Optical coherence tomography represents a sensitive and reliable tool for routine monitoring of idiopathic intracranial hypertension with and without papilledema.

BACKGROUND AND PURPOSE: We previously reported that certain optical coherence tomography (OCT) measures were sensitive and reliable in identifying idiopathic intracranial hypertension (IIH). This prospective study aimed to define OCT measures that allow differentiation of IIH with and without papilledema, thereby helping clinical decision-making. METHODS: Eight patients with IIH with papilledema, nine without papilledema and 19 with other neurological diseases were included. OCT measures were obtained before lumbar puncture and within 2 h, 1, 3 and 6 months after lumbar puncture with cerebrospinal fluid (CSF) removal. RESULTS: All patients with papilledema had increased retinal nerve fiber layer (RNFL) thickness and elevated CSF pressure. All patients without papilledema had normal RNFL but elevated CSF pressure. After CSF removal, reduced RNFL thickness was registered in all eight patients with IIH with papilledema. No significant change in RNFL thickness after CSF removal was observed in IIH without papilledema or in patients with other neurological diseases, although reduced CSF pressure was documented. RNFL thickness tended to be normal in patients with IIH with papilledema at 3-6 months after CSF removal. All patients with IIH showed increased rim area and rim thickness, but reduced optic cup volume regardless of RNFL thickness or papilledema. CONCLUSIONS: Retinal nerve fiber layer thickness is sensitive for monitoring acute IIH and evaluating treatment effect. Increased rim area and rim thickness and decreased optic cup volume are reliable parameters that indicate persistently increased CSF pressure and risk of relapse. OCT measures are sensitive and reliable for diagnosing subtle IIH even in the absence of papilledema.

[A HaNDL case with papilledema mimicking transient ischemic attack]. / Geçici iskemik atagi taklit eden ve papilödem saptanan bir HaNDL olgusu.

The syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL), is a rare, benign, self-limiting syndrome that is not well known. Cerebrospinal fluid examination (CSF) on clinical suspicion has diagnostic value for this syndrome, which has a rich neurological symptomatology that can mimic many diseases causing mortality and morbidity. Although the ethiopathogenesis of this syndrome is not fully known, autoimmunity is thought to be in the foreground. Described in this article is a HaNDL case that on first clinic presentation appeared to be a transient ischemic attack (TIA) with temporary but recurring neurological findings and normal CSF pressure accompanied by papilledema. Clinical features, epidemiology, ethiopathogenesis, differential diagnosis, and treatment of this syndrome are reviewed according to the literature.

Low cerebrospinal fluid protein in prepubertal children with idiopathic intracranial hypertension.

PURPOSE: To evaluate a clinical observation that prepubertal children with idiopathic intracranial hypertension (IIH) have low cerebrospinal fluid (CSF) protein levels compared to healthy children and pubertal patients with IIH. METHODS: The medical records of prepubertal and pubertal IIH patients and controls seen in the pediatric neuro-ophthalmology clinic at Duke between 2003 and 2013 were retrospectively reviewed. The control group consisted of children who had normal intracranial pressure on lumbar puncture performed to evaluate for headaches or anomalous-looking optic nerves. The records were analyzed with attention to demographic characteristics, clinical presentation, course, and lumbar puncture results. RESULTS: A total of 23 prepubertal children with IIH (age range, 0.75-13 years), 16 pubertal patients with IIH (age range, 13-21 years), and 12 controls (age range 3-14 years) were included. CSF analysis revealed that prepubertal children with IIH had significantly lower CSF protein levels (17.3 ± 5.7 mg/dL) compared to pubertal subjects with IIH (23.4 ± 8.4 mg/dL; P = 0.019) or healthy controls (23.5 ± 6.4 mg/dL; P = 0.011). Furthermore, 9 of 23 prepubertal IIH patients (39%) had abnormally low CSF protein level (<15 mg/dL), compared to zero pubertal IIH patients (P = 0.005) and zero controls (P = 0.015). Acetazolamide increased CSF protein level in 100% of patients who underwent repeat lumbar puncture after starting the medication (average increase, 10.3 ± 6.6 mg/dL). CONCLUSIONS: Low CSF protein level may have diagnostic utility as a biomarker for prepubertal IIH. Furthermore, this finding suggests that some cases of prepubertal IIH may be caused by CSF overproduction rather than decreased CSF resorption.

Cerebrospinal fluid dynamics between the basal cisterns and the subarachnoid space of the optic nerve in patients with papilloedema.

AIMS: To determine cerebrospinal fluid (CSF) dynamics between intracranial CSF spaces and CSF in the subarachnoid space (SAS) of optic nerves (ONs) in 10 patients with papilloedema. METHODS: Prospective assessment of 10 patients with papilloedema and two control subjects using CT cisternography and analysis of CSF for the presence of lipocalin-like prostaglandin D synthase (betatrace protein). RESULTS: CT cisternography showed a progressively reduced influx of contrast-loaded CSF from intracranial CSF spaces into the SAS. The lowest concentration of contrast-loaded CSF was found in the region of the ON immediately behind the globe, where the ON sheath was widened (possibly by unfolding) in all patients compared with normal subjects. The concentration of lipocalin-like prostaglandin D synthase differed between the spinal CSF and the CSF in the SAS, with a markedly higher concentration in the SAS. CONCLUSION: The results of this study suggest that CSF turnover in the SAS of the ON is reduced in patients with papilloedema from various causes and that the composition of CSF differs between spinal CSF and that surrounding the ON.

Optic nerve compartment syndrome in a patient with optic nerve sheath meningioma.

PURPOSE: To report a patient with optic nerve (ON) sheath meningioma, unilateral optic disc swelling, and inhomogeneous cerebrospinal fluid (CSF) composition between lumbar CSF and CSF from the subarachnoid space (SAS) of the affected ON. METHODS: A 39-year-old woman presented with unilateral optic disc swelling and slight deterioration of visual function in the left eye. Extensive laboratory workup and magnetic resonance imaging (MRI) of the brain and orbits were performed. As radiotherapy was refused by the patient, ON sheath fenestration (ONSF) was offered and performed in order to stop deterioration. CSF from the SAS of the ON was sampled. RESULTS: Laboratory workup was within normal limits. MRI of the left orbit demonstrated enhancement of the dura in the precanalicular portion of the ON and distension of the SAS, most prominent in the bulbar portion of the ON. On lumbar puncture the opening pressure measured 19 (cm H2O). Compared to the lumbar CSF the CSF of the affected ON SAS showed markedly elevated measurements for albumin, IgG, and beta-trace protein. Visual function remained stable over a follow-up time of 18 months. CONCLUSIONS: Composition of CSF is considered to be homogenous throughout all CSF spaces. In this patient the authors found a marked concentration-gradient of albumin, IgG, and beta-trace protein between the CSF in the spinal canal and the CSF in the SAS of the affected ON. Based on the radiologic features of the left ON and the dissociated beta-trace protein concentrations in the CSF of the SAS of the ON and the lumbar CSF, the diagnosis of an ON sheath compartment syndrome due to an ON sheath meningioma was made.

Cerebrospinal fluid dynamics between the intracranial and the subarachnoid space of the optic nerve. Is it always bidirectional?

CSF is thought to flow continuously from the site of production in the ventricles into interconnected spaces; i.e. cisterns and subarachnoid spaces (SASs). Since the SAS of the optic nerve is defined by a cul-de-sac anatomy, it is not evident how local CSF might recycle from that region to the general SAS. The concept of free communication of CSF has recently been challenged by the description of a concentration gradient of beta-trace protein, a lipocalin-like prostaglandin d-synthase (L-PGDS), between the spinal CSF and that in the SAS of the optic nerve, indicating diminished local clearance or local overproduction of L-PGDS here. In fact, computed cisternography with a contrast agent in three patients with idiopathic intracranial hypertension and asymmetric papilloedema demonstrate a lack of contrast-loaded CSF in the SAS of the optic nerve despite it being present in the intracranial SAS, thus suggesting compartmentation of the SAS of the optic nerve. The concept of an optic nerve compartment syndrome is further supported by a concentration gradient of brain-derived L-PGDS between the spinal CSF and the CSF from the optic nerve SAS in the same patients.

Optic nerve sheath fenestration for intracranial hypertension associated with chronic inflammatory demyelinating polyneuropathy.

The authors report a case of papilledema in a 61-year-old woman with chronic demyelinating polyneuropathy. The cerebrospinal fluid protein level was elevated (2.68 to 4.51 g/L), as was the cerebro-spinal fluid opening pressure (27 to 40 cm H2O). A unilateral optic nerve sheath fenestration proved to be effective in preserving vision, and the papilledema resolved completely.

Utility of CSF pressure monitoring to identify idiopathic intracranial hypertension without papilledema in patients with chronic daily headache.

The aim of the present study was to report on the utility of continuous Pcsf monitoring in establishing the diagnosis of idiopathic intracranial hypertension without papilledema (IIHWOP) in chronic daily headache (CDH) patients. We report a series of patients (n = 10) with refractory headaches and suspected IIHWOP referred to us for continuous Pcsf monitoring between 1991 and 2000. Pcsf was measured via a lumbar catheter and analysed for mean, peak, highest pulse amplitude and abnormal waveforms. A 1-2 day trial of continuous controlled CSF drainage (10 cc/ h) followed Pcsf monitoring. Response to CSF drainage was defined as improvement in headache symptoms. Patients with abnormal waveforms underwent a ventriculoperitoneal (VPS) or lumboperitoneal (LPS) shunt insertion. All patients had normal resting Pcsf (8 +/- 1 mmHg) defined as ICP < 15 mmHg. During sleep, all patients had B-waves and 90% had plateau waves or near plateau waves. All patients underwent either a VPS or LPS procedure. All reported improvement of their headache after surgery. Demonstration of pathological Pcsf patterns by continuous Pcsf monitoring was essential in confirming the diagnosis of IIHWOP, and provided objective evidence to support the decision for shunt surgery. Increased Pcsf was seen mostly during sleep and was intermittent, suggesting that Pcsf elevation may be missed by a single spot-check LP measurement. The similarity between IIHWOP and CDH suggests that continuous Pcsf monitoring in CDH patients may have an important diagnostic role that should be further investigated.

Diagnostic criteria for idiopathic intracranial hypertension.

The syndrome of increased intracranial pressure without hydrocephalus or mass lesion and with normal CSF composition, previously referred to as pseudotumor cerebri, is a diagnosis of exclusion now termed idiopathic intracranial hypertension (IIH). Diagnostic criteria of this disorder have not been updated since the Modified Dandy Criteria were articulated in 1985. Since then, new developments, including advances in neuroimaging technology and recognition of additional secondary causes of intracranial hypertension, have further enhanced the ability to diagnose conditions that may mimic IIH. These factors are not addressed in the Modified Dandy Criteria. This report describes updated diagnostic criteria for IIH that may be used for routine patient management and for research purposes.

[Acute Trypanosoma brucei gambiense meningoencephalitis detected by papillary edema]. / Méningo-encéphalite aiguë à Typanosoma brucei gambiense révélée par un oedème papillaire.

A 26 year old woman, living in Brazzaville, was referred by her ophthalmologist about papillo-oedema. Neurological examination showed frontal syndrome and papillo-oedema without another sign of intracranial hypertension. Electroencephalogram revealed bilateral delta waves and bifrontal points. Significant inflammatory syndrome was noted. Cerebrospinal fluid examination showed trypanosomes with an elevated rate of protein and cells. Treatment by tetracosactide and eflornithine gave a good recovery. Authors are stressing the disconcerting feature of this clinical form. An urban from of the disease and the fight against african trypanosomiasis are also discussed.

Intracranial hypertension and cerebrospinal fluid production in dogs: effects of furosemide.

A method using chronically prepared, anesthetized dogs was devised for studying the effects of treatments of intracranial hypertension induced by applying a reversible extradural mass lesion while simultaneously measuring production of cerebrospinal fluid. This was measured with a ventricular-cisternal perfusion technique in which the rate of cisternal outflow could be controlled by a pump and matched to the inflow, allowing intracranial pressure to fluctuate despite simultaneous measurement of cerebrospinal fluid formation. Elevations of intracranial pressure to the range 20 to 35 Torr were induced and maintained during perfusion, but elevations above 35 Torr would not permit continued perfusion. At normal intracranial pressure, 10 Torr or less, rates of cerebrospinal fluid formation were the same whether the outflow controlling pump or free outflow was used. Formation of cerebrospinal fluid decreased progressively as intracranial pressure increased above 20 Torr. It also decreased with time after the start of perfusion during the course of 5 h, but returned to the initial range during the control phase of subsequent experiments in the same animal. Furosemide, 3 mg kg-1, i.v., had no significant effect on rate of formation but did induce a small decrease in ICP in time-controlled experiments in which i.v. fluid replacement limited net fluid losses to 20 ml kg-1 with no change in mean arterial or central venous pressures.

Pathogenesis of raised cerebrospinal fluid pressure.

Raised cerebrospinal fluid pressure may be caused by (a) subarachnoid CSF circulation blocks (b) obstruction of CSF absorption in the arachnoid villi (c) disorders of the venous return from the superior sagittal sinus to the heart. The common factor in these mechanisms is obstructed CSF drainage. Some clinical conditions illustrating these principles are reviewed.

Diagnostic significance and source of lactate dehydrogenase and its isoenzymes in cerebrospinal fluid of children with a variety of neurological disorders.

Two hundred and thirty-four cerebrospinal fluid (CSF) specimens from 183 different children were analysed for total lactate dehydrogenase (LD) activity and LD isoenzyme distribution. The LD activities were elevated in the CSF of patients with meningitis, especially with bacterial infections, and with central nervous system (CNS) leukaemia. The CSF LD isoenzyme patterns of both groups generally reflected the number and distribution of lymphocytes and granulocytes in the CSF. Increases in CSF LD levels also occurred in patients with other neurological disorders, such as hydrocephalus, raised intracranial pressure, and epileptic seizures. However, no significant increases in CSF LD activity nor abnormality of the isoenzyme distribution were noted in children who had had a non-specific febrile convulsion.