Monthly Archives: June 2021

Acute Disseminated Encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is characterized by a brief but intense attack of inflammation in the brain and spinal cord that damages myelin – the protective covering of nerve fibers. It often follows viral infection, or less often, vaccination for measles, mumps, or rubella.

The incidence rate of ADEM is about 8 per 1,000,000 people per year. Children are more likely than adults to have ADEM, although it occurs in all ages. The average age of onset is around 5 to 8 years old.

The symptoms of ADEM come on quickly, beginning with encephalitis-like symptoms such as:

It may also damage white matter (brain tissue that takes its name from the white color of myelin), leading to neurological symptoms such as:

  • Visual loss in one or both eyes – due to inflammation of the optic nerve
  • Weakness even to the point of paralysis
  • Difficulty coordinating voluntary muscle movements (such as those used in walking)

ADEM is sometimes misdiagnosed as a severe first attack of multiple sclerosis (MS), since some of the symptoms of the two disorders, particularly those caused by white matter injury, may be similar. However, ADEM usually has symptoms of encephalitis, as well as symptoms of myelin damage, as opposed to MS, which doesn’t have encephalitis symptoms. In addition, ADEM usually consists of a single episode or attack, while MS features many attacks over the course of time. Doctors will often use imaging techniques, such as MRI (magnetic resonance imaging), to search for old and new lesions (areas of damage) on the brain. Old “inactive” brain lesions on MRI suggest that the condition may be MS rather than ADEM, since MS often causes brain lesions before symptoms become obvious. In rare situations, brain biopsy may show findings that allow differentiation between ADEM and severe, acute forms of MS.

Treatment for ADEM is targeted at suppressing inflammation in the brain using anti-inflammatory drugs. Most individuals respond to intravenous corticosteroids such as methylprednisolone. When corticosteroids fail to work, plasmapheresis or intravenous immunoglobulin therapy has been shown to produce improvement. Additional treatment is symptomatic and supportive.

Corticosteroid therapy can shorten the duration of neurological symptoms and halt further progression of the disease in the short term, but the long term prognosis for individuals with ADEM varies. Full recovery is seen in 50 to 75% of cases, while up to 70 to 90% recover with some minor residual disability. The average time to recover is one to six months. Severe cases of ADEM can be fatal. Some individuals who initially diagnosed as having ADEM will later be reclassified as MS, but there is currently no method to determine whom those individuals will be.

Tourette Syndrome

Tourette syndrome (TS) is a neurological disorder characterized by repetitive, stereotyped, involuntary movements and vocalizations called tics.

The first symptoms of TS are almost always noticed in childhood.

Between 1 and 10 children per 1,000 have Tourette’s and as many as 10 per 1,000 people may have tic disorders.

Some of the more common tics include:

  • Eye blinking and other vision irregularities
  • Facial grimacing
  • Shoulder shrugging
  • Head or shoulder jerking

Perhaps the most dramatic and disabling tics are those that result in self-harm such as:

  • Punching oneself in the face
  • Vocal tics
    • Coprolalia – uttering swear words
    • Echolalia – repeating the words or phrases of others

Many with TS experience additional neurobehavioral problems including:

  • Inattention
  • Hyperactivity
  • Impulsivity
  • Obsessive-compulsive symptoms
    • Intrusive thoughts/worries
    • Repetitive behaviors

Although TS can be a chronic condition with symptoms lasting a lifetime, most people with the condition experience their worst symptoms in their early teens, with improvement occurring in the late teens and continuing into adulthood. As a result, some individuals may actually become symptom free or no longer need medication for tic suppression.

Because tic symptoms do not often cause impairment, the majority of people with TS require no medication for tic suppression. However, effective medications are available for those whose symptoms interfere with functioning. There is no one medication that is helpful to all people with TS, nor does any medication completely eliminate symptoms. Effective medications are also available to treat some of the associated neurobehavioral disorders that can occur in patients with TS.

Seasonal Affective Disorder

Seasonal affective disorder (SAD) is a form of depression that occurs during specific seasons, most commonly during the fall and winter. However, the condition can occur at any time of the year, including during the summer. People who get the mood disorder have normal mental health throughout most of the year and experience depressive symptoms around the same time repeatedly, year after year.

The incidence of the condition varies with geography. For example, it tends to be more common in the northern or polar regions.

The cause of SAD is not yet known, but it’s believed to be related to the availability of sunlight. Light affects your internal body clock, which helps you regulate when to sleep and when to be awake. Some scientists believe that a fluctuation in the body’s production of melatonin, a hormone that helps induce sleep, might be the cause of SAD. Other researchers speculate that a lack of serotonin, a brain chemical or neurotransmitter that seems to be triggered by sunlight, is the cause of SAD. People who are depressed often have decreased levels of serotonin in their brains.

Common symptoms of SAD include:

  • Excessive sleep
  • Inactivity and low energy levels
  • Negative feelings and depression
  • Increased appetite
  • Weight gain
  • Headaches
  • Anxiety
  • Self-imposed isolation

If you are diagnosed with seasonal affective disorder (SAD), your doctor may choose one of several approaches to your treatment.

  • Light Therapy — Short periods of exposure to light can help ease depression. Doses of sunlight are measured in “lux.” For example, the sun emits about 90,000 lux and blue sky reflects about 45,000 lux. Treatments could range from two hours of light at 2500 lux every morning to 30 to 40 minutes of light at 10,000 lux every morning. However, light therapy in the evening may interrupt sleep patterns.There are few, if any, side effects to the eyes from using light therapy. Sometimes, an hour walk in the morning can help without any other treatment.
  • Medication — Your doctor may prescribe an antidepressant in combination with light therapy or if light therapy isn’t effective. Antidepressants often are used when the condition occurs in the summer.
  • Psychotherapy — Psychotherapy may help you identify ways to avoid behaviors or environments that tend to trigger episodes of SAD, or to reduce stress in your life, which may worsen the symptoms.
  • Carefully timed supplementation of the hormone melatonin.

In addition to the treatments described above, there are a number of things you can do to help your body cope with seasonal depression. For example, try increasing the amount of light in your home or workplace by opening window shades. Increase your physical activity as exercising regularly can help relieve stress. Also, you may want to consider visiting a warm, sunny place during the winter, such as vacationing in a tropical location.

Klüver-Bucy Syndrome

Klüver-Bucy syndrome is a rare behavioral impairment that is associated with damage to both of the anterior temporal lobes of the brain. The amygdala has been a particularly implicated brain region in the pathogenesis of this syndrome.

The disorder may be associated with herpes encephalitis and trauma, which can result in brain damage. Other conditions may also contribute to a diagnosis of Klüver-Bucy syndrome, including:

  • Alzheimer’s Disease
  • Ischemia
  • Anoxia
  • Progressive subcortical gliosis
  • Rett syndrome
  • Porphyria
  • Carbon monoxide poisoning

It causes individuals to put objects in their mouths and engage in inappropriate sexual behavior. Other symptoms may include:

  • Visual agnosia (inability to visually recognize objects)
  • Loss of normal fear and anger responses
  • Memory loss
  • Distractibility
  • Seizures
  • Dementia

It is rare for humans to manifest all of the identified symptoms of the syndrome; three or more are required for diagnosis.

There is no cure for Klüver-Bucy syndrome. The disorder is not life-threatening, but the patient can be difficult to manage. With treatment, symptoms may slowly decline.

Treatment is symptomatic and supportive, and may include drug therapy.

Depression

Depression is an illness that causes you to feel sad, to lose interest in activities that you’ve always enjoyed, to withdraw from others, and to have little energy. It’s different from normal feelings of sadness, grief, or low energy. Depression can also cause people to feel hopeless about the future and even to think about suicide.

Many people, and sometimes their families, feel embarrassed or ashamed about having depression. Don’t let these feelings stand in the way of getting treatment. Remember that depression is a common illness. Depression affects the young and old, men and women, all ethnic groups, and all professions.

If you think you may be depressed, tell your doctor. Treatment can help you enjoy life again. The sooner you get treatment, the sooner you will feel better.

Depression is a disease. It’s not caused by personal weakness and is not a character flaw. When you have depression, chemicals in your brain called neurotransmitters are out of balance. Most experts believe a combination of family history and stressful life events may cause depression.

Life events can include:

  • Childbirth, a death in the family, work, or relationships.
  • Finding out you have a long-term health problem, such as arthritis, heart disease, or cancer.
  • Health problems, such as anemia and an underactive thyroid gland (hypothyroidism).

Treating the health problem can usually cure the depression.

Just because you have a family member with depression or have stressful life events doesn’t mean you’ll get depression. You also may get depressed even if there is no reason you can think of.

The symptoms of depression may be hard to notice at first. They vary among people, and you may confuse them with just feeling “off” or with another health problem.

The two most common symptoms of depression are:

  • Feeling sad or hopeless nearly every day for at least 2 weeks.
  • Losing interest in or not getting pleasure from most daily activities nearly every day for at least 2 weeks.

A serious symptom of depression is thinking about death or suicide. If you or someone you care about talks about this or feeling hopeless, get help right away.

You also may:

  • Lose or gain weight. You also may feel like eating more or less than usual almost every day.
  • Sleep too much or not enough almost every day.
  • Feel restless and not be able to sit still, or you may sit quietly and feel that moving takes great effort. Others can easily see this behavior.
  • Feel tired or as if you have no energy almost every day.
  • Feel unworthy or guilty nearly every day. You may have low self-esteem and worry that people don’t like you.
  • Find it hard to focus, remember things, or make decisions nearly every day. You may feel anxious about things.

If you have some of these symptoms for at least 2 weeks, talk to your doctor. Treatment may be right for you.

Depression can be treated in various ways. Counseling, psychotherapy, or antidepressant medicines may be used. Two or all of these treatments may also be combined. Lifestyle changes, such as getting more exercise, also may help. Your doctor or mental health professional will help you find the best treatment.

If you have mild or moderate depression, your family doctor or a mental health professional, such as a counselor or psychologist, may treat you. If you have severe depression or if treatment is not helping, you may need to see a psychiatrist. Some people need to be treated in the hospital, especially if they have thoughts of suicide.

Work with your health care team to find the best treatment for you. It may take a few tries, and it can take several weeks for the medicine to start working. Try to be patient and keep following your treatment plan.

Depression can relapse. How likely you are to get depression again increases each time you have a bout of depression. Taking your medicines and continuing some types of therapy after you feel better can help keep that from happening. Some people need to take medicine for the rest of their lives. This does not stop them from living full and happy lives.

Let your doctor know if you think you are depressed. Depression is easy to overlook. The earlier you are treated, the more quickly you will get better.

Suicide

Many people who have depression have thoughts of death or thoughts of suicide, and depression can lead to suicide. Learn the warning signs of suicide, which include:

  • Talking, writing, reading, or drawing about death, including writing suicide notes and talking about items that can harm you, such as pills, guns, or knives
  • Giving things away
  • Using a lot of alcohol or drugs or both
  • Planning to harm yourself or others
  • Buying guns or bullets, stockpile medicines, or take other action to prepare for a suicide attempt. You may have a new interest in guns or other weapons
  • Hearing or seeing things that aren’t real
  • Thinking or speaking in a bizarre way that is not like your usual behavior

If a suicide threat seems real, call 911, a suicide hotline, or the police. Stay with the person, or ask someone you trust to stay with the person, until the crisis has passed. Don’t argue or challenge the person. Tell the person you don’t want him or her to die.

Attention Deficit-Hyperactivity Disorder

Attention deficit-hyperactivity disorder (ADHD) is a neurobehavioral disorder that interferes with a person’s ability to stay on a task and to exercise age-appropriate inhibition (cognitive alone or both cognitive and behavioral).

ADHD affects 3-5 percent of all North American children. ADHD is usually diagnosed in childhood, although the condition can continue into the adult years.

Some of the warning signs of ADHD include:

  • Failure to listen to instructions
  • Inability to organize oneself and school work
  • Fidgeting with hands and feet
  • Talking too much
  • Leaving projects, chores and homework unfinished
  • Having trouble paying attention to and responding to details

There are several types of ADHD: a predominantly inattentive subtype, a predominantly hyperactive-impulsive subtype, and a combined subtype.

There is no “cure” for ADHD. Children with the disorder seldom outgrow it; however, some may find adaptive ways to accommodate the ADHD as they mature.

The usual course of treatment may include medications such as methylphenidate (Ritalin) or dextroamphetamine (Dexedrine), which are stimulants that decrease impulsivity and hyperactivity and increase attention.

Most experts agree that treatment for ADHD should address multiple aspects of the individual’s functioning and should not be limited to the use of medications alone. Treatment should include:

  • Structured classroom management
  • Parent education (to address discipline and limit-setting)
  • Tutoring
  • Behavioral therapy for the child

Vascular Dementia

One of the most common types of dementia in older adults, vascular dementia (VaD) refers to a subtle, progressive decline of memory and other cognitive function, such as thinking, learning, remembering, organization skills and complex visual processing. VaD is caused by a chronic, reduced blood flow in the brain. Normally, the brain cells receive an ample supply of blood, which is delivered through a network of blood vessels called the vascular system.

Males and African Americans are at an increased risk of developing the disease.

VaD may be caused by stroke, in which the blood supply to the brain becomes blocked, resulting in permanent brain damage. VaD caused by a single stroke is called single-infarct dementia. The condition can also be caused by a series of small, often unnoticeable strokes called multi-infarct dementia. Damage to tiny blood vessels that lie deep in the brain may also lead to a type of VaD known as sub-cortical vascular dementia. VaD may also occur with Alzheimer’s disease, which causes similar symptoms, such as memory loss.

Vascular dementia can occur suddenly or progress slowly over time. At times, people with VaD experience long periods without any noticeable changes, or even improvements. However, if another stroke occurs, their symptoms may progress rapidly.

VaD risk factors include:

  • High blood pressure
  • Heart disease
  • High cholesterol
  • Diabetes
  • Smoking
  • Alcoholism

It is important that these conditions are diagnosed and treated promptly as a way to prevent VaD.

The condition often occurs with Alzheimer’s disease, which further complicates its progression. VaD causes similar symptoms to those associated with Alzheimer’s disease (AD), such as memory loss. However, memory problems caused by VaD are usually easier to overcome with cues and reminders.

Common symptoms include:

  • Difficulty with organization and solving complex problems
  • Slowed thinking
  • Distraction or “absent mindedness”
  • Difficulty retrieving words from memory
  • Walking with rapid, shuffling steps
  • Speech difficulties
  • Difficulty following instructions

Other symptoms may include:

  • Mood and behavior changes, such as depression, irritability or apathy
  • Hallucinations or delusions, which may be very distressing to the patient and their caregiver
  • Balance and movement problems
  • Loss of bladder or bowel control
  • Symptoms of Parkinson’s disease, such as tremors

Currently, there is no cure for vascular dementia. Treatments are designed to prevent and control risk factors. Treating these conditions greatly reduces the risk of developing dementia.

Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a rare brain disorder that causes problems with walking and balance as well as dementia.

About 20,000 North Americans — or one in every 100,000 people over age 60 — have PSP. Patients with PSP are usually middle-age or elderly, and men are affected more often than women. This disease is difficult to diagnose because it is so rare and is sometimes mistaken for Parkinson’s disease.

The signs and symptoms of progressive supranuclear palsy (PSP) vary from patient to patient but loss of balance while walking is the most common first symptom. Patients may have unexplained falls or a stiffness and awkwardness when walking. Sometimes the falls are described as attacks of dizziness, which can lead to an incorrect diagnosis of an inner ear problem. In addition, with PSP speech usually becomes slurred. In rare cases, some patients will experience shaky hands.

Other common early symptoms include a loss of interest in usual hobbies or recreational activities, increased irritability and forgetfulness. Patients may suddenly laugh or cry, be apathetic or have occasional angry outbursts for no apparent reason.

As the disease progresses, many patients develop blurring of vision and problems controlling eye movements. These symptoms are caused by a gradual deterioration of brain cells at the base of the brain in an area called the brainstem. PSP patients have trouble voluntarily shifting their gaze downward and can have trouble controlling their eyelids. This can lead to involuntary closing of the eyes, prolonged or infrequent blinking, or difficulty in opening the eyes.

Another common visual problem is an inability to maintain eye contact during a conversation. This can give the mistaken impression that the patient is hostile or uninterested.

As PSP gets progressively worse, patients are at greater risk for complications, such as pneumonia, head injury and fractures caused by falls. Swallowing solid foods or liquids can be difficult and choking becomes a hazard. The most common cause of death is pneumonia. However, with good medical attention and treatment, many PSP patients live well into their 70s and beyond.

There is currently no effective treatment or cure for PSP, although some of the symptoms can respond to nonspecific measures.

Multi-Infarct Dementia

Multi-infarct dementia (MID) is a common cause of memory loss in the elderly.

MID, which typically begins between the ages of 60 and 75, affects men more often than women.

MID is caused by multiple strokes (disruption of blood flow to the brain). Disruption of blood flow leads to damaged brain tissue. Some of these strokes may occur without noticeable clinical symptoms. Doctors refer to these as “silent strokes”. An individual having a silent stroke may not even know it is happening, but over time, as more areas of the brain are damaged and more small blood vessels are blocked, the symptoms of MID begin to appear.

Symptoms include:

  • Confusion or problems with short-term memory
  • Wandering, or getting lost in familiar places
  • Walking with rapid, shuffling steps
  • Losing bladder or bowel control
  • Laughing or crying inappropriately
  • Having difficulty following instructions
  • Having problems counting money and making monetary transactions

Because the symptoms of MID are so similar to Alzheimer’s disease, it can be difficult for a doctor to make a firm diagnosis. Since the diseases often occur together, making a single diagnosis of one or the other is even more problematic. MID can be diagnosed by an MRI or CT of the brain, along with a neurological examination.

The prognosis for individuals with MID is generally poor. The symptoms of the disorder may begin suddenly, often in a step-wise pattern after each small stroke. Some people with MID may even appear to improve for short periods of time, then decline after having more silent strokes. The disorder generally takes a downward course with intermittent periods of rapid deterioration. Death may occur from stroke, heart disease, pneumonia, or other infection.

There is no treatment available to reverse brain damage that has been caused by a stroke. Treatment focuses on preventing future strokes by controlling or avoiding the diseases and medical conditions that put people at high risk for stroke:

The best treatment for MID is prevention early in life – eating a healthy diet, exercising, not smoking, moderately using alcohol, and maintaining a healthy weight.