What Are the Symptoms of Autonomic Dysreflexia?

Patient Presentation
An 18-year-old female came to the inpatient ward from her local physician’s office for a diagnosis of febrile urinary tract infection and mild dehydration that was worsening over the past 24 hours. She had strong smelling urine, a low grade fever and was not feeling well. She said she just didn’t want to drink either. Her past medical history showed she had a C7 spinal cord injury at age 16 because of a motor vehicle accident. Her main problems were constipation, repeated urinary tract infections, and some occasional dermatitis. During the first year after her injury she had some bouts of autonomic dysreflexia but this was usually well-controlled. The review of systems was otherwise negative.

The pertinent physical exam showed her lying in bed. Her temperature was 101.3&deg F with a normal blood pressure. She had generalized muscle wasting. Her abdomen was soft without organomegaly, masses or distension. Her neurological examination showed no feeling or motor movement from the upper thoracic region downward. The laboratory evaluation showed a dirty appearing urine that had a specific gravity of 1.025, was positive for leukocyte esterase, nitrate and blood, and showed 45 white blood cells, and 10 red blood cells. Her complete blood count showed a white blood cell count of 21.2 x 1000/mm2 with a left shift. Her C-reactive protein was 5.6 mg/dl. She was started on empiric antibiotics and her urine culture eventually grew E. coli. She was changed to oral antibiotics based upon the organism’s sensitivities. The patient’s clinical course initially had waxing and waning mild hypertension to 140-150 systolic and 100-110 for diastolic. The hypertension seemed to correspond to the patient being febrile and when her bladder was full. She was treated with more consistent urinary drainage by clean intermittent catheterization and given antipyretics on a scheduled basis. After 4 days she was well hydrated, afebrile, her hypertension had resolved and she was discharged home.

Discussion
Autonomic dysreflexia (AD) or hyperreflexia occurs in people with spinal cord injuries where the automatic signals and responses within the body problem do not occur normally.

The University of Alabama’s Department of Physical Medicine and Rehabilitation explains it this way: “AD occurs as a response to some type of irritation below the level of injury. The body tries to send signals to the brain to identify and respond to the irritant, but signals are blocked by the [spinal cord injury].” “Without communication between the brain and body, blood vessels begin to narrow and cause a rise in blood pressure. When sensors tell the brain that the blood pressure is getting too high, the brain then tries to lower the blood pressure….”

Causes of AD are usually normal physiological states but in this setting of spinal cord injury are irritants. These include bladder problems such as spasms, or just an overfilled bladder. Bowel problems are similar with constipation being a common irritant. Skin problems including pressure sores, wounds, rashes or just general dermatitis. Other problems include temperature extremes, trauma, pressure on body parts, abdominal cramping, and for women genitourinary states such as menstrual cramping, pregnancy and labor.

Treatment is to find the irritation and eliminate it. Hypertension may need to be treated with anti-hypertensive medication. AD can be a medical emergency.

Learning Point
Additional symptoms may be minimal or none except for hypertension.
Other signs and symptoms of AD include:

  • “Pounding headache
  • Blurred vision
  • Flushed face
  • Nasal congestion
  • Red blotches on upper body
  • Nausea
  • Cool, clammy skin
  • Chills without fever
  • Apprehension or anxiety
  • Sweating above the level of injury
  • Good bumps above the level of injury
  • Slow pulse”

Questions for Further Discussion
1. How can a physical medicine and rehabilitation specialist assist in patients with chronic medical diseases?
2. Besides AD, what other problems are patients with spinal cord disease at risk for?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Autonomic Nervous System Disorders and Spinal Cord Injuries.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Krassioukov A, Warburton DE, Teasell R, Eng JJ; Spinal Cord Injury Rehabilitation Evidence Research Team. A systematic review of the management of autonomic dysreflexia after spinal cord injury. Arch Phys Med Rehabil. 2009 Apr;90(4):682-95.

Bice T., Huang CT, Klebine P. University of Alabama Department of Physical Medicine and Rehabilitation. Spinal Cord Injury Infosheet. Available from the Internet at www.spinalcord.uab.edu (rev. 3/2009, cited 9/2/13).

Milligan J, Lee J, McMillan C, Klassen H. Autonomic dysreflexia: recognizing a common serious condition in patients with spinal cord injury. Can Fam Physician. 2012 Aug;58(8):831-5.

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    4. Patient management plans are developed and carried out.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Professionalism
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What Neoplasms are Patients with Neurofibromatosis At Risk For?

    An 8-year-old female with neurofibromatosis type 1 was admitted to the inpatient service with emesis and mild dehydration. During her admission, she received IV antibiotics and improved enough to take appropriate fluids and was sent home.

    The pertinent physical exam showed normal vital signs except for a respiratory rate of 28. She had small hamartomas of the irises, axillary freckling and 8 cafe-au-lait spots on the trunk that were more than 0.5 cm in size. There were no neurofibromas. She had decreased breath sounds with crackles bilaterally at the lung bases. The diagnosis of resolving pneumonia and neurofibromatosis was made. During rounds the medical student who was taking care of her asked what types of tumors patients with neurofibromatosis were at risk for. The residents and attending came up with neurofibromas, optic nerve and nerve sheath tumors but weren’t sure about the exact types. They also weren’t sure about malignant tumor types. The attending worked with the medical student to help perform a literature search and the next day the medical student presented his answers to the group at rounds.

    Discussion
    Neurofibromatosis Type 1 (NF1) is one of many neurocutaneous diseases with a probable underestimated incidence of 1:3000. Also called phacomatoses, a review can be found at: What is the Epidemiology and Genetics of the Major Phacomatoses?

    The key elements of NF1 are:

    • Neurofibromatosis Type 1
      • Epidemiology: 1:2500-3000 – most common phacomatosis
      • Genetics: autosomal dominant with variable penetrance, associated with chromosome 17
      • Neurological: various central nervous system tumors especially neurofibromas (often benign but may act malignant because of location or size, tumors may also degenerate into a malignant variant or cause other problems such as hypothalamic problems secondary to an optic chiasm tumor), optic nerve tumors, pheochromocytomas, mental retardation
      • Dermatological: neurofibromas, cafe-au-lait spots
      • Other clinical features: Lisch nodules of eye, other congenital anomalies may be associated including bone (rib, vertebra) and renal artery stenosis.
      • Radiological features: lesions tend to be more scattered in brain and more peripheral than tuberous sclerosis

    Patients with NF1 may have a variety of health problems including:

    • Attention deficit disorder
    • Benign and malignant neoplasms
    • Learning problems
    • Hypertension
    • Life span decreased by approximately 8 years
    • Macrocephaly
    • Orthopaedic problems – including osteoporosis and scoliosis, see also How Common Are Skeletal Problems in Patients with Neurofibromatosis?.
    • Short stature overall, precocious puberty occurs in a minority of patients.
    • Vascular lesions especially in the CNS
    • Vitamin D deficiency

    Learning Point
    The most common tumors in patients with NF1 are benign neurofibromas which can affect any organ of the body. Plexiform neurofibromas can be more diffuse and locally invasive in NF patients. The next most common neoplasms are optic nerve gliomas and brain tumors particularly gliomas, and astrocytomas of the brain stem or cerebellum, and dumbbell-shaped spinal cord tumors. Malignant peripheral nerve sheath tumors are a common cause of early death.

    Other tumors that NF patients are at increased risk for include:

  • Breast cancer
  • Hematogenous neoplasms – leukemia and other myeloid malignancies
  • Gastrointestinal stromal tumors
  • Glomus tumors
  • Malignant melanomas and other melanocyte tumors
  • Mandibular giant cell granulomas
  • Neurosarcomas
  • Pheochromocytomas
  • Tissue associated with tibial pseudoarthosis

    Questions for Further Discussion
    1. What is included in the differential diagnosis of neurofibromatosis?
    2. How is type 2 NF different from type 1 NF?
    3. What health supervision should be provided to a school age patient with NF1?

    Related Cases

      Symptom/Presentation: Pain

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Neurofibromatosis and Pneumonia.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Hersh JH; American Academy of Pediatrics Committee on Genetics. Health supervision for children with neurofibromatosis. Pediatrics. 2008 Mar;121(3):633-42.

    Pletcher BA. Type 1 Neurofibromatosis. Medscape.
    Available from the Internet at http://emedicine.medscape.com/article/1177266-overview (rev. 2/6/12, cited 7/25/13).

    Friedman, JM. Neurofibraomtosis 1. GeneReviews.
    Available from the Internet at http://www.ncbi.nlm.nih.gov/books/NBK1109/ (rev. 5/3/12, cited 7/25/13).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    4. Patient management plans are developed and carried out.
    6. Information technology to support patient care decisions and patient education is used.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.
    16. Learning of students and other health care professionals is facilitated.

  • Interpersonal and Communication Skills
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What is the Definition of Acute Pancreatitis?

    A 14-year-old male was re-admitted with emesis 1 week after surgery for acute appendicitis. After discharge he had continued to have abdominal pain and emesis that initially was 1-2 times/day, but now was 4-5 times. The emesis was non-bilious and non-bloody, and was of fluid and food only. The abdominal pain was generalized although there was some increased tenderness around the incision site. There was no radiation. He was urinating less frequently but without pain or discharge. He was passing stools that were small without pain, blood or mucous. He also had an intermittent low grade fever and diaphoresis with the emesis but not otherwise. He denied sick contacts, travel or sexual activity. The past medical history and family history were non-contributory. The review of systems showed a mild weight loss of 5 pounds but was otherwise negative.

    The pertinent physical exam showed a fatigued male with normal vital signs who was afebrile and with growth parameters in the 75-90%. HEENT showed tacky mucous membranes. Lungs and heart were normal. Abdomen revealed a healing incision, hypoactive bowel sounds, and no organomegaly or masses. There was tenderness that was more epigastric to periumbilical without radiation. There was no costovertebral angle tenderness, suprapublic tenderness or fluid wave detected. Genitourinary examination showed a Tanner V male with normal phallus and testicles. There were no hernias. Rectal examination was normal and guiac was negative.

    The laboratory evaluation of a complete blood count, and urinalysis were negative. He had a mild hypochloremic metabolic acidosis and BUN of 25 mg/dl and creatinine of 1.3 mg/dl. He had upper level normal transaminases, and total and direct bilirubin. C-reactive protein was 0.9 mg/dl and erythrocyte sedimentation rate was 28 mm/hr. His amylase and lipase were elevated at approximately 2 times normal. The radiologic evaluation of an abdominal ultrasound was negative including intact biliary system, pancreas of normal size, and a normal amount of peritoneal fluid. The diagnosis of of continued post-operative abdominal pain with increasing emesis, moderate dehydration and elevated pancreatic enzymes was made.

    The patient’s clinical course included treatment with fluid resuscitation which remedied the electrolyte abnormalities and dehydration. Over hospital day 1, he had increasing frequency of emesis and generalized pain. He was treated with analgesics and antiemetics. In the morning of hospital day 2, his pancreatic enzymes increased to ~ 2.5 x normal and later that evening he began to feel better and had decreased pain and emesis. On hospital day 3, the pancreatic enzymes had increased to ~1.8 times normal. Over the next 24 hours, he continued to improve with resolution of the emesis, and markedly decreased pain. His pancreatic enzymes were now just above normal. He was discharged home with a diagnosis of abdominal pain and emesis of unknown etiology and he had resolution of his symptoms by 1 day after discharge. His followup laboratory testing one week later was normal.

    Discussion
    Acute pancreatitis (AP) is a reversible process that involves interstitial edema, inflammatory infiltrates, hemorrhage and necrosis of the pancreas to varying degrees. AP’s incidence is increasing in recent years but the reason for the increase is unknown. The common causes of AP includes biliary abnormalities, medication, idiopathic, systemic disease, trauma, infectious, metabolic and hereditary.

    Most children with AP present with abdominal pain that is usually epigastric but may be diffuse. Radiation may occur to the back but is less common. Patients often have nausea and emesis. Abdominal distension, fever, jaundice, ascites and pleural effusions can occur. Irritability is also common in young children. The most common abdominal mass in AP are pseudocysts. Common radiographic changes include pancreatic edema, heterogeneity, and peripancreatic fluid.

    AP’s treatment is usually supportive with hydration, nutrition and analgesia. Mild AP cases resolve within 7 days. Early onset complications include shock and multi-organ system dysfunction particularly of the kidney and lung. Late onset complications are organ necrosis and pseudocyst formation. About 15-35% of patients may have a recurrence of acute pancreatitis also.

    Learning Point
    AP is a clinical entity whose generally accepted definition using the 1992 Atlanta consensus conference criteria is for the patient to have 2 of 3 criteria:

    • Abdominal pain compatable with AP
    • Elevated amylase/lipase that are 3x or more above normal
    • Anatomical changes to the pancreas on cross-sectional imaging

    Questions for Further Discussion
    1. What is included in the differential diagnosis of abdominal pain? See Acute Abdominal Pain Througout the Ages and What Causes Recurrent Abdominal Pain?
    2. How does recurrent pancreatitis differ from acute pancreatitis?
    3. What are indications for a surgical consultation in the setting of acute abdominal pain?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Abdominal Pain and Pancreatitis.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Bai HX, Lowe ME, Husain SZ. What have we learned about acute pancreatitis in children? J Pediatr Gastroenterol Nutr. 2011 Mar;52(3):262-70.

    Mekitarian Filho E, Carvalho WB, Silva FD. Acute pancreatitis in pediatrics: a systematic review of the literature. J Pediatr (Rio J). 2012 Mar-Apr;88(2):101-14.

    Dzakovic A, Superina R. Acute and chronic pancreatitis: surgical management. Semin Pediatr Surg. 2012 Aug;21(3):266-71.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What are Some Health Risks that LGBTQ Youth are at Risk For?

    Patient Presentation
    A group of residents and an attending physician were discussing adolescent sexual health. During the conversation, the question came up whether lesbian girls were at risk for pregnancy. The residents were split on the issue and the attending said she wasn’t sure how much the risk was increased, but that adolescents girls were at risk, and adolescents often were learning about their sexuality at this time, so it seemed that lesbian girls might be at the same risk or potentially even higher risk. During the clinic afternoon, the attending was able to find and circulated the newest technical report from the American Academy of Pediatrics which stated that “…sexual minority youth do not necessarily engage in sexual behaviors that are predicted by their orientation.” It also said that sexual minority youth were more likely to have had intercourse, to have started sexual intercourse before age 13 years, to have had multiple partners, and fewer were likely to use condoms and other forms of birth control/STI prevention. Girls who were having sex with other girls/women were more likely to become pregnant than girls who were only have sex with boys/men.

    Discussion
    LGBTQ (lesbian, gay, bisexual, transgender and questioning people) terminology describes sexual orientation, not sexual behavior and is a common term used. Individuals may reject this term and prefer other terminology such as a man having sex with other men (MSM) or a woman having sex with other women (WSW). Over time terminology may change also, such as the word queer that has had a negative connotation, but in contemporary usage is also used as a unifying term for anyone who is a sexual minority.

    Some sexual definitions include:

    • Sexual orientation is the sexual desire for a particular gender, or an intensive internal physiological desire for a particular gender. Homosexual, heterosexual and bisexual are examples of sexual orientation.
      Sexual orientation is stable over time, and is resistant to conscious control. It is not a choice.

    • Sexual behavior are physical behaviors that are conscious choices. These behaviors are usually motor, (e.g. “he plays with dolls”, “she’s so macho”) and may or may not change over time.
    • Gender identity is a person’s self concept of their own gender and includes an integration of the person’s biological sex, gender role expression and sexual orientation.
    • Gay refers to a male whose primary, loving, sexual and/or intimate relationships are with men.
    • Lesbian refers to a woman whose primary, loving, sexual and/or intimate relationships are with women.
    • Bisexual refers to a person who is sexually, emotionally and/or intimately attracted to either sex but not necessarily at the same time.
    • Transgender is a general term for crossing gender lines. It may refer to persons who are transsexuals or transvestites/cross-dressers.
    • Questioning refers to an individual who is not sure about their sexual orientation.

    For additional information about LGBTQ youth, see How Can I Make My Sexual Interview More Gender Neutral?

    Learning Point
    LGBTQ youth in general have good physical and mental health and like all youth should be provided comprehensive primary and specialty care.
    LGBTQ youth do have certain risk factors based on current research and therefore should be screened and treated as appropriate. There is signficantly less research for transgender youth compared to other sexual minority groups.

    • Mental health – screening for mood disorders, depression and suicide risk and body image/eating disorders is usually part of comprehensive care, but special attention may be needed for LGBTQ youth. Lesbian girls may be more contented with their bodies than heterosexual girls, but gay boys may be less contented than their heterosexual counterparts.
    • Physical health – obesity is screened for in all youth but LGBTQ are at higher risk
    • Sexual Health
      • Pregnancy – increased risk for lesbian girls relative to heterosexual girls – screening and testing as appropriate
      • Sexually transmitted infections – increased risk for both gay and lesbian youth, HIV/AIDs is specifically higher in gay youth.
        It is recommended to screen yearly if in a monogamous relationship that has 100% condom use and no substance abuse, otherwise more frequent screening is recommended.

        • Females – follow CDC guidelines and also consider specific screening for HIV, HSV, HPV, Chlamydia, Syphilis, and bacterial vaginosis
        • Males – follow CDC guidelines and strongly consider specific screening for Hepatitis B, HIV, HSV, HPV, Chlamydia, Neisseria, and Syphilis. Hepatitis C should be screened for if current or past drug user or HIV positive.
    • Prevention
      • Condoms, dental dams should be promoted for any contacts
      • HPV vaccine should also be promoted for all youth
      • Birth control and emergency contraceptive should be discussed and provided to youth

    Questions for Further Discussion
    1. What role does family acceptance of LGBTQ youth provide?
    2. What are some ways a primary care office can show adolescents that it is open to discussing sexual issues?
    3. What are your local laws for providing treatment and counseling for sexual health issues such as pregnancy, birth control, STIs for adolescents?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Ryan C, Russell ST, Huebner D, Diaz R, Sanchez J. Family acceptance in adolescence and the health of LGBT young adults. J Child Adolesc Psychiatr Nurs. 2010 Nov;23(4):205-13.

    The Health of Lesbian, Gay, Bisexual, and Transgender People: Building a Foundation for Better Understanding.
    Institute of Medicine (US) Committee on Lesbian, Gay, Bisexual, and Transgender Health Issues and Research Gaps and Opportunities.
    Washington (DC): National Academies Press (US); 2011.

    Kincaid C, Jones DJ, Sterrett E, McKee L. A review of parenting and adolescent sexual behavior: the moderating role of gender. Clin Psychol Rev. 2012 Apr;32(3):177-88.

    Levin DA and the Committee on Adolescence. Office-Based Care for Lesbian, Gay, Bisexual, Transgender, and Questioning Youth. Pediatrics. 2013;132:e297 -e313

    ACGME Competencies Highlighted by Case

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.
    16. Learning of students and other health care professionals is facilitated.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital