One Liter of Tears: "Spinocerebellar Degeneration" Explained

Have you heard about Spinocerebellar Degeneration? It is also known as Spinocerebellar Ataxia. I really don’t have any idea about this disease until I watched “One Liter of Tears” also called as “One Litre no Namida" or "A Diary with Tears" or "A Diary of Tears”. It’s a Japanese TV drama series, an adaptation of the diary from a Japanese girl named Aya Kito. This drama series is one of the best TV series I have ever watched. You will feel the pain that her family actually feels. Aya Ikeuchi the girl who suffers Spinocerebellar Degeneration was played by Erika Sawajiri. The drama series is so heart touching which will make anyone watch the entire series.

Now, I’ll change the topic and give you some information about this disease. Sad to say but this disease is not curable. I want this to be written on my blog to share it to everyone who doesn’t have any idea about this disease. Let’s help one another to share information like this so that everyone will be aware of it. I just can take the pain like what happened to the girl. So hard to see someone who always fell in the ground and getting hurt because a person suffering this will actually encounter problems in control ling their muscles.

Spinocerebellar Ataxia (SCA):

A progressive, degenerative, genetic disease with multiple types, each of which could be considered a disease in its own right.

Symptoms:

Spinocerebellar ataxia is one of a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs, and different ataxias are known to affect different regions within the cerebellum.

As with other forms of ataxia, SCA results in unsteady and clumsy motion of the body due to a failure of the fine coordination of muscle movements, along with other symptoms.

The symptoms of an ataxia vary with the specific type and with the individual patient. Generally, a person with ataxia retains full mental capacity but may progressively lose physical control.

Treatment and Prognosis:

There is no known cure for spinocerebellar ataxia, which is a progressive disease (it gets worse with time), although not all types cause equally severe disability.

Treatments are generally limited to softening symptoms, not the disease itself. The condition can be irreversible. A person with this disease will usually end up needing to use a wheelchair, and eventually they may need assistance to perform daily tasks.

The treatment of incoordination or ataxia, then mostly involves the use of adaptive devices to allow the ataxic individual to maintain as much independence as possible. Such devices may include a cane, crutches, walker, or wheelchair for those with impaired gait; devices to assist with writing, feeding, and self care if hand and arm coordination are impaired; and communication devices for those with impaired speech.

Many patients with hereditary or idiopathic forms of ataxia have other symptoms in addition to ataxia. Medications or other therapies might be appropriate for some of these symptoms, which could include tremor, stiffness, depression, spasticity, and sleep disorders, among others.

Both onset of initial symptoms and duration of disease can be subject to variation. If the disease is caused by a polyglutamine trinucleotide repeat CAG expansion, a longer expansion may lead to an earlier onset and a more radical progression of clinical symptoms.

Diagnosis:

It can be easily misdiagnosed as another neurological condition, such as multiple sclerosis (MS).

The most precise means of identifying SCA, including the specific type, is through DNA analysis. Asymptomatic and at risk family members considering a genetic test should consult with a physician and genetic counselor. An MRI of the brain may also show whether certain regions of the cerebellum have noticeably degenerated, but this is often not the most robust way to provide a diagnosis.

SCA is related to olivopontocerebellar atrophy (OPCA); SCA types 1, 2, and 7 are also types of OPCA. However, not all types of OPCA are types of SCA, and vice versa. This overlapping classification system is both confusing and controversial to some in this field.

Source: wikipedia