- Email: [email protected]
Chronic intracranial hypotension
DNT as a cause of coma/Chronic intracranial hypotension
the volume o f the cystic mass during the period o f 1 year. The radiological findings were also atypical in that they showed a spontaneously hyperdense, partially calcified nodule w h i c h was revealed histologically to be collapsed, thickened, calcified, 'degenerated' collagen bundles, containing thick-walled vessels some o f which were also calcified. The cystic c o m p o n e n t o f the tumour which extended towards the deeper layers o f the brain could have been misinterpreted in this case as a predominantly non-cortical lesion. Furthermore, at surgery skull thinning was evident but was not obvious radiologically. Histopathology is essential in arriving at a correct diagnosis o f DNTs and enables one to exclude other tumours a m o n g the differential diagnosis, including o l i g o d e n d r o g l i o m a and gliomas w h o s e treatment and p r o g n o s e s are quite different. 1,3,4,I1,12,~4,15 Prospective studies have established the stable character o f DNTs on radiological follow-up; however, special consideration must be given to those cases with cystic components. It has been s h o w n that up to 12.5% o f DNTs may present with cysts. It is possible that, in the present case, repeated haemorrhages as a result o f vascular dissections or ruptures, repeated vascular thrombosis with infarctions, in a hamartomatous vascular region, have led to a progressively expanding haemorrhagic cyst or h a e m a t o m a resulting in mass effect. Therefore, regular radiographic controls are necessary in cases o f cystic DNTs since they m a y present with progressive morphological changes. In our opinion, surgery remains the most effective treatment for DNTs located in non-functional areas o f the brain or those with mass effect.
457
Chronic intracranial hypotension Roderick A. Mackenzie, A. K. Lethlean, R. Shnier, P. W. Blum Institute of Neurological Sciences, Prince of Wales Hospital, High Street, Randwick, NSW 2031, Australia
Summary Acute intracranial hypotension can occur following lumbar puncture or a fall, and sometimes spontaneously. Most cases resolve within weeks or months but some require surgical repair of the defect causing leakage of cerebrospinal fluid (CSF). It is conceivable that such leaks could become chronic if the defect is incompletely sealed. We report the case of a 49-year-old male who presented with a 10-month history of headache associated with a leaking thoracic extradural arachnoid cyst. After this was repaired he reported relief not only of his recent headaches but also of chronic alcohol-related headaches. A long-standing anaemia resolved and tinnitus/hyperacusis improved. It is suggested that an injury 30 years before may have initiated the leak of CSF resulting in chronic intracranial hypotension. Journal of Clinical Neuroscience (1998)5(4), 457-460© HarcourtBrace& Co. Ltd 1998
Keywords: headache, spontaneous intracranial hypotension, low CSF pressure, spinal arachnoid cyst
REFERENCES 1. Daumas-DuportC, ScheithauerBW, ChodkiewiczJP, Laws ER, VedrenneC. Dysembryoplasticneuroepithelialtumor: a surgically curable tumor of young patients with intractable partial seizures. Neurosurgery 1988; 23: 545-556. 2. KuchelmeisterK, Demirel T, Schlorer E. Dysembryoplasticneuroepithelial tumour of the cerebellum. Acta Neuropathol 1995; 89: 385. 3. Leurlg SY, Gwi E, NG HK, Fung CF, Yam KY. Dysembryoplastic neuroepithelialtumor - a tumor with small neuronal cells resembling oligoderldroglioma.Amr J Surg Pathol 1994; 18: 604-614. 4. Daumas-DuportC. Dysembryoplasticneuroepithelialtumors. Brain Pathol 1993; 3: 283-295. 5. Kirkpatrick PJ, Honavar M, Polkey CE. Control of temporal lobe epilepsy following en bloc resection of low-grade tumors. J Neurosurg 1993; 78: 1%25. 6. ValiAM, Clarke MA, Kelsey A. Dysembryoplasticneuroepithelialtumour as a potentially treatable cause of intractable epilepsy in children. Clin Radiol 1993; 47: 255-258. 7. IwanagaK, Takahashi H, Kameyama S. Dysembryoplasticneuroepithelial tumor: report of a case without typical glioneuronalelements. Acta Neuropathol 1995 ; 89: 284-289. 8. RaymorldAA, Halpin SFS, Alsanjari N. Dysembryoplasticneuroepithelial tumor: features in 16 patients. Brain 1994; 117: 461-475. 9. DuncanR, Patterson J, Hadley DM, et al. CT, MR and SPECT imaging in temporal lobe epilepsy. J Neurol Neurosurg Psychiatry 1990; 53:11-15. 10. Koeller KK, Dillon WP. Dysembryoplasticneuroepithelialtumors: MR appearance. Am J Neuroradiol 1992; 13: 1319-1325. 1l. Hirose3, ScheithauerW, Lopes M, Beatriz S, VanderlbergScott R. Dysembryoplasticneuroepithelialtumor (DNT): an immunohistochemicaland ultrastructuraI study. J Neuropathol Exp Neurol 1994; 53: 184-195. 12. Prayson R, Estes L. Dysembryoplasticneuroepithelialtumor. Am J Clin Pathol 1992; 97: 398-401. 13. Pierce GB. The cancer cell and its control by the embryo. Am J Pathol 1983; 113: 117-124. 14. Gottschalk J, Korves M, Skotzek-KonradB, Goebel S, Cervos-NavarroJ. Dysembryoplasticneuroepithelialmicro-tumor in a 75-year-oldpatient with long-standing epilepsy. Clin Neuropathol 1993; 12: 175-178. 15. Prayson R, Estes L, Morris HH. Coexistence of neoplasia and cortical dysplasia in patients presenting with seizures. Epilepsia 1993; 34: 609~615.
© Harcourt Brace & Co. Ltd 1998
Received 23 September 1996 Accepted 20 December 1996 Correspodence to: Dr R. A. Mackenzie, Tel: 61 2 9525 8344; Fax: 61 2 9524
1173
INTRODUCTION The s y n d r o m e o f acute intracranial hypotension (AIH) is n o w well known. In the days following a spinal tap 1,2 or a fall, 3 and sometimes spontaneously, ~,4-16 the patient develops severe headache in the erect position w h i c h is relieved by lying down. In most o f the 100 cases reported in the literature, the duration o f s y m p t o m s was weeks to months, but in 3 cases 15a7,18 the s y m p t o m s recurred 2.2 and 10 years later, respectively. A I H is thought in most cases to result from a leak o f cerebrospinal fluid (CSF). The underlying defect causing the C S F leak usually remains obscure. Although small tears in nerve root sleeves or spinal extradural cysts have been mentioned as the likely cause o f apparently spontaneous intracranial hypotension (SIH) mzI9 these lesions have only occasionally been demonstrated with radiology and/or isotope cisternography. 12,17,2° All reported cases resolved spontaneously, following epidural blood patching, or following surgical repair o f the defect. However, it is conceivable that such leaks could recur with incidents that cause a rise in intracranial pressure such as exertion or minor trauma. Individuals with a small leak may have an unrecognized chronic intracranial hypotension (CIH) syndrome.
CASE R E P O R T A 49-year-old male presented with a 10-month history o f periodic headaches lasting 18-24 h and relieved by lying down. A slight
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chill often preceded the headache, which was felt as a dull ache in the frontal and occipital regions, building to a severe constant headache over several hours if the upright posture was maintained. Many of the headaches occurred 8-12 h after exertion, such as a vigorous game of squash or heavy lifting. He had a longer history of headaches occurring after alcohol ingestion, partly relieved by additional fluid intake. He had had a spinal tap at 4 years of age for what was probably an episode of mumps encephalitis. At 19 years of age he had fallen heavily on his buttocks and had severe headache for at least a week. From 35 years of age he had been aware of progressive deafness and tinnitus, initially on the right and then bilaterally. At 44 years of age he suddenly became aware of a defect of central vision in his right eye 36 h after heavy exertion. Examination at that time had shown impaired colour and brightness perception in the right eye associated with a small centro-caecal scotoma. Visual evoked responses were delayed and attenuated bilaterally, more so on the right, consistent with optic neuropathy. A magnetic resonance image (MRI) brain scan was reported as normal (Fig. 1). The visual disturbance improved over 6 weeks but he continued to experience blurring of right eye vision with exertion or when he had a hot drink. At the time of his presentation with periodic headache, the neurological examination was normal apart from bilateral sensorineural deafness, moderate on the right and mild on the left. Routine haematology and biochemistry was unremarkable apart from a mild normochromic normocytic anaemia (Hb 123 G/L). A second MRI brain scan (Fig. 2) showed descent of the cerebellar tonsils below the foramen magnum and thickening of the meningeal layer; gadolinium injection resulted in marked enhancement of the meninges. Review of the initial scans showed meningeal thickening, narrowed cerebral sulci and crowding of the optic chiasm that had not been appreciated previously (Fig. 1). A spinal tap produced CSF at an opening pressure of 58 mm (normal 80-150 mm) water; total protein content was 1100 mg/L (normal 200-400 rag/L). A diagnosis of CIH was made and 20 ml of the patient's blood was injected into the lumbar epidural space. He reported immediate relief of headache and a feeling of greater well-being. This recovery continued for 3 months, but he had return of headache 24 h after lifting a hea W weight. Repeat brain MRI showed no resolution of the abnormalities and an MRI scan of the thoracic spine (Fig. 3) showed an epidural fluid collection at T l l level. The cyst was located over the dorsolateral aspect and there was erosion of the left lamina and pedicle, consistent with a long standing pressure effect. At subsequent surgery, a 2 cm multi-loculated extradural arachnoid cyst was found over the left T l l nerve root sleeve. The cyst appeared to be held open by a ligamentous attachment, and CSF was leaking through the paper-thin covering membrane. The defect was oversewn and packed with muscle. The patient reported one further episode of chill followed by headache beginning 36 h after surgery and lasting 2 days, but has had no fitrther headaches of any kind in the subsequent 12 months. He reports increased well-being and his blood count is normal (Hb 142 G/L). A repeat MRI brain scan 6 months later (Fig. 4) showed resolution of the previously described abnormalities.
DISCUSSION Although most reported cases of AIH have resolved spontaneously over weeks or months, there are reports of three patients who had recurrent symptoms for 2.2 and 9 years. One patient 15had no cause found, while the other two 17'18 had leaks from raptured spinal arachnoid diverticula. The CSF leak in our patient may have resulted from his spinal tap as a child, and may have been exacerbated or caused by the fall at 19 years. Such trauma has been
Joumal of Clinical Neuroscience (1998) 5(4)
Fig. 1 MRI Tl-weighted brain (A) midline sagittal and (B) coronal sections at presentation with optic neuropathy.
Fig. 2 MRI Tl-weighted brain sections with gadolinium at presentation with periodic headaches.
known to cause a dural root sleeve tear resulting in AIH. 3 It is quite conceivable that such a tear could result in a persistent fistula admitting CSF into the soft tissue which would later become encased by a non-absorbing wall and lead to the formation of an extradural meningeal (false) cyst. 2~a3 We postulate that in our patient this cyst wall was fragile and subject to repeated leakage over many years. The MRI brain scan changes in AIH have been well defined. 12-15,24-28There is reduction in the volume of CSF, meningeal thickening and downward displacement of the brain; meningeal enhancement occurs with gadolinium injection. Fishman and Dillon26have postulated that the meningeal changes are due to dural vasodilation secondary to decreased intracranial pressure. The inner layer of dura mater contains less collagen than the tough outer layer and the vascular spaces are thin-walled, making them more susceptible to pressure changes. Mokri et al~5 have demonstrated that meningeal biopsy in AIH is usually normal but their patient with symptoms persistent over 2 years showed leptomeningeal reaction with hyperplasia of arachnoid cap cells and an increase in connective tissue. They postulated that this proliferative reaction was triggered by chronic venous engorgement in the inner layer of dura mater adjacent to the low pressure CSF (subarachnoid) compartment. Similar changes have been described in a patient with a 6-week history of AIH 27 and in relation to overdraining ventricular shunts. 28 The MRI scan of our patient showed meningeal thickening and crowding of the optic chiasm at the time of his episode of optic neuropathy but these changes were not recognized and gadolinium was not given. However, gadolinium enhancement was clearly demonstrated when he presented with periodic headaches. Both the meningeal thickening and the gadolinium enhancement resolved after successful treatment. Horton and Fishman 14 described visual obscurations, visual field defects and horizontal diplopia in AIH and suggested that traction and compression of the 2nd and 6th cranial nerves was the likely mechanism. The acute optic neuropathy in our patient was likely to be due to compression, seen as reduced CSF volume and crowding of the optic nerves on the original MRI scans. Although visual function has recovered symptomatically, visual evoked potentials (VEPs) remain delayed and attenuated, and he continues
© Harcourt Brace & Co. Ltd 1998
Chronic intracranial hypotension
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B Fig. 3 MRI T2-weighted thoracic spinal cord (A) coronal and (B) T l l horizontal sections at time of headache recurrence.
Fig. 4 MRI Tl-weighted brain sections with gadolinium 6 months after repair of defect,
to experience Uthoff's phenomenon. In the absence of any clinical or laboratory evidence of multiple sclerosis, compression of the optic nerves is the likely mechanism for the optic neuropathy. The patient's hearing problems may or may not be related to CIH. Deafness and tinnitus have been reported after spinal tap 29,3° and it has been suggested that these result from decrease in intralabyrinthine pressure because of the anatomical communication between this compartment and the subarachnoid space. Traction or compression of the auditory nerves caused by brain displacement is also possible. Our patient had progressive sensorineural deafness for 15 years that showed some improvement after the epidural repair but is still present. The cause of the anaemia is also unclear. Normocytic normochromic anaemia can be a manifestation of chronic inflammation but there was no cellular reaction in the CSF. He did report that his periodic headaches were often preceded by a slight chill, and 'occasional fever of variable degree' has been reported as part of the syndrome of AIH. It is possible that the fever and the anaemia resulted from an immune response to leaked CSF components which do not usually get through the blood-brain barrier but there is no literature support for this view.
© Harcourt Brace & Co. Ltd 1998
Alcohol-related headaches are thought to be due to cerebral vasodilatation and are exacerbated by lowered CSF pressure. While the commonest cause for this is dehydration, it appears in our patient to have been related to his CIH, since it improved dramatically following successful treatment. It seems quite possible that CIH may be an unrecognized cause of periodic and alcohol-associated headaches in other patients. Unless the typical clinical features are specifically sought, the diagnosis may be missed. Even MRI scanning may not detect subtle changes, as demonstrated here; this is especially likely to happen if gadolinium is not given. The fact that the scan is performed in the supine position also means that brain displacement may not be appreciated. The attending clinician must have a high index of suspicion for the diagnosis and perform spinal tap for confirmation. Measurement of spinal fluid pressure should again become an essential part of the assessment of patients with headache.
REFERENCES
1. BellWE, JoyntRJ, SahsAL. Low spinalfluidpressuresyndromes.Neurology 1961; 10: 512421. 2. GassH, GoldsteinAS, RuskinR, LeopoldNA. Chronicpost-myelogram headache.ArchNeurol 1971; 25: 168-170. 3. NosikWA. Intracranialhypotensionsecondaryto lumbarnerve sleevetear. JAMA 1955; 157: 1110-1111. 4. SchlatenbrandG. Neuereanschaugenzur pathophysiologicder liquorzirkulation.ZentralblNeurochir 1938; 3: 290-300. 5. PuechR LhermitteJ, BuvatJ, KoechlinA, PerrinJ. Un cas d'hypotension intracraniennespontanee,avecconstatationsanatomiques.Rev Neurol i942; 74: 316-320. 6. HesserS. Two casesof so-calledspontaneousaliquorrhoea.ActaMed Scand Suppl 1946; 170: 758-760. 7. LindquistT, MobergE. Spontaneoushypoliquorrhoea;reportof a case. Acta Med Scand I949; 132: 556460. 8. PageF. Intracranialhypotension.Lancet 1953; 2: 6749-6953. 9. ShenkinH, FinnesonB. Clinicalsignificanceof low CSF pressure.Neurology 1958; 8: 157-163. 10. LasaterGM. Primaryintracranialhypotension.Headache I970; 10: 63~56.
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11. MarcelisJ, Silberstein SD. Spontaneouslow CSF pressure headache. Headache 1990; 30: 192-196. 12. RandoTA, Fishman RA. Spontaneousintracranial hypotension:report of two cases and review of the literature. Neurology 1992; 42: 481-487. 13. PannnlloSC, Reich JB, Krol G, Deck MDF, Posner YB.MRI changes in intracranial hypotension.Neurology 1993; 43: 919-926. 14. HortonJC, Fishman RA. Neurovisual findings in the syndrome of spontaneous intracranial hypotensionfrom dural cerebrospinalfluid leak. Ophthalmology 1994; 101: 244-251. 15. MokriB, Patisi JE, ScheithanerBW, Piepgras DG, Miller GM. Meningeal biopsy in intracranialhypotension: meningeal enhancementon MRI. Neurology 1995; 45: 1801-1807. 16. Renowden SA, Gregory R, Hyman N, Hilton-JonesD. Spontaneous intracradial hypotension.J Neurol Nenrosurg Psychiatry 1995; 59: 511-515. 17. Davenport RJ, Chataway HJS, Warlow CR Spontaneousintracranial hypotensionfrom a CSF leak in a patient with Marfan's syndrome.J Neurol Neurosurg Psychiatry 1995; 59: 516-519. 18. Schievink WI, Meyer FB, Atldnson JLD, Mokri B. Spontaneousspinal cerebrospinaifluid leaks and intracraniaihypotension.J Neurosurg 1996; 84: 598--605. 19. PaulsonGW, Klawans HL Jr. Benign orgasmic cephalgia. Headache 1994; 13: 181-187. 20. Schi.evinkWI, Reimer R, Folger WN. Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. J Neurosurg 1994; 80: 736-739. 21. Schreiber F, Haddad B. Lumbar & sacral cysts causing pain. J Neurosurg 1951; 8: 504-509. 22. TarlovIM. Spinal perineural and meningeal cysts. J Neurol Neurosurg Psychiatry 1970; 33: 833-843. 23. LakePA, Minclder J, Scanlan RL. Spinal epidural cyst: theories of pathogenesis. J Neurosurg 1974; 40: 774-778. 24. SableSG, Ramadan NM. Meningeal enhancement & low CSF pressure headache. An MRI study. Cephalgia 1991; 11: 275-26. 25. HochmanMS, Naidich TE Kobetz SA, Fernandez-MaitinA. Spontaneous intracranial hypotension with pachymeningealenhancementon MRI. Neurology 1992; 42: 1628-1630. 26. Fishman RA, Dillon WE Dural enhancementand cerebral displacement secondary to intracranialhypotension.Neurology 1993; 43: 609-611. 27. MokriB, Krueger BR, Miller GM, Piepgras DG. Meningeal gadolinium enhancementin low-pressureheadaches. J Neuroimaging 1993; 3: 11-15. 28. Good DC, Ghobrial M. Pathologic changes associated with intracranial hypotension & meningeal enhancementon MRI. Neurology 1993; 43: 2698-2700. 29. VandamLD, Dripps RD. Long-term follow-up of patients who received 10 098 spinal anaesthetics.JAMA 1965; 161: 586-591. 30. Abouleish E, de la Vega S, Blendinger t, Tic T-O. Long-term follow-up of epidural blood patch. Anesth Analg 1975; 54: 459-463.
This technique may be used for approaching intraspinal pathology in high risk patients where extensive exposure may be contraindicated. Journal of Clinical Neuroscience (1998)5(4), 460-463 © HarcourtBrace& Co. Ltd 1998
Keywords: ankylosing spondylitis, cervical spine, spinal epidural hematoma, spinal endoscopy Received 22 October 1996 Accepted 3 December 1997 Correspondence to: Edmund H. Frank, Tel: 1 503 494 8070, Fax: 1 503 494
7161
INTRODUCTION Spinal epidural hematomas (SEH) are rare, but well described, lesions. They may be spontaneous or occur following trauma. Twenty cases o f SEH occurring following trauma in patients with ankylosing spondylitis (AS) have been reported.l-12 The mortality and morbidity o f spinal trauma in patients with AS is high. In the 20 reported cases o f SEH associated with AS, nine died and only eight made a good recovery. ~ Cardiorespiratory complications accounted for 50% o f the deaths following spine trauma in such patients. Our patient suffered rapidly worsening cardiac failure immediately following his injury. Systemic anticoagulation was necessary for the investigation and m a n a g e m e n t o f his coronary disease. Systemic anticoagulation is contraindicated in the face o f a pre-existing SEH and/or an extensive laminectomy. Most surgeons advocate withholding anticoagulants for at least 2 4 4 8 h following spinal procedures. We report the use o f a malleable spinal endoscope via a limited cervical laminotomy to remove the h e m a t o m a in an effort to reduce the risk o f postoperative h e m a t o m a formation. Spinal endoscopy is being increasingly used in an effort to reduce the morbidity associated with extensive approaches to the spine. The majority o f uses reported to date involve anterior approaches for thoracic pathology.i315 This case is the first reported use o f the endoscope for a SEH. The etiology and m a n a g e m e n t o f SEH is reviewed and potential applications for this technique are discussed.
CASE R E P O R T
Endoscopic treatment of spinal epidural hematoma Timothy R. Steel FRACS,Jordi X. Kellogg MD, Todd A. Kuether MD, Jacques Favre MD, Edmund H. Frank MD Oregon Health Sciences University, Division of Neurosurgery,, L-472, 3181 SW Sam Jackson Park Road, Portland, OR 97201, USA
Summary We report the use of a spinal endoscope via a limited cervical laminotomy to evacuate a spinal epidural hematoma. The patient was a 75-year-old male with a 32-year history of ankylosing spondylitis. Following a low speed motor vehicle accident he developed a cervicothoracic epidural hematoma without an associated fracture. Despite a rapidly improving neurological state, his rapidly deteriorating cardiorespiratory state required systemic anticoagulaUon necessitating decompression of the hematoma. The hematoma was successfully removed via a limited C6 and C7 laminotomy using the endoscope and a malleable disposable aspirator. We conclude that epidural hematomas can be readily evacuated via endoscopic techniques without extensive laminectomy. Joumal of Clinical Neuroscience (1998) 5(4)
A 74-year-old male with a 32-year history o f AS was brought to our emergency room following a low speed roll-over motor vehicle accident. H e suffered no head injury or loss o f consciousness. On arrival he was unable to lie flat on the spine board due to his 40% kyphosis and he complained o f severe pain in his cervicothoracic region, but was neurologically intact. Within 15 rain o f admission he complained o f rapidly developing lower limb weakness and reduced sensation b e l o w his nipple line. On examination he had 2/5 strength in his lower limbs and reduced sensation to pin prick below his axillae. Joint position sense at his toes was absent. X-rays o f his cervical and thoracic spine revealed marked ankylosis but no fracture was seen. Computed tomography (CT) scans o f his C 4 - T 4 region with sagittal reconstruction showed the kyphosis but no fracture. Magnetic resonance image (MRI) scanning o f his cervicothoracic region was performed demonstrating a lesion lying dorsal to the spinal cord extending from C4 to T2 with significant compression o f the spinal cord (Figs. 1 & 2). The lesion was o f biconvex shape tapering superiorly and inferiorly. O n T l - w e i g h t e d images the lesion was isointense with the spinal cord and on T2-weighted images the lesion was heterogeneous and hypointense to the spinal cord; this was consistent with acute epidural hematoma. 4,t6 No evidence o f fracture was seen. At the time o f his neurological deterioration he was started on intravenous methylprednisolone as per the spinal cord injury
© Harcourt Brace & Co. Ltd 1998
the volume o f the cystic mass during the period o f 1 year. The radiological findings were also atypical in that they showed a spontaneously hyperdense, partially calcified nodule w h i c h was revealed histologically to be collapsed, thickened, calcified, 'degenerated' collagen bundles, containing thick-walled vessels some o f which were also calcified. The cystic c o m p o n e n t o f the tumour which extended towards the deeper layers o f the brain could have been misinterpreted in this case as a predominantly non-cortical lesion. Furthermore, at surgery skull thinning was evident but was not obvious radiologically. Histopathology is essential in arriving at a correct diagnosis o f DNTs and enables one to exclude other tumours a m o n g the differential diagnosis, including o l i g o d e n d r o g l i o m a and gliomas w h o s e treatment and p r o g n o s e s are quite different. 1,3,4,I1,12,~4,15 Prospective studies have established the stable character o f DNTs on radiological follow-up; however, special consideration must be given to those cases with cystic components. It has been s h o w n that up to 12.5% o f DNTs may present with cysts. It is possible that, in the present case, repeated haemorrhages as a result o f vascular dissections or ruptures, repeated vascular thrombosis with infarctions, in a hamartomatous vascular region, have led to a progressively expanding haemorrhagic cyst or h a e m a t o m a resulting in mass effect. Therefore, regular radiographic controls are necessary in cases o f cystic DNTs since they m a y present with progressive morphological changes. In our opinion, surgery remains the most effective treatment for DNTs located in non-functional areas o f the brain or those with mass effect.
457
Chronic intracranial hypotension Roderick A. Mackenzie, A. K. Lethlean, R. Shnier, P. W. Blum Institute of Neurological Sciences, Prince of Wales Hospital, High Street, Randwick, NSW 2031, Australia
Summary Acute intracranial hypotension can occur following lumbar puncture or a fall, and sometimes spontaneously. Most cases resolve within weeks or months but some require surgical repair of the defect causing leakage of cerebrospinal fluid (CSF). It is conceivable that such leaks could become chronic if the defect is incompletely sealed. We report the case of a 49-year-old male who presented with a 10-month history of headache associated with a leaking thoracic extradural arachnoid cyst. After this was repaired he reported relief not only of his recent headaches but also of chronic alcohol-related headaches. A long-standing anaemia resolved and tinnitus/hyperacusis improved. It is suggested that an injury 30 years before may have initiated the leak of CSF resulting in chronic intracranial hypotension. Journal of Clinical Neuroscience (1998)5(4), 457-460© HarcourtBrace& Co. Ltd 1998
Keywords: headache, spontaneous intracranial hypotension, low CSF pressure, spinal arachnoid cyst
REFERENCES 1. Daumas-DuportC, ScheithauerBW, ChodkiewiczJP, Laws ER, VedrenneC. Dysembryoplasticneuroepithelialtumor: a surgically curable tumor of young patients with intractable partial seizures. Neurosurgery 1988; 23: 545-556. 2. KuchelmeisterK, Demirel T, Schlorer E. Dysembryoplasticneuroepithelial tumour of the cerebellum. Acta Neuropathol 1995; 89: 385. 3. Leurlg SY, Gwi E, NG HK, Fung CF, Yam KY. Dysembryoplastic neuroepithelialtumor - a tumor with small neuronal cells resembling oligoderldroglioma.Amr J Surg Pathol 1994; 18: 604-614. 4. Daumas-DuportC. Dysembryoplasticneuroepithelialtumors. Brain Pathol 1993; 3: 283-295. 5. Kirkpatrick PJ, Honavar M, Polkey CE. Control of temporal lobe epilepsy following en bloc resection of low-grade tumors. J Neurosurg 1993; 78: 1%25. 6. ValiAM, Clarke MA, Kelsey A. Dysembryoplasticneuroepithelialtumour as a potentially treatable cause of intractable epilepsy in children. Clin Radiol 1993; 47: 255-258. 7. IwanagaK, Takahashi H, Kameyama S. Dysembryoplasticneuroepithelial tumor: report of a case without typical glioneuronalelements. Acta Neuropathol 1995 ; 89: 284-289. 8. RaymorldAA, Halpin SFS, Alsanjari N. Dysembryoplasticneuroepithelial tumor: features in 16 patients. Brain 1994; 117: 461-475. 9. DuncanR, Patterson J, Hadley DM, et al. CT, MR and SPECT imaging in temporal lobe epilepsy. J Neurol Neurosurg Psychiatry 1990; 53:11-15. 10. Koeller KK, Dillon WP. Dysembryoplasticneuroepithelialtumors: MR appearance. Am J Neuroradiol 1992; 13: 1319-1325. 1l. Hirose3, ScheithauerW, Lopes M, Beatriz S, VanderlbergScott R. Dysembryoplasticneuroepithelialtumor (DNT): an immunohistochemicaland ultrastructuraI study. J Neuropathol Exp Neurol 1994; 53: 184-195. 12. Prayson R, Estes L. Dysembryoplasticneuroepithelialtumor. Am J Clin Pathol 1992; 97: 398-401. 13. Pierce GB. The cancer cell and its control by the embryo. Am J Pathol 1983; 113: 117-124. 14. Gottschalk J, Korves M, Skotzek-KonradB, Goebel S, Cervos-NavarroJ. Dysembryoplasticneuroepithelialmicro-tumor in a 75-year-oldpatient with long-standing epilepsy. Clin Neuropathol 1993; 12: 175-178. 15. Prayson R, Estes L, Morris HH. Coexistence of neoplasia and cortical dysplasia in patients presenting with seizures. Epilepsia 1993; 34: 609~615.
© Harcourt Brace & Co. Ltd 1998
Received 23 September 1996 Accepted 20 December 1996 Correspodence to: Dr R. A. Mackenzie, Tel: 61 2 9525 8344; Fax: 61 2 9524
1173
INTRODUCTION The s y n d r o m e o f acute intracranial hypotension (AIH) is n o w well known. In the days following a spinal tap 1,2 or a fall, 3 and sometimes spontaneously, ~,4-16 the patient develops severe headache in the erect position w h i c h is relieved by lying down. In most o f the 100 cases reported in the literature, the duration o f s y m p t o m s was weeks to months, but in 3 cases 15a7,18 the s y m p t o m s recurred 2.2 and 10 years later, respectively. A I H is thought in most cases to result from a leak o f cerebrospinal fluid (CSF). The underlying defect causing the C S F leak usually remains obscure. Although small tears in nerve root sleeves or spinal extradural cysts have been mentioned as the likely cause o f apparently spontaneous intracranial hypotension (SIH) mzI9 these lesions have only occasionally been demonstrated with radiology and/or isotope cisternography. 12,17,2° All reported cases resolved spontaneously, following epidural blood patching, or following surgical repair o f the defect. However, it is conceivable that such leaks could recur with incidents that cause a rise in intracranial pressure such as exertion or minor trauma. Individuals with a small leak may have an unrecognized chronic intracranial hypotension (CIH) syndrome.
CASE R E P O R T A 49-year-old male presented with a 10-month history o f periodic headaches lasting 18-24 h and relieved by lying down. A slight
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chill often preceded the headache, which was felt as a dull ache in the frontal and occipital regions, building to a severe constant headache over several hours if the upright posture was maintained. Many of the headaches occurred 8-12 h after exertion, such as a vigorous game of squash or heavy lifting. He had a longer history of headaches occurring after alcohol ingestion, partly relieved by additional fluid intake. He had had a spinal tap at 4 years of age for what was probably an episode of mumps encephalitis. At 19 years of age he had fallen heavily on his buttocks and had severe headache for at least a week. From 35 years of age he had been aware of progressive deafness and tinnitus, initially on the right and then bilaterally. At 44 years of age he suddenly became aware of a defect of central vision in his right eye 36 h after heavy exertion. Examination at that time had shown impaired colour and brightness perception in the right eye associated with a small centro-caecal scotoma. Visual evoked responses were delayed and attenuated bilaterally, more so on the right, consistent with optic neuropathy. A magnetic resonance image (MRI) brain scan was reported as normal (Fig. 1). The visual disturbance improved over 6 weeks but he continued to experience blurring of right eye vision with exertion or when he had a hot drink. At the time of his presentation with periodic headache, the neurological examination was normal apart from bilateral sensorineural deafness, moderate on the right and mild on the left. Routine haematology and biochemistry was unremarkable apart from a mild normochromic normocytic anaemia (Hb 123 G/L). A second MRI brain scan (Fig. 2) showed descent of the cerebellar tonsils below the foramen magnum and thickening of the meningeal layer; gadolinium injection resulted in marked enhancement of the meninges. Review of the initial scans showed meningeal thickening, narrowed cerebral sulci and crowding of the optic chiasm that had not been appreciated previously (Fig. 1). A spinal tap produced CSF at an opening pressure of 58 mm (normal 80-150 mm) water; total protein content was 1100 mg/L (normal 200-400 rag/L). A diagnosis of CIH was made and 20 ml of the patient's blood was injected into the lumbar epidural space. He reported immediate relief of headache and a feeling of greater well-being. This recovery continued for 3 months, but he had return of headache 24 h after lifting a hea W weight. Repeat brain MRI showed no resolution of the abnormalities and an MRI scan of the thoracic spine (Fig. 3) showed an epidural fluid collection at T l l level. The cyst was located over the dorsolateral aspect and there was erosion of the left lamina and pedicle, consistent with a long standing pressure effect. At subsequent surgery, a 2 cm multi-loculated extradural arachnoid cyst was found over the left T l l nerve root sleeve. The cyst appeared to be held open by a ligamentous attachment, and CSF was leaking through the paper-thin covering membrane. The defect was oversewn and packed with muscle. The patient reported one further episode of chill followed by headache beginning 36 h after surgery and lasting 2 days, but has had no fitrther headaches of any kind in the subsequent 12 months. He reports increased well-being and his blood count is normal (Hb 142 G/L). A repeat MRI brain scan 6 months later (Fig. 4) showed resolution of the previously described abnormalities.
DISCUSSION Although most reported cases of AIH have resolved spontaneously over weeks or months, there are reports of three patients who had recurrent symptoms for 2.2 and 9 years. One patient 15had no cause found, while the other two 17'18 had leaks from raptured spinal arachnoid diverticula. The CSF leak in our patient may have resulted from his spinal tap as a child, and may have been exacerbated or caused by the fall at 19 years. Such trauma has been
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Fig. 1 MRI Tl-weighted brain (A) midline sagittal and (B) coronal sections at presentation with optic neuropathy.
Fig. 2 MRI Tl-weighted brain sections with gadolinium at presentation with periodic headaches.
known to cause a dural root sleeve tear resulting in AIH. 3 It is quite conceivable that such a tear could result in a persistent fistula admitting CSF into the soft tissue which would later become encased by a non-absorbing wall and lead to the formation of an extradural meningeal (false) cyst. 2~a3 We postulate that in our patient this cyst wall was fragile and subject to repeated leakage over many years. The MRI brain scan changes in AIH have been well defined. 12-15,24-28There is reduction in the volume of CSF, meningeal thickening and downward displacement of the brain; meningeal enhancement occurs with gadolinium injection. Fishman and Dillon26have postulated that the meningeal changes are due to dural vasodilation secondary to decreased intracranial pressure. The inner layer of dura mater contains less collagen than the tough outer layer and the vascular spaces are thin-walled, making them more susceptible to pressure changes. Mokri et al~5 have demonstrated that meningeal biopsy in AIH is usually normal but their patient with symptoms persistent over 2 years showed leptomeningeal reaction with hyperplasia of arachnoid cap cells and an increase in connective tissue. They postulated that this proliferative reaction was triggered by chronic venous engorgement in the inner layer of dura mater adjacent to the low pressure CSF (subarachnoid) compartment. Similar changes have been described in a patient with a 6-week history of AIH 27 and in relation to overdraining ventricular shunts. 28 The MRI scan of our patient showed meningeal thickening and crowding of the optic chiasm at the time of his episode of optic neuropathy but these changes were not recognized and gadolinium was not given. However, gadolinium enhancement was clearly demonstrated when he presented with periodic headaches. Both the meningeal thickening and the gadolinium enhancement resolved after successful treatment. Horton and Fishman 14 described visual obscurations, visual field defects and horizontal diplopia in AIH and suggested that traction and compression of the 2nd and 6th cranial nerves was the likely mechanism. The acute optic neuropathy in our patient was likely to be due to compression, seen as reduced CSF volume and crowding of the optic nerves on the original MRI scans. Although visual function has recovered symptomatically, visual evoked potentials (VEPs) remain delayed and attenuated, and he continues
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B Fig. 3 MRI T2-weighted thoracic spinal cord (A) coronal and (B) T l l horizontal sections at time of headache recurrence.
Fig. 4 MRI Tl-weighted brain sections with gadolinium 6 months after repair of defect,
to experience Uthoff's phenomenon. In the absence of any clinical or laboratory evidence of multiple sclerosis, compression of the optic nerves is the likely mechanism for the optic neuropathy. The patient's hearing problems may or may not be related to CIH. Deafness and tinnitus have been reported after spinal tap 29,3° and it has been suggested that these result from decrease in intralabyrinthine pressure because of the anatomical communication between this compartment and the subarachnoid space. Traction or compression of the auditory nerves caused by brain displacement is also possible. Our patient had progressive sensorineural deafness for 15 years that showed some improvement after the epidural repair but is still present. The cause of the anaemia is also unclear. Normocytic normochromic anaemia can be a manifestation of chronic inflammation but there was no cellular reaction in the CSF. He did report that his periodic headaches were often preceded by a slight chill, and 'occasional fever of variable degree' has been reported as part of the syndrome of AIH. It is possible that the fever and the anaemia resulted from an immune response to leaked CSF components which do not usually get through the blood-brain barrier but there is no literature support for this view.
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Alcohol-related headaches are thought to be due to cerebral vasodilatation and are exacerbated by lowered CSF pressure. While the commonest cause for this is dehydration, it appears in our patient to have been related to his CIH, since it improved dramatically following successful treatment. It seems quite possible that CIH may be an unrecognized cause of periodic and alcohol-associated headaches in other patients. Unless the typical clinical features are specifically sought, the diagnosis may be missed. Even MRI scanning may not detect subtle changes, as demonstrated here; this is especially likely to happen if gadolinium is not given. The fact that the scan is performed in the supine position also means that brain displacement may not be appreciated. The attending clinician must have a high index of suspicion for the diagnosis and perform spinal tap for confirmation. Measurement of spinal fluid pressure should again become an essential part of the assessment of patients with headache.
REFERENCES
1. BellWE, JoyntRJ, SahsAL. Low spinalfluidpressuresyndromes.Neurology 1961; 10: 512421. 2. GassH, GoldsteinAS, RuskinR, LeopoldNA. Chronicpost-myelogram headache.ArchNeurol 1971; 25: 168-170. 3. NosikWA. Intracranialhypotensionsecondaryto lumbarnerve sleevetear. JAMA 1955; 157: 1110-1111. 4. SchlatenbrandG. Neuereanschaugenzur pathophysiologicder liquorzirkulation.ZentralblNeurochir 1938; 3: 290-300. 5. PuechR LhermitteJ, BuvatJ, KoechlinA, PerrinJ. Un cas d'hypotension intracraniennespontanee,avecconstatationsanatomiques.Rev Neurol i942; 74: 316-320. 6. HesserS. Two casesof so-calledspontaneousaliquorrhoea.ActaMed Scand Suppl 1946; 170: 758-760. 7. LindquistT, MobergE. Spontaneoushypoliquorrhoea;reportof a case. Acta Med Scand I949; 132: 556460. 8. PageF. Intracranialhypotension.Lancet 1953; 2: 6749-6953. 9. ShenkinH, FinnesonB. Clinicalsignificanceof low CSF pressure.Neurology 1958; 8: 157-163. 10. LasaterGM. Primaryintracranialhypotension.Headache I970; 10: 63~56.
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11. MarcelisJ, Silberstein SD. Spontaneouslow CSF pressure headache. Headache 1990; 30: 192-196. 12. RandoTA, Fishman RA. Spontaneousintracranial hypotension:report of two cases and review of the literature. Neurology 1992; 42: 481-487. 13. PannnlloSC, Reich JB, Krol G, Deck MDF, Posner YB.MRI changes in intracranial hypotension.Neurology 1993; 43: 919-926. 14. HortonJC, Fishman RA. Neurovisual findings in the syndrome of spontaneous intracranial hypotensionfrom dural cerebrospinalfluid leak. Ophthalmology 1994; 101: 244-251. 15. MokriB, Patisi JE, ScheithanerBW, Piepgras DG, Miller GM. Meningeal biopsy in intracranialhypotension: meningeal enhancementon MRI. Neurology 1995; 45: 1801-1807. 16. Renowden SA, Gregory R, Hyman N, Hilton-JonesD. Spontaneous intracradial hypotension.J Neurol Nenrosurg Psychiatry 1995; 59: 511-515. 17. Davenport RJ, Chataway HJS, Warlow CR Spontaneousintracranial hypotensionfrom a CSF leak in a patient with Marfan's syndrome.J Neurol Neurosurg Psychiatry 1995; 59: 516-519. 18. Schievink WI, Meyer FB, Atldnson JLD, Mokri B. Spontaneousspinal cerebrospinaifluid leaks and intracraniaihypotension.J Neurosurg 1996; 84: 598--605. 19. PaulsonGW, Klawans HL Jr. Benign orgasmic cephalgia. Headache 1994; 13: 181-187. 20. Schi.evinkWI, Reimer R, Folger WN. Surgical treatment of spontaneous intracranial hypotension associated with a spinal arachnoid diverticulum. J Neurosurg 1994; 80: 736-739. 21. Schreiber F, Haddad B. Lumbar & sacral cysts causing pain. J Neurosurg 1951; 8: 504-509. 22. TarlovIM. Spinal perineural and meningeal cysts. J Neurol Neurosurg Psychiatry 1970; 33: 833-843. 23. LakePA, Minclder J, Scanlan RL. Spinal epidural cyst: theories of pathogenesis. J Neurosurg 1974; 40: 774-778. 24. SableSG, Ramadan NM. Meningeal enhancement & low CSF pressure headache. An MRI study. Cephalgia 1991; 11: 275-26. 25. HochmanMS, Naidich TE Kobetz SA, Fernandez-MaitinA. Spontaneous intracranial hypotension with pachymeningealenhancementon MRI. Neurology 1992; 42: 1628-1630. 26. Fishman RA, Dillon WE Dural enhancementand cerebral displacement secondary to intracranialhypotension.Neurology 1993; 43: 609-611. 27. MokriB, Krueger BR, Miller GM, Piepgras DG. Meningeal gadolinium enhancementin low-pressureheadaches. J Neuroimaging 1993; 3: 11-15. 28. Good DC, Ghobrial M. Pathologic changes associated with intracranial hypotension & meningeal enhancementon MRI. Neurology 1993; 43: 2698-2700. 29. VandamLD, Dripps RD. Long-term follow-up of patients who received 10 098 spinal anaesthetics.JAMA 1965; 161: 586-591. 30. Abouleish E, de la Vega S, Blendinger t, Tic T-O. Long-term follow-up of epidural blood patch. Anesth Analg 1975; 54: 459-463.
This technique may be used for approaching intraspinal pathology in high risk patients where extensive exposure may be contraindicated. Journal of Clinical Neuroscience (1998)5(4), 460-463 © HarcourtBrace& Co. Ltd 1998
Keywords: ankylosing spondylitis, cervical spine, spinal epidural hematoma, spinal endoscopy Received 22 October 1996 Accepted 3 December 1997 Correspondence to: Edmund H. Frank, Tel: 1 503 494 8070, Fax: 1 503 494
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INTRODUCTION Spinal epidural hematomas (SEH) are rare, but well described, lesions. They may be spontaneous or occur following trauma. Twenty cases o f SEH occurring following trauma in patients with ankylosing spondylitis (AS) have been reported.l-12 The mortality and morbidity o f spinal trauma in patients with AS is high. In the 20 reported cases o f SEH associated with AS, nine died and only eight made a good recovery. ~ Cardiorespiratory complications accounted for 50% o f the deaths following spine trauma in such patients. Our patient suffered rapidly worsening cardiac failure immediately following his injury. Systemic anticoagulation was necessary for the investigation and m a n a g e m e n t o f his coronary disease. Systemic anticoagulation is contraindicated in the face o f a pre-existing SEH and/or an extensive laminectomy. Most surgeons advocate withholding anticoagulants for at least 2 4 4 8 h following spinal procedures. We report the use o f a malleable spinal endoscope via a limited cervical laminotomy to remove the h e m a t o m a in an effort to reduce the risk o f postoperative h e m a t o m a formation. Spinal endoscopy is being increasingly used in an effort to reduce the morbidity associated with extensive approaches to the spine. The majority o f uses reported to date involve anterior approaches for thoracic pathology.i315 This case is the first reported use o f the endoscope for a SEH. The etiology and m a n a g e m e n t o f SEH is reviewed and potential applications for this technique are discussed.
CASE R E P O R T
Endoscopic treatment of spinal epidural hematoma Timothy R. Steel FRACS,Jordi X. Kellogg MD, Todd A. Kuether MD, Jacques Favre MD, Edmund H. Frank MD Oregon Health Sciences University, Division of Neurosurgery,, L-472, 3181 SW Sam Jackson Park Road, Portland, OR 97201, USA
Summary We report the use of a spinal endoscope via a limited cervical laminotomy to evacuate a spinal epidural hematoma. The patient was a 75-year-old male with a 32-year history of ankylosing spondylitis. Following a low speed motor vehicle accident he developed a cervicothoracic epidural hematoma without an associated fracture. Despite a rapidly improving neurological state, his rapidly deteriorating cardiorespiratory state required systemic anticoagulaUon necessitating decompression of the hematoma. The hematoma was successfully removed via a limited C6 and C7 laminotomy using the endoscope and a malleable disposable aspirator. We conclude that epidural hematomas can be readily evacuated via endoscopic techniques without extensive laminectomy. Joumal of Clinical Neuroscience (1998) 5(4)
A 74-year-old male with a 32-year history o f AS was brought to our emergency room following a low speed roll-over motor vehicle accident. H e suffered no head injury or loss o f consciousness. On arrival he was unable to lie flat on the spine board due to his 40% kyphosis and he complained o f severe pain in his cervicothoracic region, but was neurologically intact. Within 15 rain o f admission he complained o f rapidly developing lower limb weakness and reduced sensation b e l o w his nipple line. On examination he had 2/5 strength in his lower limbs and reduced sensation to pin prick below his axillae. Joint position sense at his toes was absent. X-rays o f his cervical and thoracic spine revealed marked ankylosis but no fracture was seen. Computed tomography (CT) scans o f his C 4 - T 4 region with sagittal reconstruction showed the kyphosis but no fracture. Magnetic resonance image (MRI) scanning o f his cervicothoracic region was performed demonstrating a lesion lying dorsal to the spinal cord extending from C4 to T2 with significant compression o f the spinal cord (Figs. 1 & 2). The lesion was o f biconvex shape tapering superiorly and inferiorly. O n T l - w e i g h t e d images the lesion was isointense with the spinal cord and on T2-weighted images the lesion was heterogeneous and hypointense to the spinal cord; this was consistent with acute epidural hematoma. 4,t6 No evidence o f fracture was seen. At the time o f his neurological deterioration he was started on intravenous methylprednisolone as per the spinal cord injury
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