Behr's Syndrome

Definition

Definition of Behr's Syndrome
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.

Symptoms

Symptoms of Behr's Syndrome
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.

Causes

Causes of Behr's Syndrome
Consult with your doctor.

Diagnosis

Diagnosis of Behr's Syndrome
Behr syndrome results in a spectrum of optic and neurological complications for both sexes. The disorder begins from early childhood with disturbance to vision, and loss or reduction in body control and co-ordination.

It includes a partial and increasing loss of vision, and/or blind spots. Eyesight degeneration is particularly prevalent in males. Symptoms can also include rapid involuntary eye movements (nystagmus), ataxia, progressive damage to nerves, nerve inflammation, mental retardation, urinary incontinence, and unusual foot reflexes when the sole is stimulated (positive Babinski sign).

Treatment

Treatment of Behr's Syndrome
Consult with your doctor.

Prognosis

Prognosis of Behr's Syndrome
Consult with your doctor.

Prevention

Prevention of Behr's Syndrome
Consult with your doctor.