Cranial

Its Symptoms, Diagnosis And Treatment
Hydrocephalus occurs when there is an excess accumulation of cerebrospinal fluid (CSF) in the brain. It can be caused by a variety of disorders affecting the brain and spinal cord. If the cause is idiopathic, meaning that it cannot be identified, the condition is commonly known as Normal Pressure Hydrocephalus (NPH). Fortunately, NPH can often be treated effectively once a diagnosis has been made. Symptoms of NPH often resemble those of Parkinson’s Disease and/or Alzheimer’s Disease, including:

Gait Disturbances
Gait disturbances range in severity, from mild imbalance to the inability to stand or even to walk at all. For many patients, walking is wide-based, short, slow and shuffling. Patients often have trouble picking up their feet, resulting in frequent falls and difficulty climbing stairs or maneuvering curbs. Gait disturbance is often the most pronounced symptom of NPH and the first to become apparent.

Forgetfulness Or Mild Dementia
Mild dementia is described as a loss of interest in daily activities, forgetfulness, difficulty dealing with routine tasks and short-term memory loss. Patients do not usually lose language skills. Mental impairments may be quite subtle and patients themselves may not be aware of their difficulties.

Urinary Incontinence
Impairment of bladder control is usually characterized by urinary frequency and urgency. A complete loss of bladder control (urinary incontinence) can occur in more severe cases of NPH. In very rare instances, bowel incontinence may occur. Some patients, on the other hand, never display signs of bladder or bowel problems.

Because these three symptoms can be associated with the aging process in general, and a majority of the NPH population is older than 60 years, people often assume that they must live with these problems, an attitude that can lead to delays of months or even years before patients consult their physicians. NPH is a degenerative disease with a variable rate of progress, and while the earlier the diagnosis, the better the chance for successful treatment, some who have experienced symptoms for years can still improve with treatment.

Diagnosis
If NPH or another form of Adult Hydrocephalus is suspected, the next step is to order a brain imaging study, such as a CT or MRI scan. If the results indicate the need for further evaluation, the Adult Hydrocephalus Specialty Clinic can help. Our specialists review the patient’s history, physical findings, and imaging studies and make recommendations regarding surgery, further testing, or specialized diagnostic studies. These other studies may include a spinal tap (LP), a lumbar infusion study or a lumbar drainage study, and are used to help determine if surgery is indicated.

Surgical Treatment
Hydrocephalus in adults is treated by the surgical placement of a CSF drainage device known as a CSF shunt. This device, which is valve regulated, is designed to drain the excess CSF which has accumulated in the brain to another part of the body where it can be reabsorbed into the bloodstream.

The surgical procedure to implant a CSF shunt usually requires less than an hour in the operating room. After the patient is placed under general anesthesia, incisions are then made on the head and in the abdomen to allow the neurosurgeon to pass the shunt’s tubing through the fatty tissue just under the skin. A small hole is made in the skull, opening the membranes between the skull and brain to allow the ventricular end of the shunt to be passed through the brain and into the lateral ventricle. The abdominal (peritoneal) end is passed into the abdominal cavity through a small opening in the lining of the abdomen where the excess CSF will eventually be absorbed. The incisions are then closed and sterile bandages are applied.

Who Is A Likely Candidate For CSF Shunt Surgery?
Many tests and criteria have been proposed to determine which patients are best for shunting, but unfortunately, no one single factor is reliable in predicting success. The following findings are generally associated with a better prognosis:

Pituitary Tumors
In the United States, pituitary tumors represent 10% to 15% of all intracranial tumors diagnosed each year. These benign lesions originate from the cells of the pituitary gland, which is located in the sella turcica, a rounded, bony structure behind the nose and ethmoid sinus of the face. A normal pituitary gland serves the vital function of producing hormones that interact with other parts of the body, such as the thyroid and adrenal glands, to regulate hormonal output. Pituitary tumors can disrupt the output of hormones and are broadly categorized as either secreting or nonsecreting tumors.

Pituitary Tumors: Their Symptoms & Treatment
Symptomatic pituitary tumors are uncommon. While more than 99% of pituitary tumors are benign, a pituitary tumor can cause problems due to its size, its production of excessive hormones, or because it causes the pituitary gland to become underactive. Because pituitary tumors can have such a wide range of effects on the body, they often present with what seem to be a long list of unrelated symptoms. 

Effects of pituitary tumors vary depending upon the type of tumor, its size, and what area of the pituitary gland is affected. In addition to the the symptoms listed above, specific types of tumors cause other changes in body function as a result of excessive hormone secretion from the tumor.

Prolactinoma 
This is the most common type of pituitary tumor and is associated with the secretion of excess prolactin. In men this tumor most commonly causes loss of sexual function, infertility, and enlargement of the breasts. Women who have not reached menopause may experience lactation, a change in menstrual periods, or loss of menses or infertility. Headache and loss of vision may be the first indicator of a prolactinoma for menopausal women. 

Acromegaly 
These pituitary tumors are associated with excessive growth hormone secretion. The most common symptoms are excessive sweating, and enlargement of the hands, feet and face. Other problems include joint pains, sleep apnea, hypertension, diabetes mellitus, colon polyps, change in teeth spacing, oily skin and acne. 

Cushing's Disease 
This tumor produces an excessive amount of the hormone ACTH, leading to overproduction of cortisol by the adrenal glands. The result is weight gain (particularly in the abdomen and neck), loss of muscle mass in the legs and arms, muscle weakness, depression, thinning of the skin with easy bruising, hypertension, diabetes mellitus, osteoporosis with a risk for bone fractures, and weakening of the immune system. 

TSH Secreting Tumor
The least common type of hormone-producing pituitary tumor, the excessive TSH causes hyperthyroidism. Symptoms include weight loss, nervousness, rapid heartbeat, difficulty sleeping, frequent bowel movements, and scarce menstrual periods for women. 

Non-Secretory Tumor
These pituitary tumors do not produce an excessive amount of a pituitary hormone and are usually detected after they become large, causing loss of vision and/or headache and/or hypothyroidism or adrenal insufficiency. Non Secretory tumors most commonly cause sexual dysfunction in men and loss of regular menses in premenopausal women. 

Craniopharyngioma/Rathke’s Cleft Cyst 
These tumors are congenital and may cause hypopituitarism and/or diabetes insipidus. 

Pituitary Cyst 
The most common symptoms of this condition are headache and, if the cyst is large, loss of vision and loss of normal pituitary function. 

Diagnosis & Treatment 
In addition to a complete medical history and medical examination, the first step to diagnosing the presence of a pituitary tumor is the measurement of hormone levels in the blood. CT and MRI can then confirm the exact location and size of the tumor. 

Treatment can include the use of bromocriptine, hormone-replacement therapy, radiation therapy, and surgery. For many patients, surgical removal of the tumor can result in dramatic and immediate relief from some symptoms. Typically, surgical care does not require a craniotomy. Instead, the preferred approach involves a transsphenoidal route (through the nose and across the sphenoid sinus of the face), resulting in no noticeable facial scar. The surgery involves some moderate facial discomfort, but is extremely well tolerated. Best of all, the entire process usually requires only a few days of hospitalization for the patient.

Effective Treatment Options For Trigeminal Neuralgia
Trigeminal Neuralgia or Tic Douloureux is a disorder of the fifth cranial (trigeminal) nerve that causes episodes of intense, stabbing, electric shock-like pain in the areas of the face where the branches of the nerve are distributed - lips, eyes, nose, scalp, forehead, upper jaw, and lower jaw. The disorder most often affects one side of the face, but some patients experience pain at different times on both sides. Although trigeminal neuralgia is not a common disorder with an annual incidence of 4-6 in every 100,000 people, it is the most common form of neurologic facial pain in the geriatric population. Women also seem more likely to suffer from this condition.

Early in their disease course, patients tend to experience spontaneous remissions with pain-free intervals lasting weeks to months. Over time, painful episodes often become more frequent and remissions are fewer. Routine activities of daily living, such as brushing teeth, chewing, swallowing, or simply touching the skin of the face can bring on pain, causing patients to avoid these behaviors.

Diagnosis
The diagnosis of trigeminal neuralgia is made almost exclusively on the patient’s history. Neurologic exam is normal in most patients. Although imaging studies are generally normal, patients with this disorder should have magnetic resonance imaging performed because of the small incidence of associated tumors and demyelinating disease.

Treatment
Patients newly diagnosed with trigeminal neuralgia are typically treated medically. Tegretol has been the mainstay of medical treatment and approximately 70-80% of patients experience an initial therapeutic response to this medicine. The response rate to carbamazepine is typically so high that some clinicians will question the diagnosis if patients see no benefit. Up to 30% of patients will experience significant side effects related to initial therapy with carbamazepine including sedation, dizziness and nystagmus. Other medicines, including Neurontin, Elavil, Dilantin, and Baclofen have been used in the treatment of this disease with varying degrees of success.

Several authors have observed that the relief provided by Tegretol and other drugs tends to decrease over time. Additionally, medicine side effects often become more common in patients with persistent symptoms. It is estimated that up to 75% of patients fail to achieve long-term relief of their symptoms with medical therapy alone and about half of all patients with trigeminal neuralgia ultimately require an operation for pain relief. Surgical therapy for trigeminal neuralgia is reserved for cases that do not respond to medical management or when side effects of medicines become intolerable. 

Surgical Options
Three major forms of surgical therapy are presently indicated for patients with medically refactory trigeminal neuralgia. These include percutaneous attempts to destroy small portions of the trigeminal nerve, stereotactic radiosurgery and microvascular decompression of the fifth cranial nerve.

Percutaneous Techniques are well-tolerated outpatient procedures. Techniques used by Carolina Neurosurgery & Spine Associates for percutaneous rhizolysis (disruption) of the trigeminal nerve include injection of a drug (glycerol) into the trigeminal cistern or the use of heat energy (radiofrequency lesioning) in the area adjacent to the trigeminal ganglion. Both approaches result in the destruction of a small portion of the trigeminal nerve fibers and are associated with excellent initial responses, with 90-95% of patients experiencing relief of their pain. In large series, eight month pain-free rates are reported in the 60-70% range. The major downside of these minimally invasive procedures is that recurrence rates tend to be higher than with more invasive surgeries, such as microvascular decompression, and patients sometimes require repeat procedures. Because these procedures are inherently destructive, they are associated with a higher risk of sensory dysfunction. For these reasons, we generally reserve percutaneous techniques for patients with advanced age, poor medical condition or those who prefer a minimally invasive procedure.

Stereotactic Radiosurgery has recently been used to treat trigeminal neuralgia. The technique shows promise and some series have reported excellent response rates in the 80-90% range. Unfortunately, therapeutic responses may take weeks to occur in most patients. Additionally, there is very little follow-up with this technique compared to the other procedures. Because of this and because this also is a destructive procedure, physicians from our group have preferred to reserve this therapy for patients who are of advanced age or in poor medical condition. This technique may become a very important means of treating trigeminal neuralgia in the future.

Microvascular Decompression remains the gold standard by which all other procedures have been judged. Microvascular decompression involves a suboccipital craniotomy for exploration of the root entry zone of the fifth cranial nerve. Patients typically are hospitalized for a 2-3 day period. Despite the invasiveness of the procedure, microvascular decompression tends to be extremely well tolerated and very low morbidity rates have been reported in a variety of series, including our own. Sustained response rates in the 85 to 90% range are not uncommon with this procedure.