| OCR Text |
Show ORIGINAL CONTRIBUTION Spontaneous Intracranial Hypotension Presenting as a Reversible Dorsal Midbrain Syndrome Marco Fedi, MD, Roberto Cantello, MD, Neil H. Shuey, FRACP, L. Anne Mitchell, FRACR, Cristoforo Comi, MD, Francesco Monaco, MD, and Maurizio Versino, MD presenting with a reversible dorsal midbrain syndrome and discuss the possible mechanisms underlying the neurolog-ical deficit. CASE REPORT A 47-year-old woman was admitted to the hospital with a severe bifrontal throbbing headache associated with dizziness and vomiting. Two weeks before admission, the patient had noticed that the headache was accentuated in the upright position and was partially relieved in the recumbent position. The patient suffered brief episodes of lethargy associated with gait ataxia and difficulty looking down. She had a history of breast cancer with no evidence of recurrence or metastatic disease. There were no meningeal signs or fever. Mild bilateral lid retraction was noted. Pupils were symmetrical and demonstrated light-near dissociation, with no constric-tion to light but a slight constriction when viewing a target at reading distance. Horizontal eye movements were normal, but there was loss of voluntary upgaze and downgaze pursuit and saccadic movements, with partial preservation of reflex vertical eye movements provoked by the oculocephalic maneuver. Convergence was impaired. There was no nystagmus. She did not complain of diplopia, and cover testing did not identify a misalignment. Papilledema was not detected. The remainder of the neurological examination was unremarkable. Results of serum biochemistry analysis, a full blood count, coagulation tests, and serologic tests for syphilis and HIV were negative. Electroencephalography (EEG) showed no abnormalities. Results of a brain CT scan were normal. After a prolonged period of sitting up, she developed severe headache, decreased consciousness, dystonic pos-turing of the arms, and slow conjugate horizontal ocular oscillations (20-25 amplitude and 0.5 Hz frequency). Brain MRI (Fig. 1) disclosed generalized downward displacement of the midline structures with severe flatten-ing of the midbrain and pons. Postcontrast T1 axial images demonstrated mild diffuse pachymeningeal enhancement. Diffusion MRI and MRA showed no abnormalities. Lumbar puncture and CSF pressure measurement was Abstract: A 47-year-old woman with postural head-ache, episodic stupor, and vertical gaze palsy had brain imaging findings consistent with spontaneous intracranial hypotension (SIH), including severe descent of the mesodiencephalic structures and diffuse pachymeningeal enhancement. The source of the cerebrospinal fluid leakage was a ruptured dorsal perineural cyst. Clinical symptoms improved after a targeted epidural blood patch was performed. Dorsal midbrain syndrome has not been reported previously as a manifestation of SIH. Perhaps distortion of structures in this brain region can occur in SIH as it does in obstructive hydrocephalus. (J Neuro-Ophthalmol 2008;28:289-292) Spontaneous intracranial hypotension (SIH) is a rare and probably under-recognized condition associated with diminished cerebrospinal fluid (CSF) volume and pressure (1). The most common cause of SIH is CSF leakage from a spinal meningeal diverticulum or simple dural tear, even if the source of the CSF loss remains cryptic on neuroimaging (2). The diagnosis of SIH is suggested by the presence of orthostatic headache, dizziness, and tinnitus, accompanied by distinctive neuroimaging findings (2-4) based on compensatory venous hypervolemia, including dural enhancement, subdural fluid collections, dilatation of the dural sinuses and spinal epidural plexus, and enlargement of the pituitary gland. We report an unusual case of SIH Department of Neurology (MF, NHS), Austin Health, Heidelberg, Victoria, Australia; Clinical School (MF) and Department of Radiology (LAM), The University of Melbourne, Austin Health Heidelberg, Victoria, Australia; Dipartimento di Medicina Clinica e Sperimentale (RC, CC, FM), Sezione di Neurologia, Universita` del Piemonte Orientale ‘‘A. Avogadro,'' Novara, Italy; Department of Radiology (LAM), University of Melbourne, Parkville, Victoria, Australia; and Dipartimento di Scienze Neurologiche (MV), Universita` di Pavia, Fondazione Istituto Neurologico, C. Mondino IRCCS Via Mondino, Pavia, Italy. Address correspondence to Marco Fedi, MD, Department of Neurology, Austin Health, PO Box 5555, Heidelberg 3084, Victoria, Australia; E-mail: marco.fedi@austin.org.au J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 289 J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 Fedi et al FIG. 1. Brain MRI performed at the time of findings of dorsal midbrain syndrome. A. Precontrast T1 sagittal image shows descent of the brain and cerebellar tonsils, effacement of the prepontine cistern, and an enlarged pituitary gland. B. Postcontrast T1 axial image shows abnormal pachymeningeal enhancement. C, D. Axial T2 images show compression of the pontomesencephalic junction (C) and at the tentorial incisura (D) with no intraparenchymal signal abnormality. These features are typical of spontaneous intracranial hypotension. 290 q 2008 Lippincott Williams & Wilkins Spontaneous Intracranial Hypotension and Dorsal Midbrain Syndrome J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 not performed because of concern about the safety of CSF removal in this clinical context. The patient was transferred to another inpatient setting where spine MRI and radioisotope cisternography confirmed a CSF leak at the T11-12 level. An epidural blood patch was performed. One month later, our examination documented full recovery of vertical eye movements, convergence, and pupillary constriction to light. Three months later, ortho-static headache recurred but was unassociated with eye movement abnormalities or altered consciousness. An epi-dural blood patch was repeated without relief of symptoms. A follow-up MRI performed 3 months later and not available for our review reportedly showed mild elevation of the cerebellar tonsils and better visualization of the prepontine cistern. Over a follow-up period of 7 years, the patient continued to demonstrate normal eye movements and convergence. DISCUSSION SIH is a rare condition caused by spinal CSF leakage. The resulting low CSF pressure and volume have two main pathophysiological consequences: a compensatory increased cerebral venous volume and descent of the brain due to a reduction in the supportive buoyant action of the CSF (5). The latter phenomenon tends to occur when other compensatory mechanisms have been exhausted and may explain some of the atypical patterns of clinical pre-sentation associated with SIH. These patterns include diplopia, ataxia, fluctuating conscious state, and parkin-sonism (2,6-8). In the present case, the diagnosis of SIH is supported by postural headache, typical neuroimaging findings, and the initial response to an epidural blood patch. Lumbar puncture is not necessary for establishing the diagnosis, as the opening CSF pressure can be normal and may exacerbate the symptoms (2). A reversible dorsal midbrain syndrome has not been previously associated with SIH. The classic features of this syndrome include a limitation of upgaze with rela-tively preserved vestibular and oculocephalic reflexes, convergence-retraction nystagmus on attempted upgaze, and eyelid retraction (9). The pupils may be normally reactive, although light-near dissociation has been well described (10). This patient was slightly atypical in that she had a vertical gaze palsy affecting voluntary eye move-ments in upgaze and downgaze, suggesting impairment of tegmental midbrain function. Dorsal midbrain syndrome most commonly occurs in the presence of ischemia or tumors, although it may also occur with obstructive hydrocephalus (11). In these cases, dorsal midbrain dysfunction due to raised intracranial pressure has been postulated as the mechanism, supported by patients in whom the manifestations have resolved after the increased intracranial pressure was relieved (12). The sensitivity of this region to mechanical distortion, whether caused by high or low intracranial pressure, suggests that a causal association between SIH and the dorsal midbrain syndrome is biologically plausible. Presumably, the dorsal midbrain becomes distorted as the brainstem is no longer suspended and supported in its CSF jacket. In our patient, we hypothesize that the midbrain dis-placement induced by the SIH led to stretching of structures involved in the control of vertical eye movements, includ-ing the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), the interstitial nucleus of Cajal (INC), the posterior commissure (PC), and the nucleus of the posterior commissure (nPC) (13). Vertical gaze palsy and intermittent unresponsiveness have been described in patients with lesions affecting the thalamus and midbrain in the area corresponding to the riMLF (14). We postulate that the impairment of downgaze as well as upgaze implies involvement of the riMLF. Concomitant involvement of the PC and nPC or their projections must also have occurred. We suggest that the pathophysiological mechanism for involvement of these structures in SIH is similar to that postulated for the occurrence of a dorsal midbrain syn-drome in obstructive hydrocephalus, in which an enlarge-ment of the aqueduct or third ventricle results in stretching or compressing of the PC. Altered mental status is an unusual complication of SIH (7,15). Our patient presented with recurrent stupor and areflexic pupils. Clinical and pathological studies have shown that the midbrain and pontine areas critical to consciousness in humans lie in the paramedian tegmental zone immediately ventral to the ventricular system and continue rostrally to the posterior hypothalamic area. Bilateral lesions of the ventromedial thalamus have been reported in patients with recurrent episodes of stupor (14). The reduced consciousness and ocular motility abnormalities may have been related to diencephalic and rostral midbrain compression due to severe brain descent. Based on this case, we suggest that the clinical spectrum of SIH be broadened to include a dorsal midbrain syndrome. Prompt diagnosis is essential to avoid poten-tially serious consequences. REFERENCES 1. Rando TA, Fishman RA. Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 1992;42: 481-7. 2. Fishman RA, Dillon WP. Intracranial hypotension. J Neurosurg 1997;86:165. 3. Fishman RA, Dillon WP. Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology 1993;43:609-11. 291 J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 Fedi et al 4. Mokri B, Posner JB. Spontaneous intracranial hypotension: the broadening clinical and imaging spectrum of CSF leaks. Neurology 2000;55:1771-2. 5. Cserr HF. Physiology of the choroid plexus. Physiol Rev 1971;51: 273-311. 6. Pakiam AS, Lee C, Lang AE. Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol 1999;56: 869-72. 7. Beck CE, Rizk NW, Kiger LT, et al. Intracranial hypotension presenting with severe encephalopathy: case report. J Neurosurg 1998;89:470-3. 8. Lai TH, Fuh JL, Lirng JF, et al. Subdural haematoma in patients with spontaneous intracranial hypotension. Cephalalgia 2007;27: 133-8. 9. Parinaud H. Paralysie des mouvements associe´s des yeux. Arch Neurol 1883;5:145-72. 10. Baloh RW, Furman JM, Yee RD. Dorsal midbrain syndrome: clinical and oculographic findings. Neurology 1985;35:54-60. 11. Shallat RF, Pawl RP, Jerva MJ. Significance of upward gaze palsy (Parinaud's syndrome) in hydrocephalus due to shunt malfunction. J Neurosurg 1973;38:717-21. 12. Chou SY, Digre KB. Neuro-ophthalmic complications of raised intracranial pressure, hydrocephalus, and shunt malfunction. Neuro-surg Clin N Am 1998;10:587-608. 13. Bhidayasiri R, Plant GT, Leigh RJ. A hypothetical scheme for the brainstem control of vertical gaze. Neurology 2000;54:1985-93. 14. Beversdorf DQ, Jenkyn LR, Petrowski JT 3rd, et al. Vertical gaze paralysis and intermittent unresponsiveness in a patient with a thalamomesencephalic stroke. J Neuroophthalmol 1995;15:230-5. 15. Pleasure SJ, Abosch A, Friedman J, et al. Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology 1998;50:1854-7. 292 q 2008 Lippincott Williams & Wilkins |
