Psychological Aspects-1














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PORPHYRIA FACTS: Psychological Aspects -1
General Information

Mental

Referring to the mind, psychic, or that which is psychological.

Mental disorders

Psychiatric illness or diseases manifested by breakdowns in the adaptational
process expressed primarily as abnormalities of thought, feeling, and behaviour
producing either distress or impairment of function.

Mental state

A finding on physical examination that may refer to any number of abnormal
changes in baseline mental functioning.

Milder examples include mood changes, irritability, personality changes,
depression or blunted affect.

Advanced changes include confusion, lethargy, sleepiness, hallucinations,
unresponsiveness and coma


Which of the types of porphyria have the psychological symptoms associated
with them?

Acute hepatic porphyrias delta-aminolaevulinic acid dehydratase deficiency
porphyria (ALA-D), acute intermittent porphyria (AIP) , hereditary
coproporphyria (HCP) and variegate porphyria(VP) are characterized by
variable extrahepatic gastrointestinal, neurological-psychiatric and
cardiovascular manifestations.

SOURCE:
Erythropoietic and hepatic porphyrias.
Gross U, Hoffmann GF, Doss MO.
Division of Clinical Biochemistry,
Faculty of Medicine
Philipps University, Marburg, Germany.
Journal of Inherited Metabolic Disease
2000 Nov;23(7):641-61
+++++++++++++++++++++++++++++++++++
What are the psychological manifestations of acute porphyria?

Psychiatric manifestations of the acute porphyrias include hysteria, anxiety,
depression, phobias, psychosis, organic
disorders,agitation, delirium, and altered consciousness ranging from
somnolence to coma.

SOURCE:
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center, Torrance 90509, USA.
Psychother Psychosom 1995;64(3-4):121-30
++++++++++++++++++++++++++++++++++
Are there mental problems associated with acute porphyria?

Inborn errors of metabolism often present with a variety of psychiatric
symptoms.

SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry
University of California, San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
Neuropsychiatric manifestations are central to the acute porphyrias.

SOURCE:
Hepatic Porphyrias
Dr. Dweight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
++++++++++++++++++++++++++++++++++++
Acute intermittent porphyria (AIP) is a rare autosomal disease that presents with
gastrointestinal, psychiatric, and neurological symptoms.

SOURCE:
"Acute intermittent porphyria in a children's psychiatric hospital."
Boon, F. F. and C. Ellis
Journal of American Academy of
Child and Adolescent Psychiatry
28(4): 606-9.
++++++++++++++++++++++++++++++++++++

What are the cerebral forms of neuropsychiaric disturbance associated with
acute porphyria?

Cerebral forms include hysteria, depression, agitation, delirium, psychosis,
convulsions, and altered consciousness (ranging from somnolence to coma).
Anxiety is most often present and can be accompanied by insomnia and a
general restlessnesss.

SOURCE:
Dr. Robert Johnson M.D.
Internal Medicine
++++++++++++++++++++++++++++++++++++
Are the psychiatric symptoms of porphyria limited just to confusion or is there are
a large variety of expressions?

Inborn errors of metabolism often present with a variety of psychiatric symptoms.

SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry
University of California, San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
++++++++++++++++++++++++++++++++++++
What is the most common psychological aspect of acute porphyria?

The commonest psychiatric diagnosis in acutepatients is generalized anxiety.

SOURCE:
Acta Psychiatric Scandinvia
1994 Apr;89(4):262-7
Acute intermittent porphyria and mental illness
Patience DA, Blackwood DH, McColl KE, Moore MR.
Department of Psychiatry, University of Edinburgh,
Royal Edinburgh Hospital, United Kingdom
+++++++++++++++++++++++++++++++++++

Porphyria may be overrepresented in psychiatric populations.

SOURCE:
The little imitator
Porphyria: a Neuropsychiatric Disorder
Helen Crimlisk
Neurology, Neurosurgery & Psychiatry
1997
62; 319-328
++++++++++++++++

Many somatic diseases such as acute intermittent porphyria (AIP) are followed
by psychiatric and neurological symptoms."

SOURCE:
Vojnosanit Pregl
2001 Jan-Feb;58(1):95-9
Acute intermittent porphyria as a problem in differential diagnosis
Preradovic M, et. al.
++++++++++++++++++

Advances in molecular biology permit identification of patients and latent
carriers of porphyria thus lessening the number of patients who have been
characterized as psychiatric.

SOURCE:

The little imitator
Porphyria: a Neuropsychiatric Disorder
Helen Crimlisk
Neurology, Neurosurgery & Psychiatry
1997
62; 319-328
++++++++++




Do AIP have a higher incidence of psychological conditions?

Acute Intermittent Porphyria (AIP) has a higher incidence in psychiatric
populations

SOURCE:
Acute Intermittent Porphyria
Medicore Ltd.
1996

++++++++++++++++++++++++++++++++

Is there a higher percentage of porphyria patients among psychiatric
populations?

Porphyria may be overrepresented in psychiatric populations.

SOURCE:
The little imitator
Porphyria: a Neuropsychiatric Disorder
Helen Crimlisk
Neurology, Neurosurgery & Psychiatry
1997
62; 319-328

+++++++++++++++++++++++++++++++++++

When dealing with changes pertaining to your lifstyle including chronic illness,
you must prioritize the behavorial changes that will make you most confident
about your health.

SOURCE:
Healthy Lifestyle
Lawrence C. Perlmuter
Department of PSychology
Finch University of Health Sciences
Chicago Medical School
+++++++++++++++

Psychiatric hospitals have a disproportionate number of patients with porphyria
as only difficult and resistant patients accumulate there.

SOURCE:
Porphyria: reexamination
of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center, Torrance 90509, USA.
++++++++++++++

Neuropsychiatric manifestations of AIP are extensive

SOURCE:
Acute intermittent porphyria in a
children's psychiatric hospital.
Boon, F. F. and C. Ellis
J Am Acad Child Adolesc Psychiatry
1989; 28(4): 606-9.
++++++++++++++



What percentage of acute porphyria patients experience psychiatric
manifestations?

40% of clinical cases of acute porphyria present with mental confusion or
hallucinations.

SOURCE:
Acute intermittent porphyria in a
children's psychiatric hospital.
Boon, F. F. and C. Ellis
Journal of the American Academy of Children & Adolescent Psychiatry
1989; 28(4): 606-9.
Do AIP have a higher incidence of psychological conditions?
++++++++++++++++++++++++++++++++++

Porphyria is important in psychiatry as it may present with only psychiatric
symptoms; it may masquerade as a psychosis and the patient may be treated as
a schizophrenic person for years; the only manifestation may be histrionic
personality disorder which may not receive much attention.

SOURCE:
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center, Torrance 90509, USA.
Psychother Psychosom 1995;64(3-4):121-30
++++++++++++++

Psychiatric manifestations of Acute Intermittent Porphyria (AIP)
include:
anxiety
depression
hallucinations
hysteria
insomnia, paranoia
paranoia
restlessness
violence

SOURCE:
Columbia Health Care
1998
++++++++++++++


Psychiatric hospitals have a disproportionate number of porphyria patients in
their populations because diagnosis of psychiatric complaints during the times
patients are resistant and exacerbating mental symptoms.

SOURCE:.
Psychother Psychosom
1995;64(3-4):121-30
++++++++++++++

Inborn errors of metabolism often present with a variety of psychiatric
symptoms.

SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry
University of California, San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
+++++++++++++++++++

Hepatic porphyrias are a group of metabolic disorders characterized
by intermittent abdominal pains, vomiting and confusion.

SOURCE:
Genetic Metabolic Disorders of Children
Ronald H. Payne
Faculty of Medicine
Memorial University of Newfoundland
++++++++++++++++++

Acute hepatic porphyrias delta-aminolaevulinic acid dehydratase deficiency
porphyria (ALA-D), acute intermittent porphyria (AIP) , hereditary
coproporphyria (HCP) and variegate porphyria (VP) are characterized by
variable extrahepatic gastrointestinal, neurological-psychiatric and
cardiovascular manifestations.


SOURCE:
Erythropoietic and hepatic porphyrias.
Gross U, Hoffmann GF, Doss MO.
Division of Clinical Biochemistry,
Faculty of Medicine
Philipps University, Marburg, Germany.
Journal of Inherited Metabolic Disease
2000 Nov;23(7):641-61
++++++++++++++++++

Porphyria is important in psychiatry as it may present with only psychiatric
symptoms.

SOURCE:
Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509.
+++++++++++++++

With improved diagnosis and treatment options, many acute porphyric patients
have increased lifespans; consequently, issues of long-term quality of life are
coming to the forefront.

Mental health concerns are among these issues.

SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry,
University of California San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
++++++++++++








Acute Intermittent Porphyria (AIP) has a higher incidence in psychiatric
populations

SOURCE:
Acute Intermittent Porphyria
Medicore Ltd.
1996
++++++++++++++

Inborn errors of metabolism often present with a variety of psychiatric
symptoms.


SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry
University of California, San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
++++++++++++++

The autonomic or involuntary nervous system can be affected leading to
problems such as high blood pressure, excessive sweating, rapid heart rate and
changing bowel and bladder functions including constipation and urinary
retention.

SOURCE:
The Canadian Porphyria Foundation Inc.
Neepawa, Manitoba, Canada
+++++++++++++++

Inborn errors of metabolism often present with a variety of psychiatric
symptoms.

SOURCE:
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
++++++++++++++

With improved diagnosis and treatment options, many patients have increased
lifespans; consequently, issues of long-term quality of life are coming to the
forefront.

Mental health concerns are among these issues.

SOURCE:
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
++++++++++++

The connection between the course of metabolic disease and its psychiatric
manifestations, inborn errors of metabolism have been reviewed
in acute intermittent porphyria.

SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry,
University of California,
San Diego, California
++++++++++++++

There can be emotional and psychiatric problems such as anxiety, insomnia,
agitation, confusion, paranoia, depression and hallucinations, although
there is little evidence to suggest that porphyria itself is a cause of any of the
chronic psychiatric syndromes.

SOURCE:
The Canadian Porphyria Foundation Inc.
Neepawa, Manitoba, Canada
+++++++++++++++



Psychiatric manifestations include hysteria, anxiety, depression, phobias,
psychosis, organic disorders, agitation, delirium, and altered
consciousness ranging from somnolence to coma.

SOURCE:
Psychotherapy Psychosomanics
995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509.
++++++++++++++



Seizures and mental abnormalities that range form confusion ro psychosis may
be presenting features of an acute attack.

None are permanent conditions and generally no longer present during
remission.

SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition
+++++++++++++++

Cerebral forms include hysteria, depression, agitation, delirium, psychosis,
convulsions, and altered consciousness (ranging from somnolence to coma).

SOURCE:
Dr. Robert Johnson M.D.
Retired Clinician
+++++++++++++


Neuropsychiatric manifestations are central to the acute porphyrias.

SOURCE:
Hepatic Porphyrias
Dr. Dweight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
+++++++++++++



Psychiatric manifestations include hysteria, anxiety, depression, phobias,
psychosis, organic disorders, agitation, delirium, and altered consciousness
(ANS) ranging from somnolence to coma.

SOURCE:
Journal of Psychotherapy
1995;
64(3-4):121-30
"Porphyria: reexamination of psychiatric implications."
Burgovne K. MD et al.
Harbor-UCLA Medical Center,
Torrance 90509, USA.
++++++++++++++







Psychiatric symptoms during acute attacks may mimic many other disorders.

SOURCE:
The little imitator
Porphyria: a Neuropsychiatric Disorder
Helen Crimlisk
Neurology, Neurosurgery & Psychiatry
1997
62; 319-328
+++++++++++++++++

Porphyria is important in psychiatry as it may present with only psychiatric
symptoms; it may masquerade as a psychosis and the patient may be treated as
a schizophrenic person for years; the only manifestation may be histrionic
personality disorder which may not receive much attention.

SOURCE:
Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center, Torrance 90509
Acute intermittent porphyria mimics a variety of commonly occurring
disorders and thus poses a diagnostic quagmire.
+++++++++++++++++


The commonest psychiatric diagnosis in acutepatients is generalized anxiety"

SOURCE:
Acta Psychiatric Scandinvia
1994 Apr;89(4):262-7
Acute intermittent porphyria and mental illness
Patience DA, Blackwood DH, McColl KE, Moore MR.
Department of Psychiatry,
University of Edinburgh,
Royal Edinburgh Hospital, United Kingdom.
++++++++++++


Many somatic diseases such as acute intermittent porphyria (AIP) are followed
by psychiatric and neurological symptoms."

SOURCE:
Vojnosanit Pregl
2001 Jan-Feb;58(1):95-9
Acute intermittent porphyria as a problem in
differential diagnosis
Preradovic M, et. al.
++++++++++++++



Seizures and mental abnormalities that range form confusion ro psychosis may
be presenting features of an acute attack.

None are permanent conditions and generally no longer present during
remission."

SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition
++++++++++++++

Porphyria may masquerade as a psychosis and the patient may be treated as a
schizophrenic person for years; the only manifestation may be histrionic
personality disorder which may not receive much attention."

SOURCE:
Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509.
++++++++++++++


Disorientation, ANS, Hallucinations as well as some paranoia can exacerbate in
Intoxification Porphyria."

SOURCE:
Stephen B. Edelson, M.D.
The Edelson Center for Environmental
and Preventive Medicine
3833 Roswell Road, Suite 110
Atlanta, GA 30342
++++++++++++++

Psychiatric manifestations in acute porphyria include hysteria, anxiety,
depression, phobias, psychosis, organic disorders, agitation, delirium, and
altered consciousness ranging from somnolence to coma.

SOURCE:
Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center, Torrance 90509
+++++++++++++++++


Some patients develop psychosis similar to schizophrenia. during mental change
in porphyria.

SOURCE:

Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center, Torrance 90509
+++++++++++++++++

Porphyria is important in psychiatry as it may present with only psychiatric
symptoms.

SOURCE:
Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509.
+++++++++++++++

Bizarre behaviors are common.

SOURCE:
"Acute intermittent porphyria in a children's psychiatric hospital."
Boon, F. F. and C. Ellis
Journal of American Academy of
Child and Adolescent Psychiatry
28(4): 606-9.
+++++++++++++++

Acute intermittent porphyria (AIP) is a rare autosomal disease that presents with
gastrointestinal, psychiatric, and neurological symptoms.

SOURCE:
"Acute intermittent porphyria in a children's psychiatric hospital."
Boon, F. F. and C. Ellis
Journal of American Academy of
Child and Adolescent Psychiatry
28(4): 606
+++++++++++++

Psychiatric manifestations commonly include hysteria, altered
consciousness ranging from somnolence to coma.

SOURCE:
Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509.
++++++++++++++


Acute Intermittent Porphyria (AIP) has a higher incidence in psychiatric
populations

SOURCE:
Acute Intermittent Porphyria
Medicore Ltd.
1996

+++++++++++++++++++++++++++++++
Inborn errors of metabolism such as acute intermittent porphyria, often present
with a variety of psychiatric symptoms.

SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry,
University of California San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-
+++++++++++++


How prevalent are the neuropsychiatric aspects of acute porphyria?

Neuropsychiatric manifestations of AIP are extensive.

SOURCE:
Acute intermittent porphyria in a
children's psychiatric hospital.
Boon, F. F. and C. Ellis
J Am Acad Child Adolesc Psychiatry
1989; 28(4): 606-9.

+++++++++++++++++++++++++++++++

What are the psychiatric manifestations of AIP?

Psychiatric manifestations of Acute Intermittent Porphyria (AIP) include:
anxiety
depression
hallucinations
hysteria
insomnia, paranoia
paranoia
restlessness
violence

SOURCE:
Columbia Health Care
1998
+++++++++++++++++++++++++++++++++++++
Psychiatric manifestations of AIP include hysteria, anxiety, depression,
phobias, psychosis, organic disorders, agitation, delirium, and altered
consciousness ranging from somnolence to coma.

Some patients develop psychosis similar to shizophrenia.

Psychiatric hospitals have a disproportionate number ofpatients with this
disorder as only difficult and resistant patients accumulate there.

SOURCE:
Journal of Psychotherapy and Psychosomatics
1995;
64(3-4):121-30
"Porphyria: reexamination of psychiatric implications."
Burgovne K, et. al
Harbor-UCLA Medical Center,
Torrance CA.
++++++++++++++++++++++++++++
Neurological or psychiatric symptoms occur in most acute attacks.

SOURCE:
The little imitator
Porphyria: a Neuropsychiatric Disorder
Helen Crimlisk
Neurology, Neurosurgery & Psychiatry
1997
62; 319-328
++++++++++++
Patients are often dismissed for years as "mentally ill" or "drug seeking."

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++

Some research data indicate that 1 in 300 patients in psychiatric wards have
porphyria.

Data regarding the misdiagnosis of porphyria among the psychiatric population
has rarely been published in medical journals and remains relatively
unknown by the majority of practicing physicians.

SOURCE:
The Porphyrias
The Challenge of Diagnosis
United Health Services
1996
++++++++++++


Psychiatric manifestations of the acute porphyrias include anxiety, depression,
phobias, psychosis, agitation, delirium, and altered consciousness (ANS)
ranging from somnolence to coma.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++++++

Does one have to worry about neuropsychiatric aspects of acute intermittent
porphyria?

Neuropsychiatric manifestations of AIP are extensive. Treatment of
psychological manifestations is through the timely trreatment of the acute attack
itself.

Most drugs that are used for treatment of true psychiatric manifestations are
contraindicated for use in acute porphyria patients.

SOURCE:
Dr. Robert Johnson M.D.
Internal Medicine
Retired Clinician
++++++++++++++++++++++++++++
Can there be mental change during an acute attack?

Mental change and disturbances is often noted during acute attacks.

SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry
University of California, San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
There can be emotional and psychiatric problems such as anxiety, insomnia,
agitation, confusion, paranoia, depression and hallucinations, although there is
little evidence to suggest that porphyria itself is a cause of any of the chronic
psychiatric syndromes.

SOURCE:
The Canadian Porphyria Foundation Inc.
Neepawa, Manitoba, Canada


+++++++++++++++++++++++++++++++++++

What percentage of AIP patients experience psychiatric manifestationsduring
acute attacks?

About 51% of patients with Acute Intermittent Porphyria (AIP) experience
Psychiatric Manifestations.

SOURCE:
Columbia Health Care
1998
+++++++++++++++++++++++++++++++++++
Do psychiatric hospitals have a higher number of patients with porphyria?
:
Psychiatric hospitals have a disproportionate number of patients with this
disorder as only difficult and resistant patients accumulate there.

SOURCE:
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509, USA.
Psychother Psychosom 1995;64(3-4):121-30
++++++++++++++++++++++++++++++
Are long-term mental conditions associated with HCP?

Long-term severe psychiatric illness in HCP is very uncommon.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.

+++++++++++++++++++++++++++++++
Are neuro-psychiatric symptoms associated with HCP?

"HCP is characterized by episodic acute attacks that consist of various
neuro-psychiatric symptoms.

SOURCE:
Journal of Photodermatololy, Photoimmunologyand Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
University Hospital
+++++++++++++++++++++++++++++++++++

Munchausen's Syndrome is a condition in which a patient falsely claims to be
sick or injured as to receive medical treatment and hospitalization.

The patient imagines the symptoms and history of a real disease.

The unreal symptoms will get better with treatment.

However, the patient may then seek treatment later for another imaginary
disease.

Another name which can be applied to this symdrome is that of Pathomumicry.

SOURCE:
The Mosby Medical Enclopedia
Revised Edition
+++++++++++++++

Are mental abnormalities permanent in porphyria?

"Seizures and mental abnormalities that range form confusion ro psychosis may
be presenting features of an acute attack.

None are permanent conditions and generally no longer present during
remission."

SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition

Do mental changes due to AIP resolve?

Mental symptoms may occur during attacks, but are usually not chronic.

SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX

+++++++++++++++++++++++++++++++++
Can mental change in porphyria become chronic?

"There is little objective evidence that porphyria produces chronic psychiatric
illness."

SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
+++++++++++++++++++++++++++++++++
Can psychiatric symptoms of porphyria be misdiagnosed as real psychiatric
problems?

"Porphyria may masquerade as a psychosis and the patient may be treated as a
schizophrenic person for years; the only manifestation may be histrionic
personality disorder which may not receive much attention."

SOURCE:
Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509.
+++++++++++++++++++++++++++++++++

How do the mental aspects affect the quality of life in porphyria patients?

A battery of self-report psychosocial measures was mailed to 116
patients who had been referred for clinical management (clinic
attenders) or laboratory diagnosis (non-clinic attenders) to the London
Supraregional Assay Service Centre for Porphyria over the past
decade and who tested positive for porphyria.

Usable replies were received from 81 (70%) patients.

Interest focused on the prevalence of psychosocial symptoms in acute
porphyrias and the perceived effects of porphyria on quality of life and patient
experience.

Research questions examined included (i), lifestyle factors; (ii) life
events; (iii) mental health; (iv) general health; and (v) perceptions of illness of
patients receiving specialist clinical management compared
to respondents referred for diagnostic investigations, between patients
with latent or manifest symptomology and between patients with different types
of porphyria.

Patients with porphyria have an impaired quality of life, particularly manifest
cases, compared to controls and to diabetic patients.

Depression, and particularly anxiety, is more common than in the general
population or general medical outpatient attenders.

Quality of life is lower in acute intermittent porphyria (AIP) than in other forms of
porphyria and a significant number of patients had major life event
consequences, e.g. failure to secure, or loss of, employment, limitation of family
size.

Patients attending a clinic providing specialist porphyria advice, management
and counselling received some perceived lifestyle benefits.

SOURCE:
Millward LM, Kelly P, Deacon A, Senior V, Peters TJ.
Department of Clinical Biochemistry,
GKT School of Medicine, King's
College, London, UK.
Journal of Inherited Metabolic Disease
2001 Dec;24(7):733-47
+++++++++++++++++++++++

There is little objective evidence
that porphyria produces chronic
psychiatric illness."

SOURCE:

Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
+++++++++++++++++++++

Seasonal affective disorder is a type of depression brought on by the
gloom of winter. Learn how to shine a light on your darkness.

SAD can sometimes accompany acute porphyria episodes but it is not medically
related.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++++




Is porphyria true psychiatric illness?

There is little objective evidence that porphyria produces chronic psychiatric
illness.

SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
+++++++++++++++++++

Acute hepatic porphyrias delta-aminolaevulinic acid dehydratase deficiency
porphyria (ALA-D), acute intermittent porphyria (AIP) , hereditary
coproporphyria (HCP) and variegate porphyria (VP) are characterized by
variable extrahepatic gastrointestinal, neurological-psychiatric and
cardiovascular manifestations.


SOURCE:
Erythropoietic and hepatic porphyrias.
Gross U, Hoffmann GF, Doss MO.
Division of Clinical Biochemistry,
Faculty of Medicine
Philipps University, Marburg, Germany.
Journal of Inherited Metabolic Disease
2000 Nov;23(7):641-61
+++++++++++++



Porphyria is important in psychiatry as it may present with only psychiatric
symptoms.

SOURCE:
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509, USA.
Psychother Psychosom 1995;64(3-4):121-30
+++++++++++++++++++++++++++++++

Neurotransmitters are naturally occurring chemicals in the brain that act as
messengers between nerve cells, affecting brain function and mood.

Those associated with depression include serotonin, norepinephrine and
dopamine.

SOURCE:
Mental Health Resource
Mayo Foundation for Medical Education and Research
Rochester, Minnesota
2003
++++++++++++++


Porphyria may masquerade as a psychosis and the patient may be
treated as a schizophrenic person for years.

SOURCE:
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509, USA.
Psychother Psychosom 1995;64(3-4):121-30
+++++++++++++++++++++++++++++++

The most common neurologic manifestations of acute intermittent porphyria are
autonomic visceral neuropathy, peripheral motor neuropathy, and central
nervous system dysfunctions, including seizures and neuropsychiatric
disturbances

SOURCE:
Transient Cortical Blindness and Bioccipital Brain Lesions in Two Patients with
Acute Intermittent Porphyria
Hugo Kupferschmidt, MD; et. al.
Annals of Internal Medicine
American College of Physicians
15 October 1995
123:598-600.
+++++++++++


Often in porphyria the only manifestation may be histrionic personality disorder
which may not receive much attention.

SOURCE:
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509, USA.
Psychother Psychosom 1995;64(3-4):121-30
+++++++++++++++++++++++++++++++

The high level of porphyrins in the heme pathway is responsible for changes in
the CNS (central nervous system) which causes mental changes to occur.

SOURCE:
Painful porphyrins
Julie L. McDowell
Diseases and disorders
American Chemical Society
2001
+++++++++++++



Porphyria is important in psychiatry as it may present with only psychiatric
symptoms.

SOURCE:
Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509.

+++++++++++++++++++++++++++++++++++
Can an acute porphyric present with only psych symptoms and not other porph
symp[toms?


"Porphyria is important in psychiatry as it may present with only psychiatric
symptoms; it may masquerade as a psychosis and the patient may be treated as
a schizophrenic person for years; the only manifestation may be histrionic
personality disorder which may not receive much attention. "

SOURCE:
Psychotherapy Psychosomanics
1995;64(3-4):121-30
Porphyria: reexamination of psychiatric implications.
Burgovne K, Swartz R, Ananth J.
Harbor-UCLA Medical Center,
Torrance 90509

++++++++++++++++++++++++++++++++++++++

Do the psychiatric manifestations of porphyria cause a problem in getting a
correct porphyria diagnosis?

Acute intermittent porphyria mimics a variety of commonly occurring disorders
and thus poses a diagnostic quagmire.

So many aspects of porphyria immitate othe more common disease
manifestations.

SOURCE:
Robert Johnson M.D.
Internal Medicine

+++++++++++++++++++++++++++++++++
What are some of the cerebralmanifestations that occur in acute porphyria?

Cerebral forms of CNS manifestations include hysteria, depression, agitation,
delirium, psychosis, convulsions, and altered consciousness (ranging from
somnolence to coma).

SOURCE:
Acute intermittent porphyria in a
children's psychiatric hospital.
Boon, F. F. and C. Ellis
J Am Acad Child Adolesc Psychiatry
1989; 28(4): 606-9.
++++++++++++++++++++

Is medical research looking into the mental aspects of porphyria?

With improved diagnosis and treatment options, many acute porphyric patients
have increased lifespans; consequently, issues of long-term quality of life are
coming to the forefront.

Mental health concerns are among these issues.

SOURCE:
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry,
University of California San Diego
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
+++++++++++++++++++++++++++++++++
Why are porphyria patients so often thought to be "drug seekers" or "nuts"?

Because of no tangible physical findings, porphyria patients are often dismissed
for years as "functional", "mentally ill", or "drug seekers".

SOURCE:
American Journal of Clinical Pathology,
Vol. 103, No. 6, June 1995

+++++++++++++++++++++++++++++++++
Are doctors aware that porphyria symptoms are not psychiatric, but just appear
to be that way on the surface?

"Inborn errors of metabolism often present with a variety of psychiatric
symptoms.

With improved diagnosis and treatment options, many patients have increased
lifespans; consequently, issues of long-term quality of life are coming to the
forefront.

Mental health concerns are among these issues.

The connection between the course of metabolic disease and its psychiatric
manifestations, inborn errors of metabolism have been reviewed in acute
intermittent porphyria."

SOURCE:
Journal of Inherited Metabolic Disease
2000 Feb;23(1):2-6
Psychiatric symptoms of inherited metabolic disease.
Estrov Y, Scaglia F, Bodamer OA.
Department of Psychiatry,
University of California,
San Diego, California
+++++++++++++++++++++++++++++++






Do EPP patients have psychological suffering?

EPP patients suffer considerable physical and psychological distress.

SOURCE:
Erythropoietic protoporphyria: a study of its psychosocial aspects
Rufener, E.A.
British Journal of Dermatology
1987 May;116(5):703-8

+++++++++++++++++++++++++++++++++
What are the psychological aspects of Intoxification Porphyria?

"Disorientation, ANS, Hallucinations as well as some paranoia can exacerbate in
Intoxification Porphyria."

SOURCE:
Stephen B. Edelson, M.D.
The Edelson Center for Environmental and Preventive Medicine
3833 Roswell Road, Suite 110
Atlanta, GA 30342
++++++++++++++++++++++++
Can AIP patients demonstrate extreme bizarre behavior during acute attacks?

The subject was a young man known to suffer from the in Swedish form of AIP
porphyria.

The subject committed homicide in an acute attack of porphyria.

The subject died from the disease 7 days later.
SOURCE:
Homicide in acute porphyria.
Trafford PA.
Forensic Science
1976 Mar-Apr;7(2):113-20
++++++++++++++++++++++++

Porphyria is important in psychiatry as it may present with only psychiatric
symptoms; it may masquerade as a psychosis and the patient may be treated as
a schizophrenic person for years; the only manifestation may be histrionic
personality disorder which may not receive much attention.

SOURCE:
Journal of Psychotherapy
1995;
64(3-4):121-30
"Porphyria: reexamination of psychiatric implications."
Burgovne K. MD et al.
Harbor-UCLA Medical Center,
Torrance 90509, USA.
+++++++++++++++++


Inborn errors of metabolism often present with a variety of mental changes
otherwise termed "psychological" or "psychiatric".

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++


AIP is not a psychiatric disease but does have temporary mental changes that
take place during acute attacks.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++


With more timely and improved diagnosis of AIP and again timely treatment
options of AIP, most AIP patients today have increased lifespans and improved
quality of life, although no where consistent with the quality of life experienced
by normally healthy individuals.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++



Many of the mental changes that take place during an acute attack are due in
part to chemical imbalances causes by other AIP symptoms of the CNS (central
nervous system).


Electrolyte imbalances are a major factor and need to be monitored closely
during acute attacks. Imbalances can often lead to mental change during
attacks.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++


Insomnia, and stress factors play a role in mental changes in porphyria.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++


The stress of going to the emergency room alone is enough to cause a
heightened sense of anxiety and agitation.

Emergency Room worders are often unfamiliar with porphyria and patients
feel the sttress of having to explain their illness.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

Many porphyria patients feel that they are "put on hold" or "disbelieved" and at
the same time feared hearing the old cliché,"it's all in your head", "you're
imagining the gut pain", or " Munchausen's".

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++



After the confirmed diagnosis, the phrase "you must descend from royalty".
or comments in regard to red urine not being purple, cause many porphyria
patients stress and they become irritable.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++
.


It is hard enough to deal with having porphyria without others "hanging labels" or
dealing unknowledgeable humor on a person.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++


The worse of all of the mental changes is that of the ANS (altered neurologic
state.)

When the ANS is present usually there is absolutely no recall or no control
of things said or actions done.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++


A study on published in the 1996 "Scientific American" indicate that 56 % of AIP
patients experience mental change.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

As a part of that mental change 30% experience confusion, disorientation, and
hallucinations.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++


Memory is affected during the ANS. (altered neurologic state)

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++++++


Seizures leave the patient with disorientation until the activity has completely
stopped.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++


In the "Scientific American" study, it was found that 12% of AIP patients
experience seizure activity.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++



Some AIP patients complain of depression or / and migraine headache.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

While depression or migraine headache can be present, they are not a symptom
of AIP, but rather most likely an expression of having to deal with living with an
inherited disease the magnitude of AIP.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++

Irritability is one mental change that is always present during an acute attack

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++

Depression and anxiety amond porphyria patients are far more common than in
the general population.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++++++

Paranoia, as CNS involvement in porphyria is known as the so-called
porphyric encephalopathy.

SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
++++++++++++++

There is little objective evidence
that porphyria produces chronic
psychiatric illness."

SOURCE:

Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
++++++++++++++++

A battery of self-report psychosocial measures was mailed to 116 patients who
had been referred for clinical management (clinic attenders) or laboratory
diagnosis (non-clinic attenders) to the London Supraregional Assay Service
Centre for Porphyria over the past decade and who tested positive for porphyria.
Usable replies were received from 81 (70%) patients.

Our interest focused on the prevalence of psychosocial symptoms in acute
porphyrias and the perceived effects of porphyria on quality of life and patient
experience.

Research questions examined included (i), lifestyle factors; (ii) life events; (iii)
mental health; (iv) general health; and (v) perceptions of illness of patients
receiving specialist clinical management compared to respondents referred for
diagnostic investigations, between patients with latent or manifest
symptomology and between patients with different types of porphyria.

Patients with porphyria have an impaired quality of life, particularly manifest
cases, compared to controls and to diabetic patients.

Depression, and particularly anxiety, is more common than in the general
population or general medical outpatient attenders.

Quality of life is lower in acute intermittent porphyria (AIP) than in other forms of
porphyria and a significant number of patients had major life event
consequences, e.g. failure to secure, or loss of, employment, limitation of family
size. Patients attending a clinic providing specialist porphyria advice,
management and counselling received some perceived lifestyle benefits.

SOURCE:
Self-rated psychosocial consequences and quality of life in the acute porphyrias.
Millward LM, Kelly P, Deacon A, Senior V, Peters TJ.
Department of Clinical Biochemistry,
GKT School of Medicine,
King's College,
London, UK.
Journal of Inherited Metabolic Disease
2001 Dec;24(7):733-47
++++++++++++++++ ++++













































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