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CSF rhinorrhoea refers to the drainage of cerebrospinal fluid through the nose. Measures of CSF components such as glucose have been used in the past, but are neither sensitive nor specific; beta-2 transferrin, however, has been shown to have a high positive predictive value. It has also been noted to be characterized by unilateral discharge.
It is a sign of basal skull fracture. Management includes watchful waiting - leaks often stop spontaneously; if this does not occur then neurosurgical closure is necessary to prevent the spread of infection to the meninges.
Other signs of basal skull fracture includes CSF otorrhoea (drainage of CSF through the ear). It can have devastating complications in some patients, as the communication between the nasal cavity and the cerebrospinal fluid and CNS can result in bacterial infections of the CNS that can have catastrophic effects on the patient.
CSF rhinorrhoea can also be a symptom of a pituitary adenoma.
Spontaneous CSF rhinorrhea. The most common congenital or acquired defect in the skull base bones (anterior cranial fossa) at the spontaneous nasal liquorrhea localized in following formation:
- sphenoid sinus (43%)
- ethmoid bone (29%)
- cribriform plate (29%)
Most people who develop SCSFLS feel the sudden onset of a severe and acute headache. It is a headache usually made worse by standing, typically becoming prominent throughout the day, with the pain becoming less severe when lying down. Orthostatic headaches can become chronic and disabling to the point of incapacitation. Some patients with SCSFLS will develop headaches that begin in the afternoon. This is known as "second-half-of-the-day headache". This may be an initial presentation of a spontaneous CSF leak or appear after treatment such as an epidural patch, and likely indicates a slow CSF leak.
Apart from headache, about 50% of patients experience neck pain or stiffness, nausea, and vomiting. Other symptoms include dizziness and vertigo, facial numbness or weakness, unusually blurry or double vision, neuralgia, fatigue, or a metallic taste in the mouth. Leaking CSF can sometimes be felt or observed as a discharge from the nose or ear.
Lack of CSF pressure and volume can allow the brain to sag and descend through the foramen magnum (large opening) of the occipital bone, at the base of the skull. The lower portion of the brain is believed to stretch or impact one or more cranial nerve complexes, thereby causing a variety of sensory symptoms. Nerves that can be affected and their related symptoms are detailed in the table at right.
SCSFLS is classified into two main types, cranial leaks and spinal leaks. The vast majority of leaks are spinal. Cranial leaks occur in the head. In some of these cases, CSF can be seen dripping out of the nose, or ear. Spinal leaks occur when one or more holes form in the dura along the spinal cord. Both cranial and spinal spontaneous CSF leaks cause neurological symptoms as well as spontaneous intracranial hypotension, diminished volume and pressure of the cranium. While referred to as "intracranial hypotension", the intracranial pressure may be normal, with the underlying issue instead being low-volume CSF. For this reason SCSFLS is referred to as "CSF hypovolemia" as opposed to "CSF hypotension".
A subdural hygroma is a collection of cerebrospinal fluid (CSF), without blood, located under the dural membrane. Most hygromas are believed to be derived from chronic subdural hematomas. They are commonly seen in elderly patients after minor trauma but can also be seen in children after an infection. One of the common causes of subdural hygroma is a sudden decrease in pressure as a result of placing a ventricular shunt. This can lead to leakage of CSF into the subdural space especially in cases with moderate to severe brain atrophy. In these cases the symptoms such as mild fever, headache, drowsiness and confusion can be seen, which are relieved by draining this subdural fluid.
Most subdural hygromas are small and clinically insignificant. Larger hygromas may cause secondary localized mass effects on the adjacent brain parenchyma, enough to cause a neurologic deficit or other symptoms. Acute subdural hygromas can be a potential neurosurgical emergency, requiring decompression. Acute hygromas are typically a result of head trauma—they are a relatively common posttraumatic lesion—but can also develop following neurosurgical procedures, and have also been associated with a variety of conditions, including dehydration in the elderly, lymphoma and connective tissue diseases.
A cerebrospinal fluid leak (CSFL) is a medical condition where the cerebrospinal fluid(CSF) in the brain leaks out of the dura mater. This can be due to a spontaneous cerebrospinal fluid leak or result from different causes such as a lumbar puncture or physical trauma. While high CSF pressure can make lying down unbearable, low CSF pressure due to a leak can be relieved by lying flat on the back.
The most common symptoms of a CSFL is extremely high pressure in the head when sitting, standing, or bending down which can be lessened by laying down flat.
A myelogram can be used to help identify a CSFL by injecting a dye to further enhance the imaging allowing the location of the leak to be found. If it is a slow leak it may not appear on a single myelogram so more than one may be needed. Due to the ease of the procedure no anesthesia is used however a local anesthetic is given.
An epidural blood patch is the normal treatment for a CSFL, the patient's blood is drawn and it is then injected into the lumbar spine. Patients are told to lie flat without moving from 2 to 24 hours after the blood patch is done. A blood patch can be used to patch a CSFL in the cervical neck although it is rare for it to be done in that location, though it may take more than one blood patch to fully close the leak. Anesthesia is also uncommon for blood patch procedures. If you have a low pain tolerance it would be a good idea to have anesthesia for all of the procedures.
If the leak is strong or fast, the loss of CSF fluid can cause the brain to drop inside the skull due to the body's inability to replenish the CSF fluid at a quick enough pace, which would show up on a MRI of the brain. This is called a Chiari malformation where the brain is lower in the skull almost in the spinal canal.
Patients with arachnoid cysts may never show symptoms, even in some cases where the cyst is large. Therefore, while the presence of symptoms may provoke further clinical investigation, symptoms independent of further data cannot—and should not—be interpreted as evidence of a cyst's existence, size, location, or potential functional impact on the patient.
Symptoms vary by the size and location of the cyst(s), though small cysts usually have no symptoms and are discovered only incidentally. On the other hand, a number of symptoms may result from large cysts:
- Cranial deformation or macrocephaly (enlargement of the head), particularly in children
- Cysts in the suprasellar region in children have presented as bobbing and nodding of the head called bobble-head doll syndrome.
- Cysts in the left middle cranial fossa have been associated with ADHD in a study on affected children.
- Headaches. A patient experiencing a headache does not necessarily have an arachnoid cyst.
- In a 2002 study involving 78 patients with a migraine or tension-type headache, CT scans showed abnormalities in over a third of the patients, though arachnoid cysts only accounted for 2.6% of patients in this study.
- A study found 18% of patients with intracranial arachnoid cysts had non-specific headaches. The cyst was in the temporal location in 75% of these cases.
- Seizures
- Hydrocephalus (excessive accumulation of cerebrospinal fluid)
- Increased intracranial pressure
- Developmental delay
- Behavioral changes
- Nausea
- Hemiparesis (weakness or paralysis on one side of the body)
- Ataxia (lack of muscle control)
- Musical hallucination
- Pre-senile dementia, a condition often associated with Alzheimer's disease
- In elderly patients (>80 years old) symptoms were similar to chronic subdural hematoma or normal pressure hydrocephalus:
- Dementia
- Urinary incontinence
- Hemiparesis
- Headache
- Seizures
Cerebrospinal fluid, which fills the subarachnoid space between the arachnoid membrane and the pia mater surrounding the brain, is normally clear and colorless. When there has been bleeding into the subarachnoid space, the initial appearance of the cerebrospinal fluid can range from barely tinged with blood to frankly bloody, depending on the extent of bleeding. Within several hours, the red blood cells in the cerebrospinal fluid are destroyed, releasing their oxygen-carrying molecule heme, which is then metabolized by enzymes to bilirubin, a yellow pigment. The most common cause for bleeding into the subarachnoid space is a subarachnoid hemorrhage from a ruptured cerebral aneurysm.
The most frequently employed initial test for subarachnoid hemorrhage is a computed tomography scan of the head, but it detects only 98% of cases in the first 12 hours after the onset of symptoms, and becomes less useful afterwards. Therefore, a lumbar puncture ("spinal tap") is recommended to obtain cerebrospinal fluid if someone has symptoms of a subarachnoid hemorrhage (e.g., a thunderclap headache, vomiting, dizziness, new-onset seizures, confusion, a decreased level of consciousness or coma, neck stiffness or other signs of meningismus, and signs of sudden elevated intracranial pressure), but no blood is visible on the CT scan. According to one article, a spinal tap is not necessary if no blood is seen on a CT scan done using a third generation scanner within six hours of the onset of the symptoms. However, this is not standard of care.
Heme from red blood cells that are in the cerebrospinal fluid because a blood vessel was nicked during the lumbar puncture (a "traumatic tap") has no time to be metabolized, and therefore no bilirubin is present.
After the cerebrospinal fluid is obtained, a variety of its parameters can be checked, including the presence of xanthochromia. If the cerebrospinal fluid is bloody, it is centrifuged to determine its color.
Arachnoid cysts can be found on the brain or on the spine. Intracranial arachnoid cysts usually occur adjacent to the arachnoidal cistern. Spinal arachnoid cysts may be extradural, intradural, or perineural and tend to present with signs and symptoms indicative of a radiculopathy.
Arachnoid cysts may also be classified as primary (congenital) or secondary (acquired) and have been reported in humans, cats, and dogs.
Arachnoid cysts can be relatively or present with symptoms; for this reason, diagnosis is often delayed.
Low-pressure hydrocephalus (LPH) is a condition whereby ventricles are enlarged and the individual experiences severe dementia, inability to walk, and incontinence - despite very low intracranial pressure (ICP). Low pressure hydrocephalus appears to be a more acute form of normal pressure hydrocephalus. If not diagnosed in a timely fashion, the individual runs the risk of remaining in the low pressure hydrocephalic state or LPHS. Shunt revisions, even when they are set to drain at a low ICP, are not always effective. The pressure in the brain does not get high enough to allow the cerebrospinal fluid to drain in a shunt system, therefore the shunt is open, but malfunctioning in LPH. In cases of LPH, chronic infarcts can also develop along the corona radiata in response to the tension in the brain as the ventricles increase in size. Certain causes of LPH include trauma, tumor, bleeding, inflammation of the lining of the brain, whole brain radiation, as well as other brain pathology that affects the compliance of the brain parenchyma. One treatment for the LPHS is an external ventricular drain (EVD) set at negative pressures. According to Pang & Altschuler et al., a controlled, steady, negative pressure siphoning with EVD, carefully monitored with partial computer tomography scans is a safe and effective way of treating LPH. In their experience, this approach helps restore the brain mantle. They caution against dropping or raising the pressure of the EVD too quickly as it increases risk and also destabilizes the ventricles. Getting the ventricles smaller, is the initial step, stabilising them is the second step before placing a shunt – which is the final step in therapy. Any variation from this formula can lead to an ineffective, yet patent shunt system, despite a low-pressure setting. Care should be taken with EVD therapy, as mismanagement of the EVD can lead to long-term permanent complications and brain injury.
A pseudomeningocele is an abnormal collection of cerebrospinal fluid (CSF) that communicates with the CSF space around the brain or spinal cord. In contrast to a meningocele, in which the fluid is surrounded and confined by dura mater, in a pseudomeningocele, the fluid has no surrounding membrane, but is contained in a cavity within the soft tissues.
Pseudomeningocele may result after brain surgery, spine surgery, or brachial plexus avulsion injury.
Treatment for pseudomeningocele is conservative or may involve neurosurgical repair.
Patients come to clinical attention early in life (usually at birth or within the first few months), with a firm subcutaneous nodule at bridge of nose, or as a polypoid mass within the nasal cavity, or somewhere along the upper border of the nasal bow. If the patient presents with an intranasal mass, there may be obstruction, chronic rhinosinusitis, or nasal drainage. If there is a concurrent cerebrospinal fluid (CSF) leak, then an encephalocele is much more likely.
This lesion is separated into two types based on the anatomic site of presentation:
1. Extranasal (60%): Subcutaneous bridge of nose
2. Intranasal (30%): Superior nasal cavity
3. Mixed (10%): Subcutaneous tissues and nasal cavity (larger lesions)
Xanthochromia, from the Greek "xanthos (ξανθός)"=yellow and "chroma (χρώμα)"=colour, is the yellowish appearance of cerebrospinal fluid that occurs several hours after bleeding into the subarachnoid space caused by certain medical conditions, most commonly subarachnoid hemorrhage. Its presence can be determined by either by spectrophotometry (measuring the absorption of particular wavelengths of light) or simple visual examination. It is unclear which method is superior.
Certain changes in morphology are associated with cerebral edema: the brain becomes soft and smooth and overfills the cranial vault, gyri (ridges) become flattened, sulci (grooves) become narrowed, and ventricular cavities become compressed.
Symptoms include nausea, vomiting, blurred vision, faintness, and in severe cases, seizures and coma. If brain herniation occurs, respiratory symptoms or respiratory arrest can also occur due to compression of the respiratory centers in the pons and medulla oblongata.
Nasal glial heterotopia refers to congenital malformations of displaced normal, mature glial tissue, which are no longer in continuity with an intracranial component. This is distinctly different from an encephalocele, which is a herniation of brain tissue and/or leptomeninges, that develops through a defect in the skull, where there is a continuity with the cranial cavity.
The following is a list of common signs and symptoms found with neonatal meningitis.
- Fever
- poor appetite
- anterior fontanelle bulging
- seizure
- jitteriness
- dyspnea
- irritability
- anorexia
- vomiting
- diarrhea
- abdominal distention (increase in abdominal size)
- neck rigidity
- cyanosis
- jaundice
- and sunset eyes (downward gaze of the eyes)
- abnormal body temperature (hypo-or hyperthermia)
- change of activity (lethargy or irritability)
Unfortunately these symptoms are unspecific and may point to many different conditions.
Froin's syndrome – coexistence of xanthochromia, high protein level and marked coagulation of cerebrospinal fluid (CSF). It is caused by meningeal irritation (e.g. during spinal meningitis) and CSF flow blockage by tumour mass or abscess. Stagnation of the CSF within the thecal sac facilitates exudation from the tumour itself and activation of coagulation factors. A clinical test formerly used for evaluation of spinal stenosis is Queckenstedt's maneuver. Nowadays, a magnetic resonance imaging is used for identification of CSF flow obstruction. It often shows the prolongation of T1 and T2 signal in CSF caudal to a level of block. This phenomenon is named after Georges Froin (1874–1932), a French physician who first described it.
If there are symptoms, people with empty sella syndrome can have headaches, as symptoms, which subsides when lying down. Additional symptoms are as follows:
- Abnormality (middle ear ossicles)
- Cryptorchidism
- Dolichocephaly
- Arnold-Chiari type I malformation
- Meningocele
- Patent ductus arteriosus
- Muscular hypotonia
- Platybasia
Symptoms of IVH are similar to other intracerebral hemorrhages and include sudden onset of headache, nausea and vomiting, together with an alteration
of the mental state and/or level of consciousness. Focal neurological signs are either minimal or absent, but focal and/or generalized seizures may occur. Xanthochromia, yellow-tinged CSF, is the rule. Diagnosis can be confirmed by the presence of blood inside the ventricles on CT.
The most obvious clinical sign of syringomyelia is pain. Dogs with CM alone do not seem to have signs, but some appear to have facial pain. Common symptoms in human patients include, severe headache and neck pain, dizziness, vertigo, disequilibrium, visual disturbances, ringing in the ears, difficulty swallowing, palpitations, sleep apnea, muscle weakness, impaired fine motor skills, chronic fatigue and painful tingling of the hands and feet, pruritus.
Symptoms are assessed on a case by case basis. Some cysts in the CNS can be asymptomatic (producing or showing no symptoms), depending on their location in the brain or spinal cord. If the cysts develop in critical areas of the central nervous system, they can present one or more of the following symptoms:
- Pressure in the spinal cord or brain
- Rupture of nerves around the cyst
- Weakness in specific parts of the body controlled by the cyst-infected brain region
- Inflammation
- Hydrocephalus
- Brainstem hemorrhage
- Seizures
- Visual disturbances and hearing Loss
- Headache
- Difficulty with balance or walking
In general, symptoms vary depending on the type of cyst and its location within the CNS.
Brain contusions and subarachnoid hemorrhages are commonly associated with IVH. The bleeding can involve the anterior communicating artery or the posterior communicating artery.
In both adults and infants, IVH can cause dangerous increases in ICP, damage to the brain tissue, and hydrocephalus.
NPH may exhibit a classic triad of clinical findings (known as the Adams triad or Hakim's triad) of urinary incontinence, gait disturbance, and dementia (commonly referred to as "wet, wacky and wobbly" or "weird walking water").
- Gait disturbance is typically the initial and most prominent symptom of the triad and may be progressive, due to expansion of the ventricular system, particularly at the level of the lateral ventricles, leading to traction on the corticospinal tract motor fibers descending to the lumbosacral spinal cord. The gait disturbance can be classified as mild (cautious gait or difficulty with tandem gait), marked (evident difficulty walking or considerable unstable gait) or severe (unaided gait not possible) In the early stages, most often this gait disturbance occurs in the form of unsteadiness and impaired balance, especially when encountering stairs and curbs. Weakness and tiredness of the legs may also be part of the complaint, although examination discloses no paresis or ataxia. Often a mobility aid is used for added stability, once the patient has reached the mild to marked stage. Such aids may include a quad cane or wheeled walker. The patient's gait at the marked stage will often show a decrease in step height and foot-floor clearance, as well as a decrease in walking speed. This style is often referred to as a magnetic gait, in which the feet appear to be stuck to the walking surface, and is considered the characteristic gait disturbance of NPH. The gait may begin to mimic a Parkinsonian gait, with short shuffling steps and stooped, forward-leaning posture, but there is no rigidity or tremor. An increased tendency to fall backwards is also seen, and a broad-based gait may be employed by the patient in order to increase their base of support and thereby their stability. In the very late stages, the patient can progress from an inability to walk, to an inability to stand, sit, rise from a chair or turn over in bed; this advanced stage is referred to as "hydrocephalic astasia-abasia".
- Dementia is predominantly frontal lobe in nature and of the subcortical type of dementia. It presents in the form of abulia, forgetfulness, inertia, inattention, decreased speed of complex information procession (also described as a dullness in thinking and actions), and disturbed manipulation of acquired knowledge, which is reflective of the loss of integrity of the frontal lobes. Memory problems are usually a component of the overall problem and have been predominant in some cases, which can lead to the misdiagnosis of Alzheimer's disease. However, in NPH there may be an obvious discrepancy between (often severely) impaired recall and intact or much less impaired recognition. The dementia is thought to result from traction on frontal and limbic fibers that also run in the periventricular region.
- Urinary incontinence appears late in the illness, and is found to be of the spastic hyperreflexic, increased-urgency type associated with decreased inhibition of bladder contractions and detrusor instability. In the most severe cases, bladder hyperreflexia is associated with a lack of concern for micturition due to the severe frontal lobe cognitive impairment. This is also known as "frontal lobe incontinence", where the patient becomes indifferent to their recurrent urinary symptoms.
There are two types of ESS: "primary" and "secondary".
- Primary ESS happens when a small anatomical defect above the pituitary gland increases pressure in the sella turcica and causes the gland to flatten out along the interior walls of the sella turcica cavity. Primary ESS is associated with obesity and increase in intracranial pressure in women.
- Secondary ESS is the result of the pituitary gland regressing within the cavity after an injury, surgery, or radiation therapy. Individuals with secondary ESS due to destruction of the pituitary gland have symptoms that reflect the loss of pituitary functions, such as intolerance to stress and infection.
Signs of the tumor resulting from increased intracranial pressure are present in 91% of patients, with vomiting, homonymous visual field defects and headache being the most common symptoms.
Other symptoms are ear ringing and dizziness.