Raised CSF Pressure Research Articles

Ventriculo-peritoneal shunting is a safe and effective treatment for idiopathic intracranial hypertension

Purpose: To determine the outcome of ventriculo-peritoneal shunts as a treatment for idiopathic intracranial hypertension (IIH)Materials and Methods: Retrospective case series of 28 patients with IIH and evidence of raised intracranial pressure (ICP) who underwent shunt insertion. Patients were identified from a prospectively updated operative database. A case-notes review was performed and data on type of shunt, pre- and post-operative symptoms, ophthalmological findings and post-operative complications were recorded.Results: All patients had symptoms of IIH that had failed medical management. Twelve patients had previous lumbo-peritoneal shunts and 2 patients had previous venous sinus stents. All patients had evidence of raised ICP as papilloedema and raised CSF pressure on lumbar puncture. Twenty-seven patients received a ventriculo-peritoneal shunt and 1 patient a ventriculo-atrial shunt. Twenty-six patients received Orbis Sigma Valves and 2 patients Strata valves. At follow-up all patients (100%) had improvement/resolution of papilloedema, 93% had improved visual acuity and 84% had improved headaches. Mean time to last follow-up was 15 (range 4–96) months. Complications occurred in 3 patients (11%): 2 patients required revision of their peritoneal catheters and 1 patient had an anti-siphon device inserted.Conclusions: Previous literature reported a ventricular shunt revision rate of 22–42% in the management of IIH. We demonstrate ventriculo-peritoneal shunts to be an effective treatment with a revision rate of 11% compared to the previously reported 22–42%.

A Clinical Outcome of Dexamethasone therapy of Patients with Pyogenic Meningitis

Objective: Observation of hastening effect of dexamethasone on recovery time and reductions of case fatality and neurological complications of pyogenic meningitis. Methodology: A total of 39 patients with pyogenic meningitis were considered on the basis of short clinical history and laboratory findings like, fever, headache, projectile vomiting, confusion/delirium, unconsciousness and neck rigidity and kerning’s sign, raised CSF pressure, presence of turbidity or purulent CSF with identification of bacteria on gram staining or culture of CSF, High CSF cell count (> 10 to 50mg/dl). Results: Data was analyzed by using MS-Office software. Conclusions: Adjunctive dexamethasone therapy may improve the outcome of pyogenic meningitis in adolescent and adult patients. And the intensity of headache, vomiting and fever were also relieved by using dexamethasone therapy.

INTRACRANIAL HYPERTENSION DUE TO ARTERIOVENOUS MALFORMATION

IntroductionWe review a patient with a known occipital arteriovenous malformation. He subsequently presented with worsening symptoms and was found to have elevated CSF pressure. We review the pathophysiology for the...

INTRACRANIAL ARTERIOVENOUS MALFORMATION PRESENTING WITH PAPILLOEDEMA

Intracranial arteriovenous malformations (AVM) typically present clinically with haemorrhage, seizures or focal neurological deficit. AVM do not usually have a substantial mass effect or cause raised intracranial pressure. Rarely shunting...

Distinguishing between the innate immune response due to ocular inflammation and infection in a child with juvenile systemic granulomatous disease treated with anti-TNF- monoclonal antibodies

Sir, Early-onset sarcoidosis (EOS) is often diagnosed if there is granulomatous involvement of an organ or sometimes as a diagnosis of exclusion in a child presenting with fever, rash and swollen joints. Recent evidence points to the fact that EOS is synonymous with juvenile systemic granulomatous disease (JSGD), also eponymously known as Blau syndrome (BS) or Jabs disease [1, 2]. They share clinical features of uveitis, dermatitis and arthritis, and are caused by inherited (BS) or sporadic (EOS) mis-sense mutations in the NACHT [NAIP (neuronal apoptosis inhibitory protein), CIITA (MHC Class II transcription activator), HET-E (incompatibility locus protein from Podospora anserina) and TP1 (telomerase-associated protein)] protein domain of the NOD2 (CARD15) gene, a major orchestrator during innate immune responses [1, 3]. Granulomatous disease of any origin may cause a panuveitis characterized by inflammation in the anterior and vitreous chambers and also the choroid or retina. We report a case of JSGD with a novel mutation and highlight the difficulty in distinguishing between granulomatous inflammation and infection in ocular inflammation. The importance of this is magnified when anti-TNF-α agents are implicated. We also speculate on mechanisms by which infection and inflammation may share a similar clinical profile. A 15-month-old Caucasian male, C.P., presented with a 2-week history of spiking fevers, a follicular, erythematous rash and symmetrical polyarthritis affecting the small joints of hand, ankles, knees and wrists and hips (Fig. 1). He had normocytic anaemia (7 g/dl), normal ferritin and elevated ESR and CRP (100 mm; 200 mg/l). Autoantibodies including RF and ANA were negative and infection was excluded. Biopsies were not taken from skin or SF. Fig. 1 Synovitis of the ankles and follicular rash. A clinical diagnosis of systemic-onset JIA was made initially followed by treatment with MTX, IVIG and anakinra alongside frequent, intermittent courses of oral, IA and i.v. CS therapy for recurrences of rash and arthritis over the next 4 years. Consequently, etanercept and MTX were instituted when C.P. was 6 years old. Etanercept was stopped upon developing bilateral anterior uveitis [4]. In order to control both ocular disease and arthritis, adalimumab was started but 8 weeks later, C.P. was admitted as an emergency with papilloedema, headache and raised CSF pressure, CSF leucocyte count and protein. Investigations for tuberculosis were negative including CSF PCR, CSF and blood culture, ELISpot™ (Mabtech, Nacka, Sweden) IFN-γ-release assay (IGRA) and TST (tuberculin skin test). Despite negative results, he was treated empirically with a full course of quadruple therapy for tuberculous meningitis and adalimumab was stopped. Ocular inflammation relapsed during anti-tuberculous therapy with reduction of vision attributable to panuveitis [4] with choroidal infiltrate; this responded to oral CS and MTX. Aqueous fluid was sampled under general anaesthetic for MTb PCR but the sample volume was insufficient. At this stage, we revised the differential to consider inflammatory granulomatous diseases as neither granulomatous ocular disease nor recurrent rash are consistent with a diagnosis of JIA. Venous blood samples from C.P. and his biological parents were obtained for genomic DNA extraction and genotyping of the NOD2 exonic regions. This analysis revealed a novel, de novo heterozygous mutation at position c.1558 C > T in exon 4 encoding the amino acid substitution H520Y in C.P., confirming a diagnosis of JSGD. Following reported success with infliximab and its more potent TNF-α blockade, C.P. was restarted on infliximab after 9 months of anti-tuberculous therapy normal lung imaging and repeated negative ELISpot™. C.P. is now 8 years old, he remains in drug-induced remission for joint and ocular disease and has vision of 6/5 in both eyes.

Paroxysmal radiculopathy associated to raised CSF pressure in a patient with spinal meningeal cysts

Spinal meningeal cysts are diverticula of the meningeal sac, erve root sheath or arachnoid. Their spinal localization varies, and ight be extradural with orwithout nerve root fibers, or intradural 1]. These cysts can be asymptomatic or cause radiculopathy or yelopathy by exerting pressure near the spinal cord and nerve oots. There is a different form of presentation associated with aised intracranial pressure (ICP) [2]. Herein, we show that raised ressure occurs with Valsalva maneuvers or postural changes. We resent a case of paroxysmal radiculopathywith fasciculations and rises of back pain caused by this pathological pressure. We proose a method to diagnose and treat this rare clinical presentation f spinal meningeal cysts, and suggest its pathophysiology.

Magnetic resonance “flip-flop” in idiopathic intracranial hypertension

Idiopathic intracranial hypertension (IIH) is a headache syndrome with raised CSF pressure in the absence of an intracranial mass lesion. Though earlier confined to excluding intracranial lesions, magnetic resonance imaging (MRI) in recent years has been shown to identify intracranial changes from prolonged raised CSF pressure, suggestive of IIH. We present the MRI and TOF (time-of-flight) venography findings involving the orbit, sella tursica and cerebral venous structures in a 45-year-old lady with IIH and illustrate their reversibility (“flip-flop”) following CSF drainage. Our case highlights the role of imaging in evaluation and follow-up of patients with IIH, without the need for repeated lumbar punctures to monitor pressures.

Return of meningeal symptoms in a patient treated for cryptococcal meningitis

Sirs: A 28-year-old Guinese man was admitted to our hospital with a 3-week history of severe headache, vomiting and fever. Ten months earlier he was diagnosed with AIDS. The presenting AIDS-defining illness then, was disseminated cryptococcosis: Cryptococcus neoformans had been recovered from CSF, bronchoalveolar washings, and blood. Standard treatment of CM with amphotericin B and flucytosine followed by fluconazole [1] resulted in clinical and microbiological cure. The patient had been taking fluconazole 200 mg QD PO as secondary prophylaxis since. After successful treatment of CM, HAART was initiated. This preceded admission by 320 days. The CD4 cell count rose from 0 to 120 cells/µl and HIV1-RNA to undetectable levels (<50 copies/ ml). There was no reason to doubt compliance to fluconazole prophylaxis. On examination an ill-looking man was seen, with low-grade pyrexia and stable haemodynamics. He was alert and cooperative, with no signs of meningism or mental confusion. Neurological examination and further physical examination revealed no abnormalities. Laboratory results were unremarkable. Brain CT-scan without contrast did not show any mass lesions, or hydrocephalus. Clear CSF was obtained, opening pressure was >50 cm H2O. CSF contained 0.59 g/l protein, 3.0 mmol/l glucose (at a serum glucose concentration of 5.1 mmol/l) and 272 × 106/l leukocytes, mainly monocytic. It tested positive for cryptococcal antigen. Considering relapse CM, treatment appropriate to this diagnosis was initiated (amphotericin B 1 mg/kg intravenously per day and flucytosine 100 mg/ kg/d PO). After 1 week his headache and malaise had not improved. Both stain and culture were negative for Cryptococcus neoformans. Cryptococcal antigen titers were found to be low: 1:16 in serum and 1:4 in CSF. Therefore the diagnosis was revised to Cryptococcus neoformans-related immune reconstitution inflammatory syndrome (IRIS). Antifungal treatment was discontinued and prednisone was prescribed (1 mg/kg/day IV). One more lumbar puncture was needed for temporary relief of symptoms due to raised CSF pressure. Our patient made a dramatic recovery and was discharged from hospital 3 days later, symptom-free. Prednisone was tapered in 4 months. IRIS can manifest itself as a latent infection unmasked by immune reconstitution, or as sterile inflammation in response to persisting antigens of an infection already treated. Therefore, latent infection should be ruled out in patients commencing antiretroviral therapy. Key to the diagnosis of cryptococcal-related IRIS in patients treated with HAART is the absence of fungal cells in all cultures. Cryptococcal antigen in blood or CSF of HIV-infected patients previously treated for CM, is a poor marker of infection [2]. Shelburne et al. found low cryptococcal antigen titres of value in discriminating cryptococcal-related IRIS from CM in a retrospective cohort study [3]. Other factors predictive of cryptococcal-related IRIS are a higher white blood cell count in CSF, higher CSF glucose and a higher CD4 cell count. . Lumbar puncture opening pressure was significantly higher in the IRIS group. These findings reflect the inflammatory nature of the syndrome due to immune restoration. The presence of the following risk factors for IRIS support the diagnosis in this case: a rise in CD4 cell count, a decrease in viral load, and compliance with a secondary prophylactic treatment regime of fluconazole. For treatment of the aseptic meningitis, anti-inflammatory drugs are a logical choice. Short-term corticosteroid administration was shown to be safe and well tolerated in patients who are receiving antiretroviral treatment [4]. It proved beneficial to prevent paradoxical reactions in tuberculous meningitis [5] and in other described cases of cryptococcal meningitis associated IRIS [6]. At a dose of 60 mg prednisone IV, our patient responded well. However, studies defining the role of corticosteroids in IRIS are urgently needed. The use of corticosteroids was demonstrated to be harmful in CM or cryptococcoma [7]. Therefore, exclusion of active cryptococcal disease remains the most important step in managing Cryptococcus neoformans—related IRIS. Guidelines for the treatment of CM recommend daily lumbar punctures in all patients with initially elevated opening pressures (>25 cm H2O) until the pressure is adequately controlled [1, 7]. Since the mechanism for increased intracranial pressure is thought to be the same in CM and cryptococcal IRIS, adherence to these guidelines seems prudent. Concluding, the HIV-positive patient treated with highly active antiretroviral therapy and returning meningeal symptoms after treatment for cryptococcal meningitis is diagnostically challenging. Distinguishing between Cryptococcus neoformans-related IRIS and active cryptococcal disease as two different clinical entities is crucial.

MRI of optic disc edema in childhood idiopathic intracranial hypertension.

Received: 30 September 2003 Revised: 13 November 2003 Accepted: 13 November 2003 Published online: 14 January 2004 Springer-Verlag 2004 Idiopathic intracranial hypertension (IIH) (pseudotumor cerebri) is characterized by headache owing to raised CSF pressure without an intracranial mass lesion or hydrocephalus. Clinically, there is usually papilledema without focal neurologic findings, except for occasional VI cranial nerve palsy [1]. However, a report of 22 pediatric patients with IIH showed increased CSF pressure without papilledema in 4 patients [2]. Physical protrusion of the optic papilla into the vitreous of the globe is caused by transmission of increased CSF pressure along the subarachnoid space surrounding the optic nerve, resulting in distension of the perioptic subarachnoid space. Recognized MR features of IIH include flattening of the posterior sclera, empty sella, distension of the perioptic subarachnoid space, enhancement and intraocular protrusion of the prelaminar optic nerve and vertical tortuosity of the orbital optic nerve [3]. These findings are usually bilateral, although not always completely symmetrical. (Figs. 1, 2).

Alzheimer's disease, normal‐pressure hydrocephalus, and senescent changes in CSF circulatory physiology: a hypothesis

There is evidence that production and turnover of CSF help to clear toxic molecules such as amyloid-beta peptide (Abeta) from the interstitial-fluid space of the brain to the bloodstream. Two changes in CSF circulatory physiology have been noted as part of ageing: first, a trend towards lower CSF production, hence a decrease in CSF turnover; and second, greater resistance to CSF outflow. Our hypothesis is that, all else being equal, the initially dominant physiological change determines whether CSF circulatory failure manifests as Alzheimer's disease (AD) or as normal-pressure hydrocephalus (NPH). If CSF production failure predominates, AD develops. However, if resistance to CSF outflow predominates, NPH results. Once either disease process takes hold, the risk of the other disorder may rise. In AD, increased deposition of Abeta in the meninges leads to greater resistance to CSF outflow. In NPH, raised CSF pressure causes lower CSF production and less clearance of Abeta. The disorders may ultimately converge in vulnerable individuals, resulting in a hybrid as has been observed in several clinical series. We postulate a new nosological entity of CSF circulatory failure, with features of AD and NPH. NPH-AD may cover an important subset of patients who carry the diagnosis of either AD or NPH.

Lumbar puncture and the risk of herniation: when should we first perform CT?

Death following lumbar puncture (LP) is feared by physicians. Many opinions are found in literature on the question whether computed cranial tomography (CT) should be performed before LP, to prevent herniation. These opinions are mainly based on retrospective studies and pathophysiological reasoning. In this review the difficulties in the decision whether we should perform CT before LP are discussed. It is explained that the concept of "raised intracranial pressure" is confusing, and that the less ambiguous terms "brain shift" and "raised CSF pressure" should be used instead. Brain shift is a contraindication to LP, whether CSF pressure is raised or not, and whether papilloedema is present or not. Subsequently, recommendations are offered for indications to perform CT before LP, grouped according to the safety and clinical utility of LP.

Acute visual loss following administration of metronidazole: A case report

We describe a patient with acute headache, disc swelling, and progressive visual loss following short exposure to the 5-nitroimidazole, metronidazole. There were no clinical features to suggest acute meningeal disease. Investigations revealed a moderately raised CSF pressure with mild pleocytosis and elevated protein. Cerebral venous sinus thrombosis was excluded by MRI with venography. The raised intracranial pressure alone is unlikely to have produced the patient’s severe and rapid visual loss. Therefore, metronidazole probably also had a direct effect on the optic nerves. The common underlying mechanism may be the development of perivascular oedema affecting both the vasa nervorum of the optic nerves and the intracranial vessels thus impairing optic nerve function and elevating intracranial pressure. The patient’s visual loss was eventually accelerated by his marked hypermetropia with crowded discs leading to secondary anterior ischaemic optic neuropathy in the presence of grossly swollen discs. Visual loss has not previously been reported in this context and should be added to the list of neurological complications of metronidazole.

Idiopathic intracranial hypertension in female homozygous twins.

The authors report on female homozygous twins with idiopathic intracranial hypertension. At the age of 12 years, both twins simultaneously developed visual disturbances with photophobia. At the age of 19...

Persistence of papilloedema after operation for intracranial tumour

The resolution of papilloedema after removal of an intracranial tumour in 14 patients (group 1) was compared with that after a CSF shunt operation in 12 patients (group 2). In group 1, the longest time needed for resolution was 20 weeks; there was no consistent relation between severity of papilloedema and time of resolution, and in 4 patients papilloedema subsided only after more than 10 weeks. In group 2, the longest time needed for resolution was 6 weeks and the time of resolution was closely related to the severity of papilloedema. Persistence of papilloedema after removal of an intracranial tumour may be explained by raised CSF pressure caused by a postoperative CSF circulation block which occurs in some patients. If vision is threatened in such cases, a CSF shunt operation should be performed.

Papilloedema, CSF pressure, and CSF flow in cerebral tumours.

The presence or absence of papilloedema and the cisternal CSF pressure were compared with the RIHSA cisternograms in 24 patients with supratenotorial tumours. It was found that severe subarachnoid obstruction of CSF flow, leading to impaired CSF absorption at the superior sagittal sinus, was the main cause of raised CSF pressure and papilloedema associated with supratentorial tumours.

Benign intracranial hypertension: A retrospective and follow-up study

Thirty-six patients with benign intracranial hypertension (BIH) were reviewed. Follow-up was obtained on 33 patients (91%) after a mean period of 7 1 2 years. Precipitating factors were found in 27 patients (75%). On admission, 5 patients had retro-ocular pain, especially on eye movements, a complaint not yet described in BIH. Seven patients had nystagmus, two of them horizontal positional nystagmus. It is questionable whether all signs in BIH are caused by the raised CSF pressure. The general outcome was good. Only two patients sustained severe ultimate visual impairment. Both presented with retro-ocular pain and sudden loss of vision on admission. Papilloedema can persist for years in BIH without serious visual impairment. Sometimes “causal” treatment is possible. No symptomatic treatment which is free from complications has been proved to prevent visual failure.

RIHSA cisternography in cerebral tumours.

RIHSA cisternography was abnormal in all of 24 patients with supratentorial tumours. The most common finding was asymmetry caused by ipsilateral obstruction of the sylvian fissure and the subarachnoid space around the convexity. Another frequent finding was partial or total obstruction at the tentorial notch, often in combination with reduced or absent activity along the superior sagittal sinus. These disturbances of CSF flow may explain the pathogenesis of raised CSF pressure with cerebral tumours.

Normal and raised CSF pressure in cerebral tumours

Normal and raised CSF pressure in cerebral tumours

Central nervous system leukaemia in children

Infiltration of the nervous system is the most common extramedullary manifestation of acute leukaemia in childhood. Of 28 children with acute lymphoblastic leukaemia (ALL) diagnosed between 1964 and 1969 and followed regularly thereafter, 21 (75%) developed neurological leukaemia. The first episodes occurred between 20 days and 27 mth. from diagnosis, but 64% of all cases of leukaemia and 86% of all patients destined to develop neurological leukaemia had evidence of infiltration by 15 mth. from diagnosis. The common features of the initial episode were headaches (10), vomiting (13), drowsiness (9), mood changes (10), cranial nerve palsies (8), spinal or peripheral nerve involvement (l), papilloedema (14), raised CSF pressure (10/17), CSF pleocytosis (17/20), elevated CSF protein above 40 mg./100 ml. (8/17), and CSF glucose levels below 35 mg./l00 ml. (5/16). The initial complication occurred during haematological remission in 11, during frank relapse in 8, during partial control in 1, and during the remission induction phase in one patient. The 21 patients suffered from neurological leukaemia on 82 occasions, the greatest number being 13 episodes in one child. On 74 occasions there was evidence of brain and or meningeal (CNS) involvement, on 4 occasions spinal or peripheral nerve infiltration, and in 4 patients a combination was present. Six of the children had a prolonged survival of from 3-5yr. 11 mth., and the longest survivor remains in continuous haematological remission. The six children who did not develop neurological leukaemia survived from 18 to 86 mth., 3 being still alive at 34, 63 and 68 mth. from diagnosis. Of 18 patients with ALL diagnosed between January 1970 and June 1971, 4 have already developed CNS leukaemia at intervals of 4½ to 12 mth. from diagnosis. As only 3 have been followed for more than 15 mth., the incidence of neurological leukaemia is likely to increase. In view of the extremely high incidence of neurological infiltration in acute lymphoblastic leukaemia, prophylactic treatment with intrathecal methotrexate and radiotherapy soon after diagnosis appears warranted.