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Multiple Sclerosis And Diffuse Cerebral Sclerosis (Schilder's Disease)

Multiple Sclerosis

• Multiple sclerosis is a chronic, remitting-relapsed disease that causes multiple lesions in the white matter.

• Multiple sclerosis history in first-degree relatives is approximately 20%. It is thought to be the result of an autoimmune reaction to an infectious agent in people with a genetic predisposition.

• MS in children is rare, with 2%-5% of MS patients presenting before the age of 18 years. It is very mild in boys before the age of 6, while it is twice as common in girls after the age of 12.

• It gives symptoms according to the location of the sclerosis. It is a disease that progresses with demyelination of cerebral periventricular white matter, especially in the perivenous parts.

Lesions may also occur in the brain stem and spinal cord.

• It can be seen in children with ataxia, spasticity or visual disturbances. It most commonly presents with cerebellar ataxia, often asymmetrical spastic weakness, optic neuritis, and diplopia.

• Optic nerve is the most common cranial nerve involvement and there is optic atrophy and atrophy of optic nerve fibers, especially near the temporal lobe. Argil Robertson pupil can be observed. N. oculomatorius is the most commonly involved after the optic nerve. Nystagmus may occur.

• The most common cause of bilateral trigeminal neuralgia is multiple sclerosis.

• Some patients have the Charcot Triad:

1) Nystagmus

2) Essential tremor

3) Heavy limp speech: It is observed in advanced cases of the disease.

• The preferred method in diagnosis is MRI. MRI shows small 3-4 mm plaques in the corpus callosum, periventricular white matter, brain stem, and spinal cord. There is an increased IgG index and/or an oligoclonal band in CSF.

• Patients with the following items can be diagnosed:

2 demyelinating nonencephalopathic attacks lasting more than 24 hours at least 30 days apart, unexplained by another reason thought to be originating from the central nervous system

Demyelinating plaques on MRI after a nonencephalopathic demyelinating attack

Presence of one of the following in addition to positive cranial MRI findings in the presence of a clinically progressive demyelinating disease rather than an attack:

- Positive spinal MRI findings

- Detection of Oligoclonal band in CSF

Multiple Sclerosis In Children

• In the treatment, high-dose methylprednisolone is used in the attack.
• Modifying interferon therapy can reduce disease activity. Interferon-13 is the most widely used and recommended first-line therapy. It can be used in Interferon-a.
• Glatiramer acetate, fingolimod and natalizumab are agents that have been used successfully in adults. Fingolimod has been FDA approved for children and there are studies showing that it reduces the number of attacks more than interferon-beta.
• Devic's disease (neuromyelitis optica): It is a variant of multiple sclerosis. It primarily involves the optic nerve and posteriorly the spinal cord, most commonly the thoracic segment. It is thought that IgG antibodies developed against aquaporin-4 channels in the central nervous system, especially in astrocytes, cause the disease. It is known to play a role in astrocyte death in the classical complement pathway. Involvement of the optic nerve causes sudden vision loss and central scotoma. Some endocrinopathies such as inappropriate ADH, Diabetes insipidus, and pubertal arrest may accompany. Steroids (methylprednisolone) are used in the treatment. Rituximab is effective in preventing relapses. Eculizimab has been shown to reduce recurrences and reduce damage in patients with severe NMO.
Multiple Sclerosis

Diffuse Cerebral Sclerosis (Schilder's Disease)

• It is an acute, progressive and rare demyelinating disease resembling multiple sclerosis histologically.
• It occurs sporadically between the ages of 5-12 and results in death. The reason is not known exactly. But it is thought to be autoimmune or infectious.
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