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Monday, August 11, 2014

What Do You Know About Neurological Maladies?

I was never interested on neurological diseases until last year when my wife was diagnosed with Parkinson's disease. Since then I read as much as I could about Parkinson and other neurological disorders. Last year, I posted in my blog 12 maladies that I have heard of and somewhat have touched my life here in the US and in the Philippines. Today I am updating this posting with a rare disease that happened to a friend in 2006 here in the US. The diagnosis was enteroviral meningoencephalitis. For a brief summary of this very rare neurological disorder, see item number 13 below.


The following neurological diseases are familiar to me, since I have relatives and friends that are affected by one or more of the diseases listed as follows: The maladies are listed in alphabetical order except for number 13 which is an update. If you need additional details please visit the website cited and in the reference below for #13.

1. Alzheimer's disease (AZD), also known in medical literature as Alzheimer disease(AD). It is the most common form of dementia. Dementia is a serious loss of global cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging. There is no cure for the AZD disease, which worsens as it progresses, and eventually leads to death. It was first described by German psychiatrist and neuro pathologist Alois Alzheimer in 1906 and was named after him. Most often, AZD is diagnosed in people over 65 years of age, although the less-prevalent early-onset Alzheimer's can occur much earlier. In 2006, there were 26.6 million sufferers worldwide. Alzheimer's is predicted to affect 1 in 85 people globally by 2050. My aunt from the Philippines died of AZD a couple of years ago.
Last year during our winter sojourn in the Philippines, my wife and I met two of our college years contemporaries diagnosed with early AZ disease.

2. Asperger syndrome (AS), also known as Asperger disorder (AD) or simply Asperger's, is an autism spectrum disorder (ASD) that is characterized by significant difficulties in social interaction and nonverbal communication, alongside restricted and repetitive patterns of behavior and interests. It differs from other autism spectrum disorders by its relative preservation of linguistic and cognitive development. Although not required for diagnosis, physical clumsiness and atypical (peculiar, odd) use of language are frequently reported. The syndrome is named after the Austrian pediatrician Hans Asperger who, in 1944, studied and described children in his practice who lacked nonverbal communication skills, demonstrated limited empathy with their peers, and were physically clumsy. A teenage son of a close relative was diagnosed with AS just recently.

3. Chorea (or choreia, occasionally) is an abnormal involuntary movement disorder, one of a group of neurological disorders called dyskinesias. The term chorea is derived from the Greek word χορεία (=dance; see choreia), as the quick movements of the feet or hands are comparable to dancing. The term hemichorea refers to chorea of one side of the body, such as chorea of one arm but not both (comparable to hemiballismus). Chorea is characterized by brief, semi-directed, irregular movements that are not repetitive or rhythmic, but appear to flow from one muscle to the next. I know of a distant relative who has chorea in the Philippines.

4. Dystonia is a neurological movement disorder, in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. The disorder may be hereditary or caused by other factors such as birth-related or other physical trauma, infection, poisoning (e.g., lead poisoning) or reaction to pharmaceutical drugs, particularly neuroleptics. Treatment is difficult and has been limited to minimizing the symptoms of the disorder, since there is no cure available.
I have a neighbor in the Philippines with symptoms of Dystonia in the mid 1950's.

5. Epilepsy is a brain disorder in which a person has repeated seizures (convulsions) over time. Seizures are episodes of disturbed brain activity that cause changes in attention or behavior. I had a classmate in college who had an epileptic attack during class for one minute but recover immediately.

6. Huntington's disease (HD) is a neurodegenerative genetic disorder that affects muscle coordination and leads to cognitive decline and psychiatric problems. It typically becomes noticeable in mid-adult life. HD is the most common genetic cause of abnormal involuntary writhing movements called chorea, which is why the disease used to be called Huntington's chorea. It is much more common in people of Western European descent than in those of Asian or African ancestry. The disease can affect both men and women. The disease is caused by an autosomal dominant mutation in either of an individual's two copies of a gene called Huntingtin, which means any child of an affected person typically has a 50% chance of inheriting the disease. Physical symptoms of Huntington's disease can begin at any age from infancy to old age, but usually begin between 35 and 44 years of age. I have heard of this disease from my classmates in graduate school in Chicago, Illinois sometime in the mid 1960's.

7. Lou Gehrig's disease is a disorder that's also called amyotrophic lateral sclerosis or ALS. Amyotrophic means that the muscles have lost their nourishment. When this happens, they become smaller and weaker. Lateral means that the disease affects the sides of the spinal cord, where the nerves that nourish the muscles are located; and sclerosis means that the diseased part of the spinal cord develops hardened or scarred tissue in place of healthy nerves. ALS is often called Lou Gehrig's disease after Lou Gehrig, a hall-of-fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s. People in England and Australia call ALS motor neurone disease (MND). The French refer to it as maladie de Charcot, after the French doctor Jean-Martin Charcot, who first wrote about ALS in 1869. Lou Gehrig's disease damages motor neurons in the brain and spinal cord. Motor neurons are nerve cells that control muscle movement. Upper motor neurons send messages from the brain to the spinal cord, and lower motor neurons send messages from the spinal cord to the muscles. Motor neurons are an important part of the body's neuromuscular system. My knowledge of this disease comes from what I read in the Internet.


8. Meniere's disease
is a disorder of the inner ear. It can cause severe dizziness, a roaring sound in your ears called tinnitus, hearing loss that comes and goes and the feeling of ear pressure or pain. It usually affects just one ear. It is a common cause of hearing loss. Attacks of dizziness may come on suddenly or after a short period of tinnitus or muffled hearing. Some people have single attacks of dizziness once in a while. Others may have many attacks close together over several days. Some people with Meniere's disease have "drop attacks" during which the dizziness is so bad they lose their balance and fall. I have a couple close relatives suffering from a mild form of this malady.


9. Multiple sclerosis (MS
), also known as disseminated sclerosis or encephalomyelitis disseminata, is an inflammatory disease in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This damage disrupts the ability of parts of the nervous system to communicate, resulting in a wide range of signs and symptoms, including physical, mental, and sometimes psychiatric problems. MS takes several forms, with new symptoms either occurring in isolated attacks (relapsing forms) or building up over time (progressive forms). Between attacks, symptoms may go away completely; however, permanent neurological problems often occur, especially as the disease advances. While the cause is not clear, the underlying mechanism is thought to be either destruction by the immune system or failure of the myelin-producing cells. Proposed causes for this include genetics and environmental factors such as infections. MS is usually diagnosed based on the presenting signs and symptoms and the results of supporting medical tests. There is no known cure for multiple sclerosis. Treatments attempt to improve function after an attack and prevent new attacks. I know of two distant relatives affected by this malady.


10.Parkinson's disease
(PD also known as idiopathic or primary parkinsonism, hypokinetic rigid syndrome/HRS, or paralysis agitans) is a degenerative disorder of the central nervous system. The motor symptoms of Parkinson's disease result from the death of dopamine-generating cells in the substantia nigra, a region of the midbrain; the cause of this cell death is unknown. Early in the course of the disease, the most obvious symptoms are movement-related; these include shaking, rigidity, slowness of movement and difficulty with walking and gait. Later, thinking and behavioral problems may arise, with dementia commonly occurring in the advanced stages of the disease, whereas depression is the most common psychiatric symptom. Other symptoms include sensory, sleep and emotional problems. Parkinson's disease is more common in older people, with most cases occurring after the age of 50. My wife was diagnosed with PD just last year.

11. Tourette syndrome (also called Tourette's syndrome, Tourette's disorder, Gilles de la Tourette syndrome, GTS or, more commonly, simply Tourette's or TS) is an inherited neuropsychiatric disorder with onset in childhood, characterized by multiple physical (motor) tics and at least one vocal (phonic) tic. These tics characteristically wax and wane, can be suppressed temporarily, and are preceded by a premonitory urge. Tourette's is defined as part of a spectrum of tic disorders, which includes provisional, transient and persistent (chronic) tics. Tourette's was once considered a rare and bizarre syndrome, most often associated with the exclamation of obscene words or socially inappropriate and derogatory remarks (coprolalia), but this symptom is present in only a small minority of people with Tourette's. Tourette's is no longer considered a rare condition, but it is not always correctly identified because most cases are mild and the severity of tics decreases for most children as they pass through adolescence. I saw a couple of cases of children suffering from this disease here in Northern California as well as in the Philippines.

12. X-linked Dystonia Parkinsonism or Lubag
-X-linked dystonia-parkinsonism (XDP) is a genetic form of dystonia found almost entirely among males of Filipino descent of Panay Island. XDP is a recessive disorder affecting males almost exclusively. It is characterized by both dystonia and parkinsonism including signs and symptoms such as slow movement (bradykinesia), tremor, rigidity, and a loss of postural reflexes. With disease progression, the dystonia usually becomes generalized. In some patients, signs of parkinsonism may accompany, precede, or "replace" symptoms of dystonia. The disease is transmitted through unaffected females, so-called carriers. A few cases have been described in which females who carry a copy of the disease gene may manifest mild symptoms of the disorder, such as relatively mild dystonia or chorea.
When I was growing up in Panay Island in the 1960's I saw a man suffering from Lubag.
The locals believed he was a victim of the witches or the aswang in the area.

13. Enteroviral meningoencephalitis
has no cure similar to other neuro maladies. It has been managed with intravenous steroids with oral taper and intravenous immunoglobulin. It is a very rare disease and hard to diagnose. The symptoms of this disorder is similar to other neurological disorder, but common are confusion and disorientation, and distractible laugh at inappropriate moments. This case has been published on the July, 2008 issue of Neurology, Vol 4 and No 7 pages 399 to 403.

Reference:en.wikipedia.org

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