remains well 6 months later. MRI at 3 months showed resolution of subdural collections and only minimal brainstem distortion (Fig. 1df).

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J Neurol (2009) 256:10021003 Fig. 1 At presentation, there was profound sagging of the brainstem and subsequent distortion of the midbrain (a and c) with anterior displacement and attening of the surface of the pons (c). There was also marked left and to a lesser extent right medial temporal transtentorial herniation (b). Bilateral subdural collections were present over the cerebral convexities, with associated cerebral sulcal effacement. Following prolonged bedrest, the supratentorial subdural collections and brainstem sagging completely resolved (e and f). The midbrain was no longer distorted (d), and there was only minor attening of the anterior surface of the pons (f). Only mild left medial temporal herniation was now evident (e)

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Differential diagnosis of subacute anterograde amnesia includes infective or inammatory encephalitides, neoplastic or paraneoplastic processes, Wernickes encephalopathy and seizure disorders. We diagnosed SIH on the basis of compatible history, characteristic MRI appearances and dramatic clinical and radiologic recovery following prolonged bed rest. SIH is often caused by CSF leak from a spinal dural defect; this was not localised in our case. CSF volume depletion leads to descent of the brain when upright. Traction on pain-sensitive structures and cervical nerves accounts for headache and radicular symptoms, whilst involvement of meningeal vagal branches can cause hiccups. Transmission of abnormal CSF pressure to that in the perilymph could explain altered hearing [6]. Optic disc swelling may have resulted from local venous congestion (superior ophthalmic veins were noted to be dilated on MRI). We propose that bilateral uncal herniation (due to infratentorial hypotension) was the anatomical substrate for amnesia. The cardinal manifestation of SIH is orthostatic headache. Neck pain, radicular arm pain and altered hearing are also well described [3]. However, SIH can rarely present dramatically, with stupor [5], coma [1], parkinsonism [4], frontotemporal-type dementia [2] or cerebellar haemorrhage [7].

SIH should be considered part of the differential diagnosis for anterograde amnesia, and conservative management may sufce, even in severe cases.

References
1. Evans RW, Mokri B (2002) Spontaneous intracranial hypotension resulting in coma. Headache 42:159160 2. Hong M, Shah GV, Adams KM et al (2002) Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology 58:12851287 3. Mokri B (2003) Headaches caused by decreased intracranial pressure: diagnosis and management. Curr Op Neurol 169:319 326 4. Pakiam AS, Lee C, Lang AE (1999) Intracranial hypotension with parkinsonism, ataxia and bulbar weakness. Arch Neurol 56:869 872 5. Pleasure SJ, Abosch A, Friedman J et al (1998) Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology 50:18541857 6. Schievink WI (2006) Spontaneous spinal cerebrospinal uid leaks and intracranial hypotension. JAMA 295:22862296 7. Schievink WI, Maya MM (2006) Quadriplegia and cerebellar hemorrhage in spontaneous intracranial hypotension. Neurology 66:17771778

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