Re: the spiral continues

[Guest guest]

Have you tried using any antivirals? Or an antimicrobial like olive

leaf extract? I'm so sorry for him... that must be very hard on

everyone. :-(

W

> As I have posted before, my son had been plagued with OCD issues

starting

> last christmas.

[Guest guest]

Hi Phyllis,

Oh, I sure know how that is....one just doesn't know when the next

outburst is going to come from and from what. Gosh, I wish we could

call each other when we need some uplifting. Sounds like you have

tried a lot. Has someone suggested that the aggressiveness may be

due to an overgrowth of Clostridia, a bacteria in the gut? You can

get a stool test to find out if he has an overgrowth of Clostridia.

Some have tried Culturelle to get rid of it, but sometimes the only

thing that works is Flagyl (must be prescribed by a doc.) We gave

our son Flagyl and it helped immensly with the tantrums and have

continued giving him Culturelle (2 per day) since the Flagyl. We

still have the tantrums but not as many. Hope you find the key

soon. Take care, Becky in Chesapeake, VA

> As I have posted before, my son had been plagued with OCD issues

starting

> last christmas. We eliminated his prozac and that seemed to take

care of

> it and he had a great 5-6 months. I wrote last month that it was

back

> and some folks wrote me with some suggestions. So far nothing has

> alleviated this and it continues to worsen each day. just getting

out of

> the house is a major ordeal again. If you hurry him or talk to

him, he

> starts all over again and becomes agressive. First we thought he

was

> having difficulty with EPO and EFA, but both of these were removed

with

> no improvements. They were the last two things added. We then

added

> biotin, but have seen no change.

> Does anyone have an idea what is setting off this reaction in his

basal

> ganglia? WE even tried a course of amox and diflucan.

> thanks,

> Phyllis

[Guest guest]

Phyllis, how old is he? I'm wondering about the puberty issue. Also

is he having chronic allergy symptoms? Perhaps vestibular issues due

to his ears being full of fluid? I've heard of kids having " stealth "

ear infections. Is he sleeping at night? Perhaps his sleep cycle is

off, causing day irritability and inablitity to fucus/function? I'm

not real sure on any of this stuff, just trying to guess with you.

Debi

> As I have posted before, my son had been plagued with OCD issues

starting

> last christmas. We eliminated his prozac and that seemed to take

care of

> it and he had a great 5-6 months. I wrote last month that it was

back

> and some folks wrote me with some suggestions. So far nothing has

> alleviated this and it continues to worsen each day. just getting

out of

> the house is a major ordeal again. If you hurry him or talk to

him, he

> starts all over again and becomes agressive. First we thought he

was

> having difficulty with EPO and EFA, but both of these were removed

with

> no improvements. They were the last two things added. We then

added

> biotin, but have seen no change.

> Does anyone have an idea what is setting off this reaction in his

basal

> ganglia? WE even tried a course of amox and diflucan.

> thanks,

> Phyllis

[Guest guest]

Phyllis,

The amoxicillin would have depleted his biotin chemistry a lot, probably by

killing off the flora that makes biotin. I don't know what dosages you

were using of biotin, but it may take megadoses to even begin to address

the issue of biotin depletion since basal ganglia symptoms appear to be a

very late-arriver on the deficiency symptom list. In the literature, the

precedent for improving this condition is 100 mg to 5-10 mg/kg/day. Your

son may continue to stay depleted because of the lasting effect of the

antibiotic in killing off biotin producing flora.

Until his flora is restored where it is able to make the vitamins he needs,

you may need to compensate with high dose oral vitamins. If you don't

" catch up " to balance the extent of the loss, he may continue to use up his

body stores of biotin wherever biotin is left, and apparently one good

place that bioitn is stored, and one of the last places the body will

borrow from is in the basal ganglia.

Have you tried as high as 40-100 mg/day of the biotin? (Do so with the

help of your child's doctor, of course.)

Your son might also need pantothenic acid which is made by the flora as

well, but if his sulfur chemistry has been depleted, I would look for the

already cysteinylated form called pantotheine or pantothine.

Phyllis, I know you must be frustrated, and I don't know how tolerant you

are reading abstracts. If you will read these below, however, I think you

will find that you shouldn't expect to see improvements in the basal

ganglia without very high dose biotin if what you are dealing with is

biotin deficiency.

You can also see that when only 10 mg/day was used in a child with autism

with known genetic defect in biotin recycling, he made absolutely no

clinical improvement. A friend of this child's family is on sulfurstories,

so we are trying to get them and their doctors in Italy to be aware of the

necessity for using very high-dose biotin after an extended period of

deficiency as evidenced by neurological signs like basal ganglia problems.

Neuropsychol Dev Cogn Sect C Child Neuropsychol. 2003 Sep;9(3):184-8.

A case of partial biotinidase deficiency associated with autism.

Zaffanello M, Zamboni G, Fontana E, Zoccante L, Tato L.

Regional Center for Neonatal Congenital Errors of Metabolism, University of

Verona, Verona, Italy

We report the case of a child with partial biotinidase deficiency and

autistic developmental disorder. We arrived at the diagnosis of biotinidase

deficiency when the child was almost 4 years of age. Consequently, he began

cofactor biotin treatment (10 mg daily) which did not resolve his autistic

behavior. His younger brother was affected by partial biotinidase

deficiency diagnosed at birth through our neonatal screening program. He

was precociously treated with cofactor biotin therapy (10 mg daily) and did

not show any behavioral abnormality or developmental delay. Since the brain

is quite vulnerable to biotin deficiency, delayed biotin therapy could

result in neurological damage. Our patient is the first case of partial

biotinidase deficiency associated with autism. We hypothesize that the low

biotinidase activity could have caused biotin deficiency in his brain and

cerebrospinal fluids and consequently serious neurological problems, such

as stereotyped and autistic behaviors, which were irreversible in spite of

biotin supplementation.

Neurology. 2002 Oct 8;59(7):983-9. Related Articles, Links

[Click here to read]

Familial infantile bilateral striatal necrosis: clinical features and

response to biotin treatment.

Straussberg R, Shorer Z, Weitz R, Basel L, Kornreich L, Corie CI,

Harel L, Djaldetti R, Amir J.

Neurogenetics Clinic and Department of Pediatrics, Schneider

Children's Medical Center of Israel, Petah Tivka. rachelst@...

BACKGROUND: Infantile bilateral striatal necrosis (IBSN) encompasses

several syndromes of bilateral symmetric, spongy degeneration of the

caudate nucleus, putamen, and globus pallidus. The familial form of IBSN is

rare, and inheritance is either autosomal recessive or maternal. METHOD:

The authors describe an Israeli Bedouin kindred in which 15 children born

to consanguineous parents were affected with familial IBSN. They evaluated

the clinical and radiologic evolution of the disease in 11 patients and the

cerebral pathologic findings in one patient. Three of the children were

treated with oral biotin 100 mg/day. RESULTS: Inheritance was apparently

autosomal recessive. The untreated children had a similar clinical picture

including developmental arrest beginning at the age of 7 to 15 months,

choreoathetosis, and dysphagia. Pendular nystagmus appeared at a late

stage. MRI, performed at various stages of the disease, showed severe basal

ganglia atrophy. Postmortem study in one patient showed severe atrophy of

the lenticular nuclei with gliosis and loss of neurons. Biotin, 100 mg/day,

administered to the proband over a period of 15 months, may have slowed

progression. In two other children treatment was initiated earlier and

appeared to arrest or improve disease. CONCLUSIONS: Familial infantile

bilateral striatal necrosis was inherited as an autosomal recessive trait.

Clinical features included developmental arrest, dysphagia, and

choreoathetosis. Imaging and pathology showed atrophy and degeneration of

the basal ganglia. Oral biotin may have benefited three children.

PMID: 12374138 [PubMed - indexed for MEDLINE]

Brain. 1998 Jul;121 ( Pt 7):1267-79. Related Articles, Links

[Click here to read]

Biotin-responsive basal ganglia disease: a novel entity.

Ozand PT, Gascon GG, Al Essa M, Joshi S, Al Jishi E, Bakheet S, Al

Watban J, Al-Kawi MZ, Dabbagh O.

Department of Paediatrics, King Faisal Specialist Hospital and

Research Centre, Riyadh, Saudi Arabia. ozand@...

We describe a novel, biotin-responsive basal ganglia disease in 10

patients. At onset, it appears as a subacute encephalopathy, with

confusion, dysarthria and dysphagia with occasional supranuclear facial

nerve palsy or external ophthalmoplegia, and progresses to severe cogwheel

rigidity, dystonia and quadriparesis. These symptoms disappear within a few

days if biotin (5-10 mg/kg/day) is administered, and there are no

neurological sequelae. They reappear within 1 month if biotin is

discontinued. Patients diagnosed late, or who have had repeated episodes,

suffer from residual symptoms such as paraparesis, mild mental retardation

or dystonia. The numerous biochemical studies of intermediary metabolism,

like the autoimmune and toxicological studies, enzyme assays including

biotinidase, carboxylase and lysosomal activities, and bacterial and viral

studies were all normal. The aetiology may be related to a defect in the

transporter of biotin across the blood-brain barrier. The only consistent

radiological abnormality was central necrosis of the head of the caudate

bilaterally and complete, or partial, involvement of the putamen on brain

MRI. This was present during the initial acute encephalopathy and remained

unchanged during follow-up of 3-10 years. Although its aetiology is

unknown, it is important to recognize this disease, since its symptoms may

be reversed and the progression of its clinical course prevented simply by

providing biotin.

PMID: 9679779 [PubMed - indexed for MEDLINE]

J Neurosci Methods. 1991 Sep;39(2):163-74. Related Articles, Links

The anterograde and retrograde transport of neurobiotin in the central

nervous system of the rat: comparison with biocytin.

Lapper SR, Bolam JP.

MRC Anatomical Neuropharmacology Unit, Department of Pharmacology,

Oxford, U.K.

In order to test whether neurobiotin, an analogue of biotin, is

transported by neurones in the central nervous system, injections of

varying volumes of a 5% solution were made into different regions of the

rat brain. Following perfusion-fixation, the sites of injection and

possible sites of transport were sectioned and incubated with an

avidin-biotin-peroxidase complex and then subjected to a peroxidase

reaction. All injection parameters and sites were directly compared to

equivalent injections of the closely related substance, biocytin, which is

effectively transported in an anterograde fashion. The sites of injection

of neurobiotin were characterised by large areas of labelling that were

more extensive than those produced by equivalent injections of biocytin but

with less intense labelling of individual neurones. Each of the injections

of neurobiotin gave rise to marked anterograde labelling, the

characteristics of which were similar to those produced by biocytin, but

due to the larger injection sites was heavier than that produced by

biocytin. Each of the injections of biocytin or neurobiotin also gave rise

to retrograde labelling; the degree of labelling was far greater with

neurobiotin but varied between pathways. Retrograde labelling only occurred

to a minor degree in some pathways but was particularly marked in others,

e.g., the striatonigral pathway. As with biocytin, tract-tracing with

neurobiotin can be applied to electron microscopy and can readily be

combined with immunocytochemistry for endogenous substances. It is

concluded that neurobiotin is an effective anterograde and retrograde

marker that may be of use in studies in the central nervous system,

particularly in large homogeneous structures such as cortical fields and

the striatum.

PMID: 1724681 [PubMed - indexed for MEDLINE]

Neurology. 1988 Aug;38(8):1326-8. Related Articles, Links

Basal ganglia calcifications in a case of biotinidase deficiency.

Schulz PE, Weiner SP, Belmont JW, Fishman MA.

Baylor College of Medicine, Department of Neurology, Houston, TX.

Biotinidase deficiency leads to a biotin-deficient state, with

cardinal symptoms of ataxia, alopecia, and skin rash presenting in infancy.

Previous reports of head CTs in patients with biotinidase deficiency did

not note basal ganglia calcifications. We report the first case of

biotinidase deficiency with basal ganglia calcifications. There were no

symptoms referable to basal ganglia dysfunction.

PMID: 3399084 [PubMed - indexed for MEDLINE]

J Neurosci Res. 1984;11(4):419-35. Related Articles, Links

Vitamin content of some normal human brain segments.

Baker H, O, Chen T, Feingold S, DeAngelis B, Baker E.

Nicotinates, pantothenates, riboflavin, vitamins B6 and B12' free

(acetyl) and total (free and bound) choline, biopterin, thiamin, biotin,

methylated and nonmethylated folates in frontal, temporal, precentral,

postcentral, and occipital cortex, thalamus, cerebellum, pons, basal

ganglia, and substantia nigra were estimated. Nicotinates are significantly

more concentrated in basal ganglia and thalamus than pons. Nonmethylated

folate content is not significantly varied in brain segments; the pons

contains more methylated folate. Riboflavin content is higher in the basal

ganglia and temporal cortex than frontal cortex. Biotin is concentrated in

pons and basal ganglia. Thiamin concentration is less in the postcentral

cortex than the thalamus and substantia nigra. Biopterin is significantly

higher in substantia nigra and basal ganglia than the other brain segments.

Total choline content is high in substantia nigra, pons, and thalamus; free

(acetyl) choline is significantly elevated in basal ganglia. B12 content is

less concentrated in the cortex segments. B6 is highly concentrated in the

basal ganglia. Pantothenate content is elevated in pons when compared to

the various cortex segments and cerebellum.

PMID: 6235380 [PubMed - indexed for MEDLINE]

Physiol Res. 1999;48(3):175-87. Related Articles, Links

[Click here to read]

Vitamins and brain development.

Ramakrishna T.

Department of Life Sciences, University of Calicut, Kerala, India.

trana@...

Effects of deficiency of vitamins on early development of brain have

been reviewed. Unusual developmental problems in neurogenesis specific for

the brain and impairment of its functional capacities due to vitamin

deficiency have been discussed. The species-specific " critical periods " in

development of various systems have been mentioned. Indices such as reflex

activity, locomotion, special senses, cognition and adaptive behavior were

used for assessing brain maturation in experimental models and humans.

Significant examples include brain anomalies in humans and other mammals

caused by retinoid excess or deficit; increase in calbindin D28K, a vitamin

D dependent calcium-binding protein during postnatal period in rat;

hydrocephalus and exencephaly in prenatal rats and subarachnoidal or

intracerebral hemorrhage in infants caused by vitamin E deficiency.

Peripheral neuropathic lesions leading to infantile beriberi is caused by

thiamine deficiency. Impaired growth in retinal layers leading to delay in

maturation of electroretinogram and depth-perception in postnatal rats

occur due to pyridoxine deficiency. Infants of severely vitamin B12

deficient mothers show abnormalities in behavior involving basal ganglia

and pyramidal tract. Folic acid deficiency results in delayed maturation of

the basic electroencepalographic patterns. In addition,

vitamin-interactions leading to developmental errors have been pointed out.

Vitamin B6 deficiency impairs vitamin B12 absorption and biotin deficiency

may be aggravated by pantothenic acid deficiency. Vitamin C deficiency

resulting in impaired metabolism may produce symptoms of deficiency of

folic acid. Another characteristic examples is that iron absorption from

dietary sources is dependent on ascorbic acid.

Publication Types:

* Review

* Review, Tutorial

PMID: 10523053 [PubMed - indexed for MEDLINE]

At 10:00 PM 9/25/2003 -0400, you wrote:

>As I have posted before, my son had been plagued with OCD issues starting

>last christmas. We eliminated his prozac and that seemed to take care of

>it and he had a great 5-6 months. I wrote last month that it was back

>and some folks wrote me with some suggestions. So far nothing has

>alleviated this and it continues to worsen each day. just getting out of

>the house is a major ordeal again. If you hurry him or talk to him, he

>starts all over again and becomes agressive. First we thought he was

>having difficulty with EPO and EFA, but both of these were removed with

>no improvements. They were the last two things added. We then added

>biotin, but have seen no change.

>Does anyone have an idea what is setting off this reaction in his basal

>ganglia? WE even tried a course of amox and diflucan.

>thanks,

>Phyllis

>

>

>=======================================================

>

[Guest guest]

,

We are currently on a low dose of lauricidin and working up.

Phyllis

[Guest guest]

Becky,

He did have the clostridia a few months ago and we treated it with the

flagyl and then lots of probiotics which we are still using. His last

blood work showed the clostridia was gone.

Thanks,

Phyllis

[Guest guest]

Debi,

thanks for all the ideas. He is only 8, so I don't think puberty would

be an issue here. He is an allergic kid, but no runny nose etc here. He

has been outside all summer with no changes at all. Plus, he is

sleeping wonderfully(knock on wood) as I believe the repetitive behavior

is making him tired. He goes to bed by 8:15 and sleeps until after 7.

Phyllis

[Guest guest]

,

the symptoms began prior to even using the amox. That is why we

thought it was strep at first and tried the amox since he has pandas like

symptoms. WE have been using 10 mg of biotin with no change. Maybe I

should increase it?

Phyllis

[Guest guest]

Phyllis,

Yes, I think you should increase it because of his history. Can you try

increasing it by 2 or 3 mg per day and see if he tolerates it, and at

whichever pace you choose, work up to several times what he is taking

now? From what I hear from other parents, when you get to the amount that

he needs, he'll notice the difference, not just you!

At 12:16 PM 9/26/2003 -0400, you wrote:

>,

> the symptoms began prior to even using the amox. That is why we

>thought it was strep at first and tried the amox since he has pandas like

>symptoms. WE have been using 10 mg of biotin with no change. Maybe I

>should increase it?

>Phyllis

>

>

>=======================================================

>

[Guest guest]

>>Plus, he is

> sleeping wonderfully(knock on wood) as I believe the repetitive

behavior

> is making him tired. He goes to bed by 8:15 and sleeps until after

7.

I don't remember your previous messages on this subject, but this is a

LOT of sleep, and chronic fatigue is the adult sign of phenol

intolerance. Have you considered that?

http://www.danasview.net/phenol.htm

Dana

[Guest guest]

--------- Forwarded message ----------

From: <PMRSKizner@...>

Date: Sat, 27 Sep 2003 23:33:53 -0400

Subject: Re: [ ] Re: the spiral continues

Dana,

Yes, you answered my post with several suggestions about this downward

spiral of OCD again. He does not get many phenols at all. We keep him

gfcfsf and free from additives and preseratives and low phenol.

He is only sleeping that much now as he is tired after retracing his

steps all day and doing each task multiple times.

Phyllis