Arachnoid cyst Q04.

6
Arachnoid cysts are cerebrospinal fluid covered by arachnoidal cells and collagen[1] that
may develop between the surface of the brain and the cranial base or on the arachnoid
membrane, one of the three membranes that cover the brain and the spinal cord.[2]
Arachnoid cysts are a congenital disorder,[3] and most cases begin during infancy;
however, onset may be delayed until adolescence.[2]

Classification
Arachnoid cysts can be intracranial (in the cranium), or on the spine. Intracranial
arachnoid cysts usually occur adjacent to the arachnoidal cistern.[4] Spinal arachnoid cysts
may be extradural, intradural, or perineural and tend to present with signs and symptoms
indicative of a radiculopathy.[4]
Arachnoid cysts can be relatively asymptomatic or present with insidious symptoms; for
this reason, diagnosis is often delayed.

Signs and symptoms

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 or location.
Symptoms vary by the size and location of the cyst(s), though small cysts usually have no
symptoms and are discovered only incidentally.[2] On the other hand, a number of
symptoms may result from large cysts:
Cranial deformation or macrocephaly (enlargement of the head), particularly in
children[5]
Cysts in the suprasellar region in children have presented as bobbing and nodding
of the head called Bobble-Head Doll Syndrome.[5]
Cysts in the left middle cranial fossa have been associated with ADHD in a study
on affected children.[6]
Headaches.[2] While the most common symptom,[citation needed] 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.[7]
A study found 18% of patients with intracranial arachnoid cysts had nonspecific headaches. The cyst was in the temporal location in 75% of these
cases.[8]
Seizures[2]

Hydrocephalus (excessive accumulation of cerebrospinal fluid)[2]

Increased intracranial pressure[2]
Developmental delay[2]
Behavioral changes[2]
Hemiparesis (weakness or paralysis on one side of the body)[2]
Ataxia (lack of muscle control)[2]
Musical hallucination[9]
Pre-senile dementia,[10] a condition often associated with Alzheimer's disease
In elderly patients (>65 years old) symptoms were similar to chronic subdural
hematoma or normal pressure hydrocephalus:[11]
Dementia
Urinary incontinence
Hemiparesis
Headache
Seizures

Location-specific symptoms
The following list of location-specific symptoms should be interpreted in the
context of what they represent: results from several independent, unrelated
studies. As of September 2006
, no published research comprehensively maps physical and neuropsychiatric symptoms
to a specific arachnoid cyst location.[10]
A supratentorial arachnoid cyst can mimic a Mnire's disease attack.[12]
Frontal arachnoid cysts have been associated with depression.[13]
Cysts on the left temporal lobe have been associated with psychosis.[14] [15] A left
fronto-temporal cyst more specifically showed symptoms of alexithymia.[16]
Cyst on the right sylvian fissure resulted in new onset of schizophrenia-like
symptoms at age 61.[17]
A patient with a cyst on the left middle cranial fossa had auditory hallucinations,
migraine-like headaches, and periodic paranoia[8]
Patients with left temporal lobe cysts had mood disturbances similar to manic
depression (bipolar disorder)[18]

Causes
The exact cause of arachnoid cysts is not known. Researchers believe that most cases of
arachnoid cysts are developmental malformations that arise from the unexplained
splitting or tearing of the arachnoid membrane. According to the medical literature, cases
of arachnoid cysts have run in families (familial cases) suggesting that a genetic
predisposition may play a role in the development of arachnoid cysts in some individuals.
In some cases, arachnoid cysts occurring in the middle fossa are accompanied by
underdevelopment (hypoplasia) or compression of the temporal lobe. The exact role that

temporal lobe abnormalities play in the development of middle fossa arachnoid cysts is
unknown.
In a few rare cases, intracranial arachnoid cysts may be inherited as an autosomal
recessive trait. In recessive disorders, the condition does not appear unless a person
inherits the same defective gene for the same trait from each parent. If an individual
receives one normal gene and one gene for the disease, the person will be a carrier for the
disease, but usually will not show symptoms. The risk of transmitting the disease to the
children of a couple, both of whom are carriers for a recessive disorder, is 25 percent.
Fifty percent of their children risk being carriers of the disease, but generally will not
show symptoms of the disorder. Twenty-five percent of their children may receive both
normal genes, one from each parent, and will be genetically normal (for that particular
trait). The risk is the same for each pregnancy.
In a few rare cases, spinal intradural arachnoid cysts may be inherited as an autosomal
dominant trait..
Some complications of arachnoid cysts can occur when a cyst is damaged because of
minor head trauma. Trauma can cause the fluid within a cyst to leak into other areas (e.g.,
subarachnoid space). Blood vessels on the surface of a cyst may tear and bleed into the
cyst (intracystic hemorrhage), increasing its size. If a blood vessel bleeds on the outside
of a cyst, a collection of blood (hematoma) may result. In the cases of intracystic
hemorrhage and hematoma, the individual may have symptoms of increased pressure
within the cranium and signs of compression of nearby nerve (neural) tissue.
Arachnoid cysts can also occur secondary to other disorders such as Marfans syndrome,
arachnoiditis, or agenesis of the corpus callosum. (For more information on these
disorders, choose the specific disorder name as your search term in the Rare Disease
Database.)[3]

Diagnosis
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 may 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.[10] Myelograms are contraindicated for
people with arachnoid cysts.

Treatment/Management

Treatment for arachnoid cysts occurs when symptoms present themselves.[2] A variety of
procedures may be used to decompress (remove pressure from) the cyst.
Surgical placement of a cerebral shunt:[19]
An internal shunt drains into the subdural compartment.[20]
A cystoperitoneal shunt drains to the peritoneal cavity.[citation needed]
Fenestration:
Craniotomy with excision[citation needed]
Various endoscopic techniques are proving effective,[21] including laserassisted techniques.[22]
Drainage by needle aspiration or burr hole. While these procedures are relatively
simple, there is a high incidence of recurrence.[citation needed]
Capsular resection[11]
Pharmacological treatments may address specific symptoms such as seizures or
pain.
A 1994 study found surgery necessary for good outcome in patients >65 years old when
the cysts began displaying symptoms.[23]

Prognosis
Untreated, arachnoid cysts may cause permanent severe neurological damage due to the
progressive expansion of the cyst(s) or hemorrhage (bleeding).[2] With treatment most
individuals with arachnoid cysts do well with the double edge sword of developing
surgical arachnoiditis which can cause its own specific problems and can lead to
"popping" another cyst.
More specific prognoses are listed below:

Patients with arachnoid cysts of the left temporal fossa who experienced impaired
preoperative cognition had postoperative improvement.[24]
Surgery can resolve psychiatric manifestations in selected cases.[25]

Epidemiology
Arachnoid cysts are seen in 4% of the population.[26] Only 20% of these have symptoms,
usually from secondary hydrocephalus.[26]
A study that looked at 2,536 healthy young males found a prevalence of 1.7% (95% CI
1.2 to 2.3%). Only a small percentage of the detected abnormalities require urgent
medical attention.[27]