What is riboflavin transporter deficiency?
Description. Collapse Section. Riboflavin transporter deficiency neuronopathy is a disorder that affects nerve cells (neurons). Affected individuals typically have hearing loss caused by nerve damage in the inner ear (sensorineural hearing loss) and signs of damage to other nerves.
Whats BVVL?
The Brown-Vialetto-Van Laere syndrome (BVVL) is a rare neurological disorder characterized by progressive pontobulbar palsy associated with sensorineural deafness. Fifty-eight cases have been reported in just over 100 years. The female to male ratio is approximately 3:1.
How do I get more riboflavin?
You can get recommended amounts of riboflavin by eating a variety of foods, including the following:
- Eggs, organ meats (such as kidneys and liver), lean meats, and low-fat milk.
- Some vegetables (such as mushrooms and spinach)
- Fortified cereals, bread, and grain products.
How much riboflavin should I take for migraines?
For migraine headaches: The most common dose is riboflavin 400 mg daily for at least three months. A specific product (Dolovent; Linpharma Inc., Oldsmar, FL ) dosed at two capsules in morning and two capsules in the evening for 3 months has also been used.
Can motor neuron disease affect hearing?
Madras motor neuron disease (MMND) is characterized by weakness and atrophy of limbs, multiple lower cranial nerve palsies and sensorineural hearing loss.
How long do you live with bulbar palsy?
The limbs are less affected but as the disease progresses the patient may experience weakness in the arms and legs [3]. Life expectancy is between 6 months and 3 years from the onset of symptoms.
How much B2 should I take to prevent migraines?
An analysis of nine studies showed that taking 400 mg per day of vitamin B2 for 3 months significantly decreased pain associated with migraine attacks, as well as the duration and frequency of episodes ( 1 ). What’s more, vitamin B2 appears to be well-tolerated and to have no serious side effects.
Can riboflavin cure migraine?
Riboflavin plays an important role in cellular energy production in the body. Its role in mitochondrial energy metabolism suggests it may be effective in migraine. From the evidence available it is well tolerated and potentially effective as a preventive treatment for migraine.
What is the Brown-Vialetto-Van Laere syndrome?
The Brown-Vialetto-Van Laere syndrome (BVVL) is a rare neurological disorder characterized by progressive pontobulbar palsy associated with sensorineural deafness. Fifty-eight cases have been reported in just over 100 years.
What is type 1 riboflavin transporter deficiency neuronopathy?
Type 1 riboflavin transporter deficiency neuronopathy, also referred to as Brown–Vialetto–Van Laere syndrome-1 (BVVLS1) or Fazio–Londe disease (OMIM 211530 ), is an autosomal-recessive progressive neurologic disorder that is caused by mutations in the SLC52A3 gene.
What are the Brown-Vialetto-Van Laere and Fazio Londe syndromes?
The Brown-Vialetto-Van Laere (BVVL) and Fazio Londe (FL) syndromes are pontobulbar palsies usually of juvenile onset. The conditions appear identical, except that BVVL syndrome cases also have hearing loss.
Can riboflavin supplements help children with BVVL syndrome?
Treatment with riboflavin supplements has proved a life-saving treatment for some young patients. Recently, mutations in the SLC52A2 gene coding for another human riboflavin transporter (hRFT3) have been associated with BVVL syndrome as well, and in these cases oral riboflavin supplements have again proved beneficial.