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Behr's Syndrome



Behr's Syndrome Complications


Complications from Behr’s Syndrome include learning disabilities and optic atrophy. Optic atrophy is the dysfunction of the optic nerve which results in impaired vision. These are secondary conditions that the disease has caused. In many cases, distinctions between symptoms of Behr syndrome and its complications are unclear or arbitrary.


Behr's Syndrome Definition


Behr's Syndrome is a rare inherited neurological condition named after Carl Behr, who first described it in 1909 as a syndrome of variable pyramidal tract signs. It is characterized by spastic paraplegia and sometimes optic atrophy. It is a hereditary familial syndrome that falls under ocular and neurologic disorders affecting both men and women. Behr's Syndrome starts in infancy with disturbed vision, disturbed coordination with ataxia, mental deficiency, and urinary sphincter weakness. Other characteristics include optic atrophy prevalent in males, scotoma, bilateral retrobulbar neuritis, nystagmus, progressive temporal nerve atrophy, increased tendon reflexes, Babinski sign, and incoordination. Its inheritance is autosomal recessive, meaning it is not linked to sex chromosomes, although heterozygotes may still manifest much attenuated symptoms.


Behr's Syndrome Prevalence


Behr’s syndrome has been listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Behr’s syndrome, or one of its subtypes, affects less than 200,000 people in the US population. The symptom has been reported to affect both men and women.


Behr's Syndrome Symptoms and Signs


Patients with Behr's Syndrome experience impaired vision, loss or reduction of coordination, and may have an inability to control any voluntary movements. These symptoms usually appear at the onset of infancy or early childhood. There may also be mental deficiency, optic atrophy, and rapid involuntary eye movements or eye twitching. Symptoms include an increasing loss of vision or blind spots where there was previously normal sight. This degeneration of eyesight is particularly prevalent in males affected with Behr's Syndrome. Unusual foot reflexes (a positive Babinski sign) may also be experienced by the patient when the sole is stimulated. Increasing tremors in both hands are also symptoms of this disease. It is associated with ataxia, spasticity, mental retardation, and posterior column sensory loss. Successful treatments of hand tremors using deep brain stimulation (DBS) of the ventral intermediate thalamic nucleus (VIM) have been reported. There have been cases that indicate that deep brain stimulation of the ventral intermediate nucleus is adequate as an operative intervention. It has been known to help reduce tremors even in patients with complicated movement disorders brought about by the disease.


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