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Neuroimaging, usually with computed tomography (CT/CAT) or magnetic resonance imaging (MRI), is used to exclude any mass lesions. In IIH these scans typically appear to be normal, although small or slit-like ventricles, dilatation and buckling of the optic nerve sheaths and "empty sella sign" (flattening of the pituitary gland due to increased pressure) and enlargement of Meckel's caves may be seen.
An MR venogram is also performed in most cases to exclude the possibility of venous sinus stenosis/obstruction or cerebral venous sinus thrombosis. A contrast-enhanced MRV (ATECO) scan has a high detection rate for abnormal transverse sinus stenoses. These stenoses can be more adequately identified and assessed with catheter cerebral venography and manometry. Buckling of the bilateral optic nerves with increased perineural fluid is also often noted on MRI imaging.
Lumbar puncture is performed to measure the opening pressure, as well as to obtain cerebrospinal fluid (CSF) to exclude alternative diagnoses. If the opening pressure is increased, CSF may be removed for transient relief (see below). The CSF is examined for abnormal cells, infections, antibody levels, the glucose level, and protein levels. By definition, all of these are within their normal limits in IIH. Occasionally, the CSF pressure measurement may be normal despite very suggestive symptoms. This may be attributable to the fact that CSF pressure may fluctuate over the course of the normal day. If the suspicion of problems remains high, it may be necessary to perform more long-term monitoring of the ICP by a pressure catheter.
The diagnosis may be suspected on the basis of the history and examination. To confirm the diagnosis, as well as excluding alternative causes, several investigations are required; more investigations may be performed if the history is not typical or the patient is more likely to have an alternative problem: children, men, the elderly, or women who are not overweight.
A combination of lifestyle modifications and medications can be used for the treatment of dolichoectasias.
- Antihypertensive medications such as Thiazides, Beta Blocker, ACE Inhibitor
- Trental or other Pentoxifylline drugs
- Dietary changes
- Weight loss
- Regular exercise
Diagnosis is principally by MRI. Frequently, arachnoid cysts are incidental findings on MRI scans performed for other clinical reasons. In practice, diagnosis of symptomatic arachnoid cysts requires symptoms to be present, and many with the disorder never develop symptoms.
Additional clinical assessment tools that can be useful in evaluating a patient with arachnoid cysts include the mini-mental state examination (MMSE), a brief questionnaire-based test used to assess cognition.
Diagnostic methods include:
- Angiogram
Due to positive remodeling the plaque build-up shown on angiogram may appear further downstream on the x-ray where the luminal diameter would look normal even though there is severe narrowing at the real site. Because angiograms require x-rays to be visualized the number of times an individual can have it done over a year is limited by the guidelines for the amount of radiation they can be exposed to in a one-year period.
- Magnetic resonance imaging (MRI)
Magnetic resonance imaging has the ability to quantify the plaque anatomy and composition. This allows physicians to determine certain characteristics of the plaque such as how likely it is to break away from the wall and become an embolus. MRI does not use ionizing radiation, so the number of times that it is used on a single person is not a concern; however since it uses strong electric fields those who have metal implants in cannot use this technique.
- Computed tomography (CT)
Multidirectional computed tomography (MDCT) is better than regular CT scans, because it can provide a higher spatial resolution and it has a shorter acquisition time. MDCT uses x-rays to obtain the image; however it can identify the composition of the plaque. Thus it can be determined whether the plaque is calcified plaque and lipid-rich plaque, so the inherent risks can be determined. Subjects are exposed to a substantial amount of radiation with this procedure, so their use is limited.
Diagnosis commonly occurs later in childhood and often occurs incidentally in asymptomatic patients or as a cause of visual impairment. The first symptoms are commonly found during routine vision screenings.
A number of examinations can be used to determine the extent of the syndrome and its severity. Fluorescein angiography is quite useful in diagnosing the disease, and the use of ultrasonography and optical coherence tomography (OCT) are helpful in confirming the disease. Neuro-ophthalmic examinations reveal pupillary defects (see Marcus Gunn Pupil). Funduscopic examinations, examinations of the fundus of the eye, allow detection of arteriovenous malformations. Neurological examinations can determine hemiparesis and paresthesias. Malformations in arteriovenous connections and irregular functions in the veins may be distinguished by fluorescein angiographies. Cerebral angiography examinations may expose AVMs in the cerebrum. MRIs are also used in imaging the brain and can allow visualization of the optic nerve and any possible atrophy. MRI, CT, and cerebral angiography are all useful for investigating the extent and location of any vascular lesions that are affecting the brain. This is helpful in determining the extent of the syndrome.
The diagnostic criteria for SCSFLS is based on the 2004 International Classification of Headache Disorders, 2nd edn (ICHD-II) (Table 1) (50) criteria. However, the presentation of patients with confirmed diagnosis may be very different from that of the clinical diagnostic criteria and cannot be considered authoritative.
When cranial CSF leak is suspected because of discharge from the nose or ear that is potentially CSF, the fluid can be collected and tested with a beta-2 transferrin assay. This test can positively identify if the fluid is cerebrospinal fluid.
Imaging studies are performed before surgery or biopsy to preclude an intracranial connection. Images usually show a sharply circumscribed but expansile mass. It may be difficult to exclude the intracranial connection if the defect is small whether employing computed tomography or magnetic resonnance.
MRI is the most sensitive imaging technique that can be used for diagnosing NBD. As for the parenchymal NBD, medical doctors mainly monitor the upper brainstem lesion. In fact, it is possible that lesions extends to thalamus and basal ganglia. Another advantage of using MRI is the ability to perform Diffusion-weighted imaging, or diffusion MRI. This technique is the most sensitive tool to image an acute infarct. In the case of NBD, Diffusion MRI can determine whether the lesion were due to cerebral infarction. In other words, it can distinguish NBD from non-NBD neural disease. When only spinal cord is affected by NBD, brain looks perfectly normal when scanned by MRI. Therefore, it is necessary to scan the spinal cord as well when diagnosing possible NBD involvement. As for the non-parenchymal NBD, venous sinus thrombosis can be detected.
Midline shift measurements and imaging has multiple applications. The severity of brain damage is determined by the magnitude of the change in symmetry. Another use is secondary screening to determine deviations in brain trauma at different times after a traumatic injury as well as initial shifts immediately after. The severity of shift is directly proportional to the likeliness of surgery having to be performed. MLS also has the aptitude to diagnoses the very pathology that caused it. The MLS measurement can be used to successfully distinguish between a variety of intracranial conditions including acute subdural hematoma, malignant middle cerebral artery infarction, epidural hematoma, subarachnoid hemorrhage, chronic subdural hematoma, infarction, intraventrical hemorrhage, a combination of these symptoms, or the absence of pertinent damage altogether.
The term dolichoectasia means elongation and distension. It is used to characterize arteries throughout the human body which have shown significant deterioration of their tunica intima (and occasionally the tunica media), weakening the vessel walls and causing the artery to elongate and distend.
The major differential to consider in empty sella syndrome is intracranial hypertension, of both unknown and secondary causes, and an epidermoid cyst, which can mimic cerebrospinal fluid due to its low density on CT scans, although MRI can usually distinguish the latter diagnosis.
There are different approaches to non-invasive intracranial pressure measurement, which include ultrasound "time-of-flight" techniques, transcranial Doppler, methods based on acoustic properties of the cranial bones, EEG, MRI, tympanic membrane displacement, oto-acoustic emission, ophthalmodynamometry, ultrasound measurements of optic nerve sheath diameter, and Two-Depth Transorbital Doppler. Most of the approaches are "correlation based". Such approaches can not measure an absolute ICP value in mmHg or other pressure units because of the need for individual patient specific calibration. Calibration needs non-invasive "gold standard" ICP meter which does not exists.
Non-invasive absolute intracranial pressure value meter, based on ultrasonic Two-Depth Transorbital Doppler technology, has been shown to be accurate and precise in clinical settings and prospective clinical studies. Analysis of the 171 simultaneous paired recordings of non-invasive ICP and the "gold standard" invasive CSF pressure on 110 neurological patients and TBI patients showed good accuracy for the non-invasive method as indicated by the low mean systematic error (0.12 mmHg; confidence level (CL) = 0.98). The method also showed high precision as indicated by the low standard deviation (SD) of the random errors
(SD = 2.19 mmHg; CL = 0.98).
This measurement method and technique (the only non-invasive ICP measurement technique which already received EU CE Mark approval) eliminates the main limiting problem of all other non-successful "correlation based" approaches to non-invasive ICP absolute value measurement - the need of calibration to the individual patient.
Most arachnoid cysts are asymptomatic, and do not require treatment. Where complications are present, leaving arachnoid cysts untreated, may cause permanent severe neurological damage due to the progressive expansion of the cyst(s) or hemorrhage (bleeding). However, with treatment most individuals with symptomatic arachnoid cysts do well.
More specific prognoses are listed below:
- Patients with impaired preoperative cognition had postoperative improvement after surgical decompression of the cyst.
- Surgery can resolve psychiatric manifestations in selected cases.
Magnetic resonance imaging (MRI) and computed tomography (CT) brain scans can be used to identify these tumors.
The most common missed lesion is within the nasal cavity, where a fibrosed nasal polyp may be considered. However, it does not have glial tissue. Further, a polyp usually has mucoserous glands. The lesion is frequently misintrepreted as scar in the subcutaneous tissues, but scar in a <2 year old child would be uncommon. Special stains are frequently required to highlight the diagnosis.
Doctors detect midline shift using a variety of methods. The most prominent measurement is done by a computed tomography (CT) scan and the CT Gold Standard is the standardized operating procedure for detecting MLS. Since the midline shift is often easily visible with a CT scan, the high precision of Magnetic Resonance Imaging (MRI) is not necessary, but can be used with equally adequate results. Newer methods such as bedside sonography can be used with neurocritical patients who cannot undergo some scans due to their dependence on ventilators or other care apparatuses. Sonography has proven satisfactory in the measurement of MLS, but is not expected to replace CT or MRI. Automated measurement algorithms are used for exact recognition and precision in measurements from an initial CT scan. A major benefit to using the automated recognition tools includes being able to measure even the most deformed brains because the method doesn’t depend on normal brain symmetry. Also, it lessens the chance of human error by detecting MLS from an entire image set compared to selecting the single most important slice, which allows the computer to do the work that was once manually done.
The diagnosis of ESS, done via examination (and test), may be linked to early onset of puberty, growth hormone deficiency or pituitary gland dysfunction(at an early age). Additionally there is:
The treatment for Bonnet–Dechaume–Blanc syndrome is controversial due to a lack of consensus on the different therapeutic procedures for treating arteriovenous malformations. The first successful treatment was performed by Morgan et al. They combined intracranial resection, ligation of ophthalmic artery, and selective arterial ligature of the external carotid artery, but the patient did not have retinal vascular malformations.
If lesions are present, they are watched closely for changes in size. Prognosis is best when lesions are less than 3 cm in length. Most complications occur when the lesions are greater than 6 cm in size. Surgical intervention for intracranial lesions has been done successfully. Nonsurgical treatments include embolization, radiation therapy, and continued observation. Arterial vascular malformations may be treated with the cyberknife treatment. Possible treatment for cerebral arterial vascular malformations include stereotactic radiosurgery, endovascular embolization, and microsurgical resection.
When pursuing treatment, it is important to consider the size of the malformations, their locations, and the neurological involvement. Because it is a congenital disorder, there are not preventative steps to take aside from regular follow ups with a doctor to keep an eye on the symptoms so that future complications are avoided.
Diagnosis of IIA is based on finding an intracranial aneurysm on vascular imaging in the presence of predisposing infectious conditions. Positive bacterial cultures from blood or the infected aneurysm wall itself may confirm the diagnosis, however blood cultures are often negative. Other supporting findings include leukocytosis, an elevated erythrocyte sedimentation rate and elevated C-reactive protein in blood.
Colloid cysts can be diagnosed by symptoms presented. Additional testing will be required and the colloid cyst symptoms can resemble those of other diseases. MRI and CT scans are often used to confirm diagnosis.
Diagnosis of cerebrovascular disease is done by (among other diagnoses):
- clinical history
- physical exam
- neurological examination.
It is important to differentiate the symptoms caused by a stroke from those caused by syncope (fainting) which is also a reduction in cerebral blood flow, almost always generalized, but they are usually caused by systemic hypotension of various origins: cardiac arrhythmias, myocardial infarction, hemorrhagic shock, among others.
The most important initial investigation is computed tomography of the brain, which is very sensitive for subarachnoid hemorrhage. If this is normal, a lumbar puncture is performed, as a small proportion of SAH is missed on CT and can still be detected as xanthochromia.
If both investigations are normal, the specific description of the headache and the presence of other abnormalities may prompt further tests, usually involving magnetic resonance imaging (MRI). Magnetic resonance angiography (MRA) may be useful in identifying problems with the arteries (such as dissection), and magnetic resonance venography (MRV) identifies venous thrombosis. It is not usually necessary to proceed to cerebral angiography, a more precise but invasive investigation of the brain's blood vessels, if MRA and MRV are normal.
The development of accurate and reliable non-invasive ICP measurement methods for VIIP has the potential to benefit many patients on earth who need screening and/or diagnostic ICP measurements, including those with hydrocephalus, intracranial hypertension, intracranial hypotension, and patients with cerebrospinal fluid shunts. Current ICP measurement techniques are invasive and require either a lumbar puncture, insertion of a temporary spinal catheter, insertion of a cranial ICP monitor, or insertion of a needle into a shunt reservoir.