I was diagnosed with Neuroangiodema as a child.  I know very little
about it and would like to know more.  I have been told that it is a
blood disorder that is inherited.  I have had one seizure/cardiac
arrest due to this "disorder".  If there is anyone who can send me ANY
information, please send it asap.  Thank you.

---

Request for Question Clarification by pinkfreud-ga on 23 Feb 2003 19:26 PST

I have not been able to find any online references to
'neuroangiodema'. However, there is a condition called 'angioneurotic
edema' that can be inherited.

You'll find an excellent discussion of angioneurotic edema here:

http://www.users.globalnet.co.uk/~aair/angioedema.htm

If this is the condition you have, I'd be glad to help you find more
information about it. If this does not sound like your condition, it
would be very helpful if you could describe some of your symptoms in
detail, so that we can pinpoint the correct name of the disease and
find the exact information you need.

Thanks! The more you tell us, the better we'll be able to help.

~pinkfreud

gagrl...

When I plugged 'Neuroangiodema' into a Google search, nothing came up,
so I tried 'neuro angiodema' and Google asked: "Did you mean:
'neuro angioedema'?", which turned up quite a few results.

A search on PubMed for 'neuro angiodema' turned up an abstract of a 
study titled:

"Studies of four Japanese families with hereditary angioneurotic
edema:
 simultaneous activation of plasma protease systems and exogenous
 triggering stimuli.", by:

Kodama J, Uchida K, Yoshimura S, Katayama Y, Kushiro H, Yutani C,
Funahashi S, Takamiya O, Matsumoto Y, Ando Y, et al.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6388669&dopt=Abstract

You can read the article at the link above, however it's written in
complex medical terminology. What's most useful is the precise term
"hereditary angioneurotic edema" (HANE), which most closely matches
your suggested diagnosis.


The PureliNatural website has a glossary which describes this
condition
in simpler language:

Quincke's disease --> Angioneurotic edema

"Angioedema is a form of localized swelling of the deeper layers
 of the skin and fatty tissues underneath the skin. Angioedema
 can occur as a result of an allergic reaction, in association
 with other diseases (such as blood vessel inflammation,
 vasculitis), with cancers (such as lymphoma), or for unknown
 reasons."

"Hereditary angioneurotic edema, also referred to as
 hereditary angioedema, is an inherited form of angioedema.
 Patients with hereditary angioneurotic edema from birth lack
 an inhibitor protein (called C1 esterase inhibitor) that
 normally prevents activation of a cascade of proteins that lead
 to the swelling of angioedema. Patients with hereditary
 angioneurotic edema can develop recurrent attacks of swollen
 tissues, pain in the abdomen, and swelling of the voice box
 (larynx), which can cause impaired breathing. The diagnosis of
 hereditary angioneurotic edema is suspected when a patient has
 a history of recurrent angioedema. It is confirmed by finding
 abnormally low levels of C1 esterase inhibitor in the blood.
 Treatment options of hereditary angioneurotic edema include
 antihistamines and male steroids (androgens) that can also
 prevent the recurrent attacks."
http://www.purelinatural.com/Glossary_q.html


The eMedicine site has a page on Angioedema which is returned
in searches for the following synonyms and keywords:

angioneurotic edema, oedema, laryngeal edema, hereditary angioedema,
HAE, acquired angioedema, AAE, allergic reactions, airway obstruction,
hereditary angioneurotic edema, swelling

"Angioedema can occur as a result of (1) hereditary angioedema (HAE);
 (2) acquired angioedema (AAE); (3) angioedema associated with
allergic
 reactions, which is often associated with urticaria; (4) angioedema
 secondary to medications; and (5) idiopathic angioedema. In this
 article, the hereditary and acquired forms are discussed in detail.
 Angioedema induced by drugs, specifically angiotensin-converting
 enzyme (ACE) inhibitors, is explored briefly. Angioedema related
 to reactions mediated by immunoglobulin E is discussed in
Anaphylaxis."

"In 1888, Osler first described HAE when he treated a 24-year-old
 woman for chronic episodic attacks of edema. By interviewing the
 woman’s 92-year-old grandfather, Osler learned that 5 successive
 generations of the family had a history of similar attacks. Osler
 proposed an inherited etiology and named the entity hereditary
 angioneurotic edema. In 1917, Crowder and Crowder determined that
 the condition is inherited as an autosomal dominant trait."

"In 1963, Donaldson and Evans determined that an inherited deficiency
 of C1-esterase inhibitor (C1-INH) is at the core of this disease.
 C1-INH is one of the first components of the complement system.
 Two phenotypic variants of this disorder exist. In type I, which
 accounts for 80-85% of cases of HAE, serum levels of C1-INH (as
 determined by immunoassays) are low. In type II, patients have
 normal or elevated levels of C1-INH (as determined by immunoassay)
 but this C1-INH is dysfunctional (as determined by a functional
 assay). This deficiency in functioning C1-INH leads to
 autoactivation of the complement system and release of kininlike
 mediators, resulting in edema of the subcutaneous or submucosal
 tissues."
There's more on the source page:
http://www.emedicine.com/med/topic135.htm


Another page on eMedicine is more precisely focused on 'hereditary
angioedema':

"Background: Hereditary angioedema (HAE) is an autosomal dominant
 disorder manifested by painless, nonpruritic swelling of the skin.
 Type I HAE is defined by low plasma levels of a normal C1-INH
 protein. Type II HAE is characterized by the presence of normal
 or elevated levels of a dysfunctional C1-INH."
Again, there's more on the source page:
http://www.emedicine.com/derm/topic24.htm


There's also an entire website devoted to the condition, here:
http://www.hereditaryangioedema.com/

This extensive site has many resources, including diagnostic
information, treatment alternatives and much, much more.

Frequently Asked Questions about Hereditary Angioedema:
http://www.hereditaryangioedema.com/phamplet.htm


According to the Who Named It site, Quincke's disease,
or angioedema has the following other names:

Bannister’s disease
Bannister’s syndrome
Milton’s urticaria
Milton-Quincke syndrome
Quincke’s oedema
Quincke’s syndrome

The following are synonyms for the condition:

"Acute circumscribed oedema, acute essential oedema,
 angioderma syndrome, angioneurotic oedema, angioneurotic
 oedema of the skin, circumscribed oedema, cutaneous
 angioneurosis, oedema cutis circumscriptum, giant hives,
 giant urticaria, migratory oedema, urticaria, oedema
 angioneuroticum, urticaria oedematosa, urticaria gigantea,
 wandering oedema."

And here is their description of hereditary angioneurotic oedema:

"The heritable form, hereditary angioneurotic oedema, is
 transmitted as an autosomal dominant trait, involving the
 larynx and viscera. It is marked by abdominal pain, and
 has occasional severe and fatal respiratory complications.
 It is due to C1 esterase inhibitor deficiency. This inhibitor
 usually keeps the inflammatory reaction in check, but when it
 fails, a Quincke’s oedema may result. The term Quincke's oedema
 should be maintained, as the term angioneurotic oedema is less
 accurate. The sporadic form may be caused by allergy, infection,
 or emotional stress. Both sexes affected."
http://www.whonamedit.com/synd.cfm/482.html


Please do not rate this answer until you are satisfied that
the answer cannot be improved upon by means of a dialog
established through the "Request for Clarification" process.

sublime1-ga


Searches done, via Google:

neuroangiodema
://www.google.com/search?q=neuroangiodema

neuro angiodema
://www.google.com/search?q=neuro+angiodema

neuro angioedema
://www.google.com/search?q=neuro+angioedema

hereditary neuro angioedema
://www.google.com/search?q=hereditary+neuro+angioedema

hereditary angioneurotic edema
://www.google.com/search?q=hereditary+angioneurotic+edema

Quincke's disease
://www.google.com/search?q=Quincke%27s+disease

---

Request for Answer Clarification by gagrl-ga on 24 Feb 2003 10:54 PST

Neuroangiodema is a blood disorder that causes hives, seizures,
possible cardiac arrest, and an assortment of other, less significant
problems.  Every doctor I have seen knows nothing about this
"disorder".  I was told to keep my blood pressure and heart rate low
because stress triggers problems.  When I had my first child in 1993,
I had a seizure and went into cardiac arrest.  I have been trying to
find out information since then with no luck.  I have searched every
internet site I could think of with no luck either.  Maybe you know of
a site that I don't.

---

Clarification of Answer by sublime1-ga on 24 Feb 2003 15:59 PST

gagrl...

The word 'neuroangiodema' doesn't appear anywhere on the entire
internet, according to searches on the major search engines and
medical databases. This makes it seem highly unlikely to be the
exact spelling of an exact diagnosis, since even the rarest of
disorders have considerable presence on the internet.

'Angiodema' does show up, quite often, but as an alternate
spelling for angioedema. The fact that angioedema or angiodema
does have a hereditary form and that an alternate name is
hereditary angioneurotic edema makes it seem very likely that
this is the condition you are looking for. The fact that it is
closely associated with hives, and that stress can trigger it,
makes it seem even more likely (one of the alternate names is
'giant hives').

Since the condition is diagnosed based on blood tests for low
levels of C1 Esterase Inhibitor, it is understandable that one
might then think it to be a blood disorder, but, in fact, the
deficiency is due to an abnormal gene, which results in C1 being
deficient in the blood.

You say you were diagnosed as a child, which makes it seem likely
that either your doctor at the time or you parent(s) informed you
that there was something wrong with your blood (which is true, to
an extent) and you came away believing you had a blood disorder.
While it's true that your blood will never test 'normal', it is
due to a hereditary deficiency in the ability to produce C1. In
other words, it's in your genes, really, but it shows up in your 
blood. Since you were diagnosed at a young age, it also makes sense
that the terminology of the diagnosis could have been scrambled
as it was passed on from the doctor to your parent(s) to you
(or do you have it written/typed by the diagnosing doctor from
your childhood?).

Taking all of this into consideration, I am reasonably confident
that your actual diagnosis is hereditary angioneurotic edema.
A blood test would confirm it, by showing abnormally low levels
of C1.

Can you give me any concrete reasoning as to why the above
scenario might not be true?

sublime1-ga