Bridge Brain Syndrome

**Most syndrome - Cerebellar ataxia**.

R.N. Levina identified the hereditary pons-cerebellar syndrome ** (syn.** *** *** pons cerebellum ***, cerebral pons syndrome), which is based on a neurodegenerative disease of the nigrospinal tract with an autosomal recessive type of inheritance, caused by damage to the neurons of the substantia nigra, located in the pons and cerebellum. The term ***“brain syndrome”*** combines both independent diseases and multiple syndromes of various etiologies. The course of the disease is characterized by a progressive course with age: disorders of motor coordination, convulsive attacks, and pseudobulbar disorders appear. 27% of patients die from a stroke.

**Clinical picture**. Complaints and medical history are described in the section “Amyotrophic lateral sclerosis”.

Neurological status: central coordination disorders. First, intermittent difficulty appears when walking due to a “drunk” gait in a circle, handwriting disorders; later, paresis of the muscles of the trunk and limbs and ataxia occur. Dissociation of muscle and coordination disorders is characteristic. There is a moderate increase in strength in the proximal parts of the arms and legs, spastic paraplegia, loss of the sensory sphere according to the conduction type, moderate ataxia when performing coordination tests, especially finger-nose tests, and vertical type nystagmus.

Sensory disintegration is observed due to a combination of pyramidal and extrapyramidal insufficiency.

Pontine cerebri syndrome is a rare genetic disorder that affects the brain and is often fatal. It is also called Horner's syndrome, after the English surgeon James Horner.

Pontine syndrome, also known as Adam Oppenheim syndrome, is a series of rare congenital disorders of the nervous system that are usually caused by recessive mutations in genes. It is almost always associated with various abnormalities of the eyes, ears, nerves and muscles. Symptoms manifest individually, but any pontine type syndrome is life-threatening and requires immediate treatment.

Signs of pontine syndrome The condition of Horner's syndrome manifests itself gradually, the first sign may be incorrect positioning of the head due to weakening of the tone of the neck muscles, a decrease in the size of the palpebral fissure, divergent strabismus, swelling of the eyelid, drooping mucous membrane, scars on the eyelids, loss of eyelashes, abnormalities of the oculomotor and muscle nerves, changes in eye pigmentation. Symptoms of the syndrome appear between the ages of several weeks and a year, intensifying as the child grows. There is a lag in the development of speech, some children cannot swallow, convulsions, facial paralysis, disturbances in the functioning of body systems, blindness, swelling of the optic nerve, and brain damage. * A group of muscles called atropine is responsible for closing the eye before sleep. Primary signs include loss of vision, especially in the left eye, improper eyelid alignment, and inadequate retraction of the cheeks when the head is tilted down. Patients may have difficulty opening their eyelids and blinking. There is no treatment for this syndrome and the prognosis in most cases is unfavorable. Patients' life expectancy can range from two to three months to five years or more.