Re: Pediatric Neurology Appointment....What to ask??? gluten encephalopathies

[Guest guest]

Ask if the doctor believes that gastrointestinal pathology (eg, food

hypersensitivity) might be etiologically significant in your son. Ask if

he'd be willing to work with other physicians in determining whether or

not there is a gut-related (nutritional and/or autoimmune) problem that

ought be considered in the differential diagnosis.

Base your question upon citations, send them and the above questions to

him at least a week before the appointment so that he has time to

contemplate. Of course, if he reads the gluten articles in the journal

neurology, he'll probably want to MRI and bill for the MRI and will not

say, Let's try GF for a while.

With few exceptions, I have found the neurologists are among the most

adamant in preaching against biomedical evals and interventions.

1: Curr Treat Options Neurol. 2005 Jan;7(1):43-48.

Peripheral Neuropathy and Celiac Disease.

Chin RL, Latov N.

Weill Medical College of Cornell University, Department of Neurology and

Neuroscience, Peripheral Neuropathy Center, 635 Madison Avenue 4th Floor, New

York, NY 10022, USA.

Peripheral neuropathy (PN) is one of the most frequently reported neurologic

manifestations associated with celiac disease (CD), a multigenetic,

T-cell-mediated autoimmune disorder that results from a loss of tolerance to

gluten. Sensory axonal and small fiber sensory polyneuropathies are the most

frequently reported PN subtypes. Multifocal motor or sensorimotor neuropathies

and a more fulminant neuropathy, associated with ataxia and other neurologic

manifestations, also have been reported. The effect of a gluten-free diet on

CD-associated PN has not been studied systematically or prospectively;

nevertheless, a gluten-free diet currently is the cornerstone of therapy.

Although idiopathic ataxia associated with anti-gliadin antibodies and other

neurologic complications have been reported to respond to this diet; there is

data that indicate that neurologic manifestations may develop or persist,

independent of gluten exposure. There is evidence to suggest that inflammatory

processes may be involved. Immunomodulatory agents (such as intravenous

immunoglobulin or infliximab), described to be beneficial in the treatment of

refractory CD or CD-associated ataxia, may have a role in the management of

CD-associated PN.

PMID: 15610706 [PubMed - as supplied by publisher]

2: Neurology. 2003 May 27;60(10):1581-5.

Comment in:

Neurology. 2003 May 27;60(10):1566-8.

Celiac neuropathy.

Chin RL, Sander HW, Brannagan TH, Green PH, Hays AP, Alaedini A, Latov N.

Peripheral Neuropathy Center, Department of Neurology and Neuroscience, Weill

Medical College of Cornell University, New York, NY 10022, USA.

RUC9002@...

BACKGROUND: Celiac disease (CD) is a chronic inflammatory enteropathy resulting

from sensitivity to ingested gluten. Neurologic complications are estimated to

occur in 10% of affected patients, with ataxia and peripheral neuropathy being

the most common problems. The incidence and clinical presentation of patients

with CD-associated peripheral neuropathy have not previously been investigated.

OBJECTIVE: To determine the incidence of CD in patients with neuropathy and to

characterize the clinical presentation. METHODS: The records of 20 patients with

neuropathy and biopsy-confirmed CD were reviewed. RESULTS: Six of the 20

patients had neuropathic symptoms alone without gastrointestinal involvement,

and neuropathic symptoms preceded other CD symptoms in another 3 patients. All

patients had burning, tingling, and numbness in their hands and feet, with

distal sensory loss, and nine had diffuse paresthesias involving the face,

trunk, or lumbosacral region. Only two had weakness. Results of

electrophysiologic studies were normal or mildly abnormal in 18 (90%) of the

patients. Sural nerve biopsies, obtained from three patients, revealed mild to

severe axonopathy. Using the agglutination assay, 13 (65%) of the patients were

positive for ganglioside antibodies. Excluding patients who were referred with

the diagnosis of celiac neuropathy, CD was seen in approximately 2.5% of all

neuropathy patients and in 8% of patients with neuropathy and normal

electrophysiologic studies seen at our center. CONCLUSION: CD is commonly

associated with sensory neuropathy and should be considered even in the absence

of gastrointestinal symptoms.

Publication Types:

Review

Review, Multicase

PMID: 12771245 [PubMed - indexed for MEDLINE]

3: Neurology. 2001 Feb 13;56(3):385-8.

Headache and CNS white matter abnormalities associated with gluten sensitivity.

Hadjivassiliou M, Grunewald RA, Lawden M, Davies- GA, T, CM.

Department of Clinical Neurology, The Royal Hallamshire Hospital, Sheffield, UK.

m.hadjivassiliou@...

The authors describe 10 patients with gluten sensitivity and abnormal MRI. All

experienced episodic headache, six had unsteadiness, and four had gait ataxia.

MRI abnormalities varied from confluent areas of high signal throughout the

white matter to foci of high signal scattered in both hemispheres. Symptomatic

response to gluten-free diet was seen in nine patients.

PMID: 11171906 [PubMed - indexed for MEDLINE]

4: Neurology. 2000 Jun 27;54(12):2346.

Celiac disease and idiopathic cerebellar ataxia.

Combarros O, Infante J, -Hoyos M, Bartolome MJ, Berciano J, Corral J,

Volpini V.

Neurology Service, " Marques de Valdecilla " University Hospital, Santander,

Spain. neuro@...

PMID: 10881272 [PubMed - indexed for MEDLINE]

5: Neurology. 1999 Oct 22;53(7):1606-8.

Cerebellar ataxia associated with subclinical celiac disease responding to

gluten-free diet.

Pellecchia MT, Scala R, Perretti A, De Michele G, Santoro L, Filla A, Ciacci C,

Barone P.

Department of Neurological Sciences, University of Napoli Federico II, Italy.

Publication Types:

Case Reports

PMID: 10534283 [PubMed - indexed for MEDLINE]

1: Neurol Sci. 2001 Nov;22 Suppl 2:S117-22.

Neurological manifestations of gastrointestinal disorders, with particular

reference to the differential diagnosis of multiple sclerosis.

Ghezzi A, Zaffaroni M.

Centro Studi Sclerosi Multipla, Ospedale di Gallarate, Universita di Milano,

Gallarate, Varese, Italy.

Neurological manifestations of gastrointestinal disorders are described, with

particular reference to those resembling multiple sclerosis (MS) on clinical or

MRI grounds. Patients with celiac disease can present cerebellar ataxia,

progressive myoclonic ataxia, myelopathy, or cerebral, brainstem and peripheral

nerve involvement. Antigliadin antibodies can be found in subjects with

neurological dysfunction of unknown cause, particularly in sporadic cerebellar

ataxia ( " gluten ataxia " ). Patients with Whipple's disease can develop mental and

psychiatric changes, supranuclear gaze palsy, upper motoneuron signs,

hypothalamic dysfunction, cranial nerve abnormalities, seizures, ataxia,

myorhythmia and sensory deficits. Neurological manifestations can complicate

inflammatory bowel disease (e.g. ulcerative colitis and Crohn's disease) due to

vascular or vasculitic mechanisms. Cases with both Crohn's disease and MS or

cerebral vasculitis are described. Epilepsy, chronic inflammatory

polyneuropathy, muscle involvement and myasthenia gravis are also reported. The

central nervous system can be affected in patients with hepatitis C virus (HCV)

infection because of vasculitis associated with HCV-related cryoglobulinemia.

Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is a disease caused

by multiple deletions of mitochondrial DNA. It is characterized by peripheral

neuropathy, ophthalmoplegia, deafness, leukoencephalopathy, and gastrointestinal

symptoms due to visceral neuropathy. Neurological manifestations can be the

consequence of vitamin B1, nicotinamide, vitamin B12, vitamin D, or vitamin E

deficiency and from nutritional deficiency states following gastric surgery.

Publication Types:

Review

Review, Tutorial

PMID: 11794474 [PubMed - indexed for MEDLINE]

2: Tidsskr Nor Laegeforen. 2000 Feb 10;120(4):439-42.

Comment in:

Tidsskr Nor Laegeforen. 2000 Apr 30;120(11):1357.

[Neurological diseases associated with celiac disease]

[Article in Norwegian]

Hagen EM, Gjerde IO, Vedeler C, Hovdenak N.

Nevrologisk avdeling, Haukeland Sykehus, Bergen.

During the period from May 1997 to October 1998, eight patients with coeliac

disease or dermatitis herpetiformis and neurological disorders were admitted to

the Department of Neurology, University Hospital of Bergen. The most frequent

conditions were polyneuropathy (seven patients) and spinocerebellar ataxia

(three patients). Other conditions were lower motor neuron disease, myelopathy,

epilepsy and encephalopathy. The patients used various degrees of gluten-free

diet at the time of admission. It remains unclear whether there is a shared

common pathogenetic mechanism or the neurological disorder is a complication to

the coeliac disease. Both vitamin depletion and immunological mechanisms may

cause neurological disorder. Neurological manifestations may occur before the

gastrointestinal symptoms. With reference to our patients and available

literature we discuss prevalence, clinical picture, pathogenesis, treatment and

prognosis. Neurologists, gastroenterologists and general practitioners should be

aware that coeliac disease can cause neurological diseases, especially

polyneuropathy, cerebellar ataxia and encephalopathy.

Publication Types:

Case Reports

PMID: 10833932 [PubMed - indexed for MEDLINE]

3: Arch Pediatr. 1996 Oct;3(10):1013-9.

[Celiac disease, cerebral calcifications and epilepsy syndrome]

[Article in French]

Cuvellier JC, Vallee L, Nuyts JP.

Service des maladies infectieuses et de neurologie infantiles, centre

hospitalier regional et universitaire de Lille, hopital B, France.

The syndrome of coeliac disease, epilepsy and cerebral calcifications is a rare

complication of coeliac disease. The pathological changes consist in a patchy

pial angiomatosis and resemble those of Sturge-Weber syndrome, whose variant

without port-wine angioma must be ruled out. Typical course includes three

stages leading to a severe encephalopathy. However, the mental impairment is

extremely variable. The pathogenetic process is so for unknown; main clues

involve a chronic folic acid deficiency or a HLA-related autoimmune disorder.

Treatment requires early gluten-free diet and anti-epileptic drug.

Publication Types:

Review

Review, Tutorial

PMID: 8952798 [PubMed - indexed for MEDLINE]

4: Postgrad Med J. 1991 Nov;67(793):1023-4.

Coeliac disease presenting with cerebellar degeneration.

Hermaszewski RA, Rigby S, Dalgleish AG.

Northwick Park Hospital and Clinical Research Centre, Harrow, UK.

A case of rapidly progressive cerebellar degeneration with bilateral sixth nerve

palsies is described in whom investigation revealed the presence of unsuspected

coeliac disease. In spite of treatment with a gluten free diet, rapid fatal

deterioration occurred. Coeliac disease should be considered in patients with

encephalopathy of obscure origin.

Publication Types:

Case Reports

PMID: 1775412 [PubMed - indexed for MEDLINE]

5: J Am Diet Assoc. 1979 Oct;75(4):449-52.

Diet therapy in gastrointestinal disease: a commentary.

Arvanitakis C.

Dietary regimens in gastrointestinal disease can be divided into two categories:

First, those of proven value include: (a) Disaccharide elimination for

disaccharidase deficiency and exclusion of monosaccharides for sugar

malabsorption; ( gluten-free diet for celiac/sprue; © elimination of certain

allergens because of food allergies; (d) protein restriction for portal systemic

encephalopathy; (e) low-carbohydrate diet for dumping syndrome; (f) low-fiber

diet for diarrheal syndromes; and (g) low-fat diet for steatorrhea. Second,

controversial diets include a bland diet for acid-peptic disorders, a high-fiber

diet for colonic disorders, and a low-fat diet for gallbladder disease. It is

important to separate facts from fancy in the dietary management of patients

with gastrointestinal disease and base the recommendations for a particular diet

on available objective evidence, not on traditional or fashionable trends.

PMID: 479489 [PubMed - indexed for MEDLINE]

cynthiahughes wrote:

>My son is 8 years old and was diagnosed at age 3 with medium

>functioning PDD. He has a neurology appointment soon and I need

>suggestions on what I should ask the doctor. He is on Risperdal and

>nothing else. He has never had any sort of detox or anything of that

>nature. Does anyone have any suggestions? He is starting to get

>really agressive at times and is very strong.

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[Guest guest]

ps: If you are able to talk physiology with the doc (eg, Amino acids),

he may steer you towards a genetic metabolic clinic. The lab data there

may find something, but most such evals are geared toward primary

genetic disorders and don't find the types of pathologies (eg, gastro,

nutritional) occurring in most autistic kids. Of course, old-guard

physicians (regardless of age) are trained to accept the genetic

metabolic screens, use old-guard reference ranges geared to the

must-be-a-primary-genetic-syndrome model, and will poo-poo or in other

ways belittle the possibilities of brain dysfunction derived from

nutritional suboptimality.

If the neurologist is somewhat open-minded (which happens occasionally),

he can consult with neurogist Hardy, MD, and/or with neugologist

Martha Herbert, MD, PhD (Harvard, Mass Gen). Each is well acquainted

with the need for physiological workups in autism and with brain

improvements via diet, supplements, etc.

Binstock wrote:

>Ask if the doctor believes that gastrointestinal pathology (eg, food

>hypersensitivity) might be etiologically significant in your son. Ask if

>he'd be willing to work with other physicians in determining whether or

>not there is a gut-related (nutritional and/or autoimmune) problem that

>ought be considered in the differential diagnosis.

>

>Base your question upon citations, send them and the above questions to

>him at least a week before the appointment so that he has time to

>contemplate. Of course, if he reads the gluten articles in the journal

>neurology, he'll probably want to MRI and bill for the MRI and will not

>say, Let's try GF for a while.

>

>With few exceptions, I have found the neurologists are among the most

>adamant in preaching against biomedical evals and interventions.

>

>

>

>1: Curr Treat Options Neurol. 2005 Jan;7(1):43-48.

>

>Peripheral Neuropathy and Celiac Disease.

>

>Chin RL, Latov N.

>

>Weill Medical College of Cornell University, Department of Neurology and

>Neuroscience, Peripheral Neuropathy Center, 635 Madison Avenue 4th Floor, New

>York, NY 10022, USA.

>

>

>

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