Unraveling Neurological Mysteries

Neurological diseases can hit any one at any time, sometimes with little warning. Here’s what you need to know about diagnosing and treating disorders of the nervous system.

They often strike like lightning. Predicting when, where and to whom a neurological disease will hit is nearly impossible.
And while they can be loosely grouped as “genetic” or “sporadic,” the occurence of nervous system disorders may also be influenced by the environment and climate.
Among the thousands of disorders associated with the body’s nervous system are common ones such as pinched nerves, carpal tunnel syndrome, backaches, migraine headaches, seizures and dementias. Less common are multiple sclerosis (MS), muscular dystrophy (MD), epilepsy, Parkinson’s disease, Huntington’s disease and amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig’s disease).
By comparing a home’s electrical system to the human nervous system, we begin to understand what happens when a neurological disease strikes. The master switch box (brain) is the source of electrical power that flows through conduits to every part of the house. When a fuse blows, wires cross, or the coating on electrical wires wears thin, short-circuits occur. Similar events happen within the body, but are much more difficult to pinpoint, because neurological symptoms are easily confused with other problems.
When patients report to their primary doctors symptoms such as weakness, numbness, tremors, headache, double vision, back pain, seizures, balance or memory problems, they’re often referred to neurologists for a further workup.
At SwedishAmerican Hospital, the examination is pretty straightforward, says Dr. Mohammed Afzal, a board-certified neurologist.
“It’s basically a matter of one plus one equaling two,” he explains. “A good clinical neurological examination can diagnose most of the neurological disorders more than 90 percent of the time, and then other investigations confirm the diagnosis.”
Untreatable neurological diseases, such as Huntington’s, are relatively uncommon. Afzal believes there are about 120 diagnosed cases in the greater Rockford area. Huntington’s is unusual in that it’s specifically inherited, and with each generation, its onset occurs at an earlier and earlier age. On the plus side, patients can be tested for the trait before symptoms develop, as with breast cancer and other genetic diseases.
“The only way to prevent the spread of Huntington’s is through genetic counseling,” says Dr. Sulaiman Mohammad, a board-certified neurologist at KSB Hospital, Dixon. “Families are tested for the gene to determine risks, so they can decide whether or not to have children. While there are trials being conducted on several levels at this time, we still don’t know why Huntington’s occurs.”
Some neurological diseases offer more clues to their development.
“Multiple sclerosis [MS] occurs in a higher density in some areas and populations,” Mohammad explains. “When outsiders move into the region, the incidence of MS increases with each generation, leading us to suspect environment may play a role. Among Ashkenazi Jews in Israel, however, MS appears to be a genetic disease.”
According to the Multiple Sclerosis Association of America, MS is the most common neurological disorder diagnosed in people ages 15 to 50. About 400,000 Americans have MS, and about 10,000 new cases are diagnosed each year. Its occurrence is not entirely random, Mohammad points out. Women are three times more likely to develop MS than men. Populations living at least 40 degrees north or south of the equator are more likely to see cases of MS than people living in warmer climates, another tantalizing hint that may help researchers to unravel this mysterious disease.
By contrast, ALS appears to be familial in younger patients, while older patients develop the disease without any traceable source. Because there’s really no way to pinpoint how or why neurological diseases start, it’s nearly impossible to prevent them. According to the ALS Association, the disease is directly hereditary in only a small percentage of families. Most patients with adult-onset ALS – about 90 percent – have no family history and present as isolated cases. This is where the term “sporadic” is applied. The remaining 10 percent have a close family member with ALS.
“ALS studies conducted recently seem to indicate that an enzyme within the body called superoxide dismutase [SOD1] is present in about 20 percent of familial ALS patients,” Mohammad says. “While the presence of SOD1 is a predictor, it still doesn’t tell us if the patient will develop ALS or when.”
Most neurodegenerative disorders, including dementia and Parkinson’s disease, are addressed by treating symptoms, either with medication or surgery. The goal is to give patients relief, while working to slow the progress of the disease.
Doctors carefully consider a patient’s ability to function and his or her cognitive capabilities when designing a treatment plan. At SwedishAmerican, Afzal works with a team of specialists that may include neurosurgeons, physical therapists, psychiatrists and other professionals relevant to a specific case.
The team takes a multiple-level approach when addressing each patient’s case. For example, says Afzal, Parkinson’s disease first may be treated with medications and physical therapy to improve a patient’s balance, but later may be treated with a surgically implanted deep-brain stimulator that helps to control tremors. Exactly how much good this procedure does will depend upon the specific patient.
At Monroe Clinic, Dr. Kara Tower, DO, a neurology specialist, describes deep brain stimulation as one of the more recent breakthroughs in the treatment of Parkinson’s disease and essential tremors. “It’s becoming more popular to further control tremors and other involuntary movements in place of, or to limit, medications, which can have serious side effects,” she explains. “Depending on the patient, the stimulator is typically implanted in the globus pallidus or subthalamic nucleus. Stimulation focuses on the opposite side of the brain from the problem areas. Some patients require stimulation on both sides, depending on their individual symptoms.”
When an electrode is surgically implanted in the brain, a wire is placed beneath the skin from the brain to the chest, where a device similar to a pacemaker also is implanted. This procedure is conducted by skilled neurosurgeons and neurological clinicians, working as a team.“Once the stimulator and controlling device are in place, the patient is monitored, either at bedside in the hospital or at home,” Tower says. “The level of stimulation can be programmed through a hand-held device that reads and can adjust the internal control. It retrieves information and helps the neurologist make adjustments, as well as turns the control device on and off. In cases when the patient needs both sides of the brain stimulated, separate electrodes and devices are implanted.”
Neurological disorders naturally affect muscles as nerves pass through them. Dr. Javaid Iqbal is a board-certified neurologist and neuromuscular diseases specialist in the Illinois Neurological Institute at OSF Saint Anthony Medical Center in Rockford. He says neuromuscular diseases include disorders which affect motor nerve cells in the brain and spinal cord, nerve roots inside the spinal column, brachial and lumbosacral plexuses, peripheral nerves, sites where nerve fibers and muscles meet, and muscles themselves.
Common neuromuscular diseases include ALS, pinched nerves inside the spinal column, peripheral neuropathies, entrapment neuropathies like carpal tunnel syndrome, ulnar neuropathy, myasthenia gravis, muscular dystrophies, polymyositis (inflammation of muscles), among many dozens of other muscle disorders.
Clinical symptoms of neuromuscular disorders may include numbness, tingling, burning sensations in the arms or legs, muscle fatigue, muscle atrophy, cramps or spasms, twitching, droopy eyelids, double vision and fluctuating weaknesses that cause difficulty swallowing or chewing, and lead to choking spells.
Pain in the neck, back or other spinal areas is also associated with neuromuscular disorders.
While no treatment exists for some neuromuscular disorders, many can be treated adequately and effectively, including inflammatory forms of peripheral neuropathies like Guillain-Barre syndrome, fibromyalgia and multifocal motor neuropathy.
Pinched nerves in the spine and limbs can be treated surgically.
Myasthenia gravis can be very adequately controlled with drugs and surgery (such as thymus removal), as can inflammatory and metabolic muscle diseases.
Sadly, no curative treatments are available yet for ALS or muscular dystrophies, but much can be done to ease a patient’s suffering and improve quality of life, with physical and speech therapy, orthopedic devices, counseling, surgery and medications.
Iqbal points out that in about 80 percent of patients, epilepsy can be successfully controlled with newer medications and surgical procedures. “This is especially true of the newer drugs,” he says.
More radical-sounding treatments may actually cure epilepsy in very young children suffering from uncontrollable seizures. On rare occasions, it’s possible to remove almost half of a very young child’s brain, in cases when he or she is experiencing severe seizure episodes that can’t be controlled by medications, says Tower at Monroe Clinic. This procedure is performed only at major epilepsy centers, and not in the greater Rockford region.
“In certain cases, if children do not have this procedure, they may be more likely to spend the rest of their lives affected with possible cognitive and physical impairments secondary to the effects of the uncontrolled epilepsy,” Tower explains. “At that early age, it is entirely possible for children to significantly rebuild and restore major parts of their brain function. Of course, with adults, you can’t do that radical of an operation on them. They just can’t recover.”
However, it’s still possible for adult epilepsy patients to benefit from some surgeries. For example, neurosurgeons can sometimes remove a tiny piece of the brain where the initial seizure impulse is located, which may result in partial to total recovery.
“Not only is this procedure effective for a select population of patients with medication-resistant epilepsy, but it often reduces or even eliminates the need for medications that can have serious side effects,” Tower says.
KSB’s Mohammad emphasizes that the best possible way to treat any neurological disease is to recognize early symptoms and get help immediately.
“Don’t take symptoms such as persistent headache, tremors or other non-normal feelings lightly,” he cautions.
Efforts are well underway to find treatments for frustrating degenerative disorders like muscular dystrophies, hereditary ataxias, MS, epilepsy, Parkinson’s disease, Huntington’s disease, ALS, Alzheimer’s disease and other dementias.
Meanwhile, doctors in our region are dedicated to alleviating pain and improving quality of life for such patients, says Iqbal at OSF. He and his peers are hopeful that the medical community soon will be able to offer more.
He says, “With the ongoing research in every field, we have reason to believe that breakthroughs and newer novel therapies will emerge in the near future.”

A Closer Look At …

Amyotrophic Lateral Sclerosis (ALS)

Often referred to as “Lou Gehrig’s Disease,” this progressive neurodegenerative disease attacks nerve cells in the brain and spinal cord. When the motor neurons die, the brain can no longer initiate and control muscle movement.
Early symptoms often include increasing muscle weakness in the arms and legs or when talking, swallowing or breathing. When muscles no longer receive messages from motor neurons, they begin to atrophy (become smaller).
Source: ALS Association, alsa.org

Alzheimer’s Disease

With this disorder, certain types of nerve cells in particular areas of the brain degenerate and die for unknown reasons. It accounts for 50 to 80 percent of all dementia cases.
Warning signs may include memory loss that disrupts daily life; difficulty in planning, problem solving or completing familiar tasks; confusion with time or place; trouble understanding visual images and spatial relationships; new problems with words in speaking or writing; misplacing things and inability to retrace steps; decreased or poor judgment; withdrawal from work or social activities; and changes in mood or personality.
Source: Alzheimer’s Association, alz.org

Huntington’s Disease

This devastating degenerative brain disorder is hereditary and slowly diminishes the patient’s ability to walk, talk and reason.Early symptoms may include changes in cognitive ability or mobility and/or depression, mood swings, forgetfulness, clumsiness, involuntary twitching and lack of coordination.
As the disease progresses, concentration and short-term memory diminish and involuntary movements of the head, trunk and limbs increase. There is no cure for HD and only one FDA-approved treatment drug. However, since the discovery of the gene that causes HD, scientific research has accelerated.
Source: Huntington’s Disease Society of America

Muscular Dystrophies (MD)

This refers to more than 30 genetic diseases characterized by progressive weakness and degeneration of the skeletal muscles that control movement. They differ in terms of distribution and extent of muscle weakness (some forms of MD also affect cardiac muscle), age of onset, rate of progression, and pattern of inheritance.
Duchenne MD is the most common form and primarily affects boys. It’s caused by the absence of dystrophin, a protein involved in maintaining the integrity of muscle.
Source: National Institute of Neurological Disorders and Stroke, hih.gov

Multiple Sclerosis (MS)

This is a chronic, often disabling disease that damages the myelin (the fatty sheath that surrounds and insulates nerve fibers) and nerve fibers in the central nervous system. This interferes with the transmission of nerve signals between the brain and spinal cord and other parts of the body.
Symptoms may be mild, such as numbness in the limbs, or severe, such as paralysis or loss of vision.
Source: National Multiple Sclerosis Society, nationalmssociety.org

Parkinson’s Disease (PD)

This motor system disorder results from the loss of dopamine-producing brain cells. The four primary symptoms are tremor or trembling in hands, arms, legs, jaw, and face; rigidity or stiffness of the limbs and trunk; bradykinesia, or slowness of movement; and impaired balance and coordination. As these symptoms become more pronounced, patients may have difficulty walking, talking, or completing other simple tasks.
Sources: National Parkinson Foundation, Parkinson.org; National Institute of Neurological Disorders and Stroke, nih.gov