Adolescent Parkinsonism has similar symptoms to Parkinson's disease, but it affects children and adults before the age of 21. What are the causes of juvenile parkinsonism? What are the symptoms typical for him? What is the treatment?

Juvenile Parkinsonismis a very rare disease that affects children and adults under the age of 21. Ifparkinsonian symptomsbegin between the ages of 21 and 40, it is referred to as "early-onset Parkinson's disease".

Juvenile Parkinsonism - causes

The cause of juvenile parkinsonism may be a mutation in the parkin gene. This gene is inherited in an autosomal recessive manner. that is, to get sick, you must get one copy of the defective gene from each parent. In a situation where each parent carries one abnormal copy of the gene, the probability of their child developing the disease is 25%.

Juvenile Parkinsonism - symptoms

The main symptoms of juvenile parkinsonism include:

1) early onset of parkinsonian symptoms (before the age of 20), i.e.

  • tremor - unlike Parkinson's disease, it is postural in nature, i.e. it occurs when the patient opposes the force of gravity, i.e. when he takes a certain position, e.g. when he raises or straightens his arms. Parkinson's disease is characterized by resting tremor - it occurs at rest, i.e. when the limb is supported and the patient does not have to overcome gravity
  • motor slowdown, mainly due to "freezing the gait", eg the patient cannot take the first or second step or is unable to get through the door. Sick shiver in place or has a feeling that her feet "stick" to the ground
  • muscle stiffness
  • postural stability disorders

Characteristic of juvenile parkinsonism is that after sleep all symptoms improve for 30 minutes to 2 hours.

However, these symptoms are not as severe as in Parkinson's disease.

2) Presence of dystonic movements (involuntary performance of unnatural movements). They are more severe than in Parkinson's disease.

3) Good response to L-dopa (drug used forin Parkinson's disease) and early presentation of dyskinesias as a result of its administration. A specific symptom of juvenile parkinsonism is dyskinesia, usually of the foot, expressed as the extension of the big toe, clubfoot or plantar flexion.

In women, parkinsonian symptoms clearly worsen for several days before and after the first day of menstruation.

In cases of juvenile parkinsonism, dementia, orthostatic disorders, constipation, dysuria and increased facial maskiness are not found.

Important

The hallmarks of juvenile parkinsonism are:

  • occurring mainly in the female gender
  • mild severity of symptoms
  • dyskinesia
  • good response to levodopa treatment
  • rapid onset of side effects of this drug (i.e. dyskinesia)
  • premature termination of its operation

Juvenile Parkinsonism - diagnosis

The diagnosis is made on the basis of, inter alia, early-onset parkinsonian symptoms, the order in which symptoms appear, the course of action and the effect on L-dopa. It is also important to distinguish juvenile parkinsonism from other diseases in which parkinsonism is typical or less common, such as:

  • Wilson's disease
  • Huntington's disease
  • Hallervorden-Spatz disease
  • spinocerebellar ataxia

The probability of their occurrence in adolescence is much greater than in adolescent parkinsonism. Genetic testing is required to ensure that there is autosomal recessive juvenile parkinsonism. However, in Poland they are hard to find.

Worth knowing

Adolescent Parkinsonism and early-onset Parkinson's disease

Early-onset Parkinson's disease differs from juvenile Parkinsonism in a few more details than the age of its occurrence. Early-onset Parkinson's disease has more features similar to late-onset Parkinson's: a rare family history, no gender advantage, a poorer levodopa effect, later development of dyskinesia or premature withdrawal symptoms.

Juvenile Parkinsonism - treatment

Therapy comes down to fighting the symptoms of parkinsonism. It mainly consists of the so-called substitution treatment, i.e. replenishing the amount of dopamine in the body. Since dopamine is rapidly broken down directly, levodopa is used (in combination with benserazide - e.g. madopar - or with carbidopa - e.g. nakom, sinemet, poldomet), which is a dopamine precursorand transforms into it only after it reaches the brain. Drugs (e.g. selgres, jumex) are also administered, which increase the concentration of dopamine in the brain, which allows to reduce the doses of levodopa.

As mentioned, however, in the course of juvenile parkinsonism, side effects of levodopa (i.e. dyskinesia) appear rapidly and the drug's effect is prematurely extinguished.

Sources used in the article:

1. Wendorff J., Nadolski M.,Juvenile Parkinsonism , "Advances in Psychiatry and Neurology", 2002, No. 11

Category:

Neurology