-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVE: To develop a concise and statistically robust instrument to assess autonomic symptoms that provides clinically relevant scores of autonomic symptom severity based on the well-established 169-item Autonomic Symptom Profile (ASP) and its validated 84-question scoring instrument, the Composite Autonomic Symptom Score (COMPASS). PATIENTS AND METHODS: We assessed the internal consistency of COMPASS using Cronbach α coefficients based on the ASP of 405 healthy control subjects recruited and seen in the Mayo Clinic Autonomic Disorders Center between March 1, 1995, and March 31, 2010. Applying a simplified scoring algorithm, we then used exploratory factor analysis with orthogonal rotation and eigenvalue calculations to extract internally consistent domains and to reduce dimensionality. This analysis was followed by expert revisions to eliminate redundant content and to retain clinically important questions and final assessment of the new instrument. RESULTS: The new simplified scoring algorithm alone resulted in higher Cronbach α values in all domains. Factor analysis revealed 7 domains with a total of 54 questions retained. Expert revisions resulted in further reduction of questions and domains with a remaining total of 31 questions in 6 domains (COMPASS 31). Measures of internal consistency were much improved compared to those for COMPASS. Following appropriate weighting, this instrument provides an autonomic symptom score from 0 to 100. CONCLUSION: COMPASS 31 is a refined, internally consistent, and markedly abbreviated quantitative measure of autonomic symptoms. It is based on the original ASP and COMPASS, applies a much simplified scoring algorithm, and is suitable for widespread use in autonomic research and practice.
Mayo Clinic Proceedings 12/2012; 87(12):1196-1201. · 5.70 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVE: Mutations in SCN9A have been reported in (1) congenital insensitivity to pain (CIP); (2) primary erythromelalgia; (3) paroxysmal extreme pain disorder; (4) febrile seizures and recently (5) small fibre sensory neuropathy. We sought to investigate for SCN9A mutations in a clinically well-characterised cohort of patients with CIP and erythromelalgia. METHODS: We sequenced all exons of SCN9A in 19 clinically well-studied cases including 6 CIP and 13 erythromelalgia (9 with family history, 10 with small-fibre neuropathy). The identified variants were assessed in dbSNP135, 1K genome, NHLBI-Exome Sequencing Project (5400-exomes) databases, and 768 normal chromosomes. RESULTS: In erythromelalgia case 7, we identified a novel Q10>K mutation. In CIP case 6, we identified a novel, de novo splicing mutation (IVS8-2A>G); this splicing mutation compounded with a nonsense mutation (R523>X) and abolished SCN9A mRNA expression almost completely compared with his unaffected father. In CIP case 5, we found a variant (P610>T) previously considered causal for erythromelalgia, supporting recently raised doubt on its causal nature. We also found a splicing junction variant (IVS24-7delGTTT) in all 19 patients, this splicing variant was previously considered casual for CIP, but IVS24-7delGTTT was in fact the major allele in Caucasian populations. CONCLUSIONS: Two novel SCN9A mutations were identified, but frequently polymorphism variants are found which may provide susceptibility factors in pain modulation. CIP and erythromelalgia are defined as genetically heterogeneous, and some SCN9A variants previously considered causal may only be modifying factors.
Journal of neurology, neurosurgery, and psychiatry 11/2012; · 4.87 Impact Factor
-
-
[show abstract]
[hide abstract]
ABSTRACT: To study the frequency and degree of deconditioning, clinical features, and relationship between deconditioning and autonomic parameters in patients with orthostatic intolerance.
We retrospectively studied all patients seen for orthostatic intolerance at Mayo Clinic between January 2006 and June 2011, who underwent both standardized autonomic and exercise testing.
A total of 184 patients (84 with postural orthostatic tachycardia syndrome [POTS] and 100 without orthostatic tachycardia) fulfilled the inclusion criteria. Of these, 89% were women, and median age was 27.5 years (interquartile range [IQR] 22-37 years). Symptom duration was 4 years (IQR 2-7.8). Of the patients, 90% had deconditioning (reduced maximum oxygen uptake [VO(2max)%] <85%) during exercise. This finding was unrelated to age, gender, or duration of illness. The prevalence of deconditioning was similar between those with POTS (95%) and those with orthostatic intolerance (91%). VO(2max)% had a weak correlation with a few autonomic and laboratory parameters but adequate predictors of VO(2max)% could not be identified.
Reduced VO(2max)% consistent with deconditioning is present in almost all patients with orthostatic intolerance and may play a central role in pathophysiology. This finding provides a strong rationale for retraining in the treatment of orthostatic intolerance. None of the autonomic indices are reliable predictors of deconditioning.
Neurology 09/2012; 79(14):1435-9. · 8.31 Impact Factor
-
Peter J Dyck,
Carol J Overland, Phillip A Low,
William J Litchy,
Jenny L Davies,
P James B Dyck,
Rickey E Carter,
L Joseph Melton,
Henning Andersen,
James W Albers, [......],
John D England,
Christopher J Klein,
Gareth Llewelyn,
Michelle L Mauermann,
James W Russell,
Dinesh Selvarajah,
Wolfgang Singer,
A Gordon Smith,
Solomon Tesfaye,
Adrian Vella
[show abstract]
[hide abstract]
ABSTRACT: OBJECTIVE To repeat the Clinical vs Neurophysiology (Cl vs N Phys) trial using "unequivocally abnormal" signs and symptoms (Trial 2) compared with the earlier trial (Trial 1), which used "usual" signs and symptoms. DESIGN Standard and referenced nerve conduction abnormalities were used in both Trials 1 and 2 as the standard criterion indicative of diabetic sensorimotor polyneuropathy. Physician proficiency (accuracy among evaluators) was compared between Trials 1 and 2. SETTING Academic medical centers in Canada, Denmark, England, and the United States. PARTICIPANTS Thirteen expert neuromuscular physicians. One expert was replaced in Trial 2. RESULTS The marked overreporting, especially of signs, in Trial 1 was avoided in Trial 2. Reproducibility of diagnosis between days 1 and 2 was significantly (P = .005) better in Trial 2. The correlation of the following clinical scores with composite nerve conduction measures spanning the range of normality and abnormality was improved in Trial 2: pinprick sensation (P = .03), decreased reflexes (P = .06), touch-pressure sensation (P = .06), and the sum of symptoms (P = .06). CONCLUSIONS The simple pretrial decision to use unequivocally abnormal signs and symptoms-taking age, sex, and physical variables into account-in making clinical judgments for the diagnosis of diabetic sensorimotor polyneuropathy (Trial 2) improves physician proficiency compared with use of usual elicitation of signs and symptoms (Trial 1); both compare to confirmed nerve conduction abnormality.
Archives of neurology 09/2012; · 6.31 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To prospectively evaluate patients who met standard criteria for postural tachycardia syndrome (POTS), at baseline and 1-year follow-up, using standard clinical and laboratory methods to assess autonomic function.
Fifty-eight patients met the study criteria (orthostatic symptoms and a heart rate increment of ≥ 30 beats/min on head-up tilt) and completed 12 months of follow-up. All patients were enrolled and completed the study from January 16, 2006, through April 15, 2009. Patients underwent standardized autonomic testing, including head-up tilt, clinical assessment, and validated questionnaires designed to determine the severity of autonomic symptoms.
Patients were predominantly young females (n=49, 84%), with 20 patients (34%) reporting an antecedent viral infection before onset of symptoms. More than one-third (37%) no longer fulfilled tilt criteria for POTS on follow-up, although heart rate increment on head-up tilt did not differ significantly at 1 year (33.8 ± 15.1 beats/min) compared with baseline (37.8 ± 14.6 beats/min) for the entire cohort. Orthostatic symptoms improved in most patients. Autonomic dysfunction was mild as defined by a Composite Autonomic Severity Score of 3 or less in 55 patients (95%) at baseline and 48 patients (92%) at 1 year.
To our knowledge, this is the first prospective study of the clinical outcomes of patients with POTS. Orthostatic symptoms improved in our patients, with more than one-third of patients no longer fulfilling tilt criteria for POTS, although the overall group change in heart rate increment was modest. Our data are in keeping with a relatively favorable prognosis in most patients with POTS.
Mayo Clinic Proceedings 07/2012; 87(8):746-52. · 5.70 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We report the result of a follow-up, open-label study of pyridostigmine for treatment of orthostatic hypotension. Aim of the study was to evaluate self-reported efficacy and patients satisfaction with the medication. Most patients were very satisfied with the medication and chose to continue it.
Clinical Autonomic Research 04/2012; 15(1):51-53. · 1.30 Impact Factor
-
Valeria Iodice,
Axel Lipp,
J Eric Ahlskog,
Paola Sandroni,
Robert D Fealey,
Joseph E Parisi,
Joseph Y Matsumoto,
Eduardo E Benarroch,
Kurt Kimpinski,
Wolfgang Singer,
Tonette L Gehrking,
Jade A Gehrking,
David M Sletten,
Ann M Schmeichel,
James H Bower,
Sid Gilman,
Juan Figueroa, Phillip A Low
[show abstract]
[hide abstract]
ABSTRACT: Multiple system atrophy (MSA) is a sporadic progressive neurodegenerative disorder characterised by autonomic failure, manifested as orthostatic hypotension or urogenital dysfunction, with combinations of parkinsonism that is poorly responsive to levodopa, cerebellar ataxia and corticospinal dysfunction. Published autopsy confirmed cases have provided reasonable neurological characterisation but have lacked adequate autonomic function testing.
To retrospectively evaluate if the autonomic characterisation of MSA is accurate in autopsy confirmed MSA and if consensus criteria are validated by autopsy confirmation.
29 autopsy confirmed cases of MSA evaluated at the Mayo Clinic who had undergone formalised autonomic testing, including adrenergic, sudomotor and cardiovagal functions and Thermoregulatory Sweat Test (TST), from which the Composite Autonomic Severity Score (CASS) was derived, were included in the study.
Patient characteristics: 17 men, 12 women; age of onset 57±8.1 years; disease duration to death 6.5±3.3 years; first symptom autonomic in 18, parkinsonism in seven and cerebellar in two. Clinical phenotype at first visit was MSA-P (predominant parkinsonism) in 18, MSA-C (predominant cerebellar involvement) in eight, pure autonomic failure in two and Parkinson's disease in one. Clinical diagnosis at last visit was MSA for 28 cases. Autonomic failure was severe: CASS was 7.2±2.3 (maximum 10). TST% was 65.6±33.9% and exceeded 30% in 82% of patients. The most common pattern was global anhidrosis. Norepinephrine was normal supine (203.6±112.7) but orthostatic increment of 33.5±23.2% was reduced. Four clinical features (rapid progression, early postural instability, poor levodopa responsiveness and symmetric involvement) were common.
The pattern of severe and progressive generalised autonomic failure with severe adrenergic and sudomotor failure combined with the clinical phenotype is highly predictive of MSA.
Journal of neurology, neurosurgery, and psychiatry 04/2012; 83(4):453-9. · 4.87 Impact Factor
-
Kurt Kimpinski,
Valeria Iodice,
Duane D Burton,
Michael Camilleri,
Brian P Mullan,
Axel Lipp,
Paola Sandroni,
Tonette L Gehrking,
David M Sletten,
J E Ahlskog,
Robert D Fealey,
Wolfgang Singer, Phillip A Low
[show abstract]
[hide abstract]
ABSTRACT: Differentiation of idiopathic Parkinson's disease (PD) from multiple system atrophy (MSA) can be difficult. Methods devised to help distinguish the two disorders include standardized autonomic testing and cardiac imaging with iodine-123 meta-iodobenzylguanidine myocardial scintigraphy. MSA patients had more severe adrenergic and overall autonomic dysfunction when compared to control and PD patients. Area of anhidrosis on thermoregulatory sweat test was greater in MSA (67.4±12.42, p<0.001) versus PD patients (area of anhidrosis, 1.7±2.96). Postganglionic cardiac sympathetic innervation (iodine-123 meta-iodobenzylguanidine) expressed as heart to mediastinal ratio was significantly lower in Parkinson's disease patients (1.4±0.40, p=0.025) compared to controls (2.0±0.29), but not in multiple system atrophy (2.0±0.76). These findings indicate that autonomic dysfunction is generalized and predominantly preganglionic in multiple system atrophy, and postganglionic in Parkinson's disease. In our hands the thermoregulatory sweat test provides the best distinction between MSA and PD. However further confirmatory studies using larger patient numbers are required. Currently a combination of clinical judgment and autonomic testing is recommended to help differentiate MSA and PD.
Journal of the neurological sciences 03/2012; 317(1-2):92-6. · 2.32 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To evaluate whether the use of adult heart rate (HR) criteria is appropriate for diagnosing orthostatic intolerance (OI) and postural tachycardia syndrome (POTS) in children and adolescents, and to establish normative data and diagnostic criteria for pediatric OI and POTS.
A total of 106 normal controls aged 8-19 years (mean age, 14.5±3.3 years) underwent standardized autonomic testing, including 5 minutes of 70-degree head-up tilt. The orthostatic HR increment and absolute orthostatic HR were assessed and retrospectively compared with values in 654 pediatric patients of similar age (mean age, 15.5±2.3 years) who were referred to our Clinical Autonomic Laboratory with symptoms of OI.
The HR increment was mildly higher in patients referred for OI/POTS, but there was considerable overlap between the patient and control groups. Some 42% of the normal controls had an HR increment of ≥30 beats per minute. The 95th percentile for the orthostatic HR increment in the normal controls was 42.9 beats per minute. There was a greater and more consistent difference in absolute orthostatic HR between the 2 groups, although there was still considerable overlap.
The diagnostic criteria for OI and POTS in adults are unsuitable for children and adolescents. Based on our normative data, we propose new criteria for the diagnosis of OI and POTS in children and adolescents.
The Journal of pediatrics 02/2012; 160(2):222-6. · 4.02 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We have previously shown that sudomotor dysfunction in autoimmune autonomic ganglionopathy is severe, widespread, and predominantly post-ganglionic. However, the long-term changes in sudomotor function have not been studied in detail. Our objective was to characterize the long-term changes in sudomotor dysfunction in patients with autoimmune autonomic ganglionopathy.
Changes in sudomotor function were compared in a cohort of nine α3 nAChR antibody positive autoimmune autonomic ganglionopathy patients over an approximate 5-year period. Standard measurements of sudomotor function were used including the thermoregulatory sweat test and quantitative sudomotor axon reflex test.
Total body anhidrosis on thermoregulatory sweat testing showed improvement in four of nine patients. Quantitative sudomotor axon reflex testing for both forearm and foot sites was variable with four of nine patients showing improvement in total sweat output. Distribution of sudomotor dysfunction at follow-up was post-ganglionic in seven of nine patients at the foot site and three of nine patients at the forearm site. Overall, sudomotor dysfunction was post-ganglionic in seven of nine patients throughout the follow-up period (62.4 ± 19.4 months).
Sudomotor dysfunction in autoimmune autonomic ganglionopathy was severe and widespread throughout the follow-up period for the majority of patients studied. Sudomotor dysfunction was predominantly post-ganglionic throughout the follow-up period.
Clinical Autonomic Research 11/2011; 22(3):131-6. · 1.30 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To investigate whether patients with postural orthostatic tachycardia syndrome (POTS) developed unexpected perioperative complications.
Retrospective case series.
Academic medical center.
The records of 13 patients with POTS, who underwent surgical procedures during general anesthesia, were studied. Details of disease management, anesthetic induction, hemodynamic response to induction and intubation, intraoperative course, and immediate postoperative management were analyzed.
Three patients developed prolonged intraoperative hypotension, which was not associated with induction of anesthesia. All 13 patients were successfully treated and they recovered without complications. There were no unplanned hospital or intensive care admissions.
Intraoperative hypotension, but not tachycardia, was observed in three of 13 patients with POTS who received general anesthesia for a variety of surgical procedures using multiple medications and techniques.
Journal of clinical anesthesia 08/2011; 23(5):384-92. · 1.32 Impact Factor
-
Dan Ziegler, Phillip A Low,
William J Litchy,
Andrew J M Boulton,
Aaron I Vinik,
Roy Freeman,
Rustem Samigullin,
Hans Tritschler,
Ullrich Munzel,
Joachim Maus,
Klemens Schütte,
Peter J Dyck
[show abstract]
[hide abstract]
ABSTRACT: To evaluate the efficacy and safety of α-lipoic acid (ALA) over 4 years in mild-to-moderate diabetic distal symmetric sensorimotor polyneuropathy (DSPN). RESEARCH DESIGN AND METHODS In a multicenter randomized double-blind parallel-group trial, 460 diabetic patients with mild-to-moderate DSPN were randomly assigned to oral treatment with 600 mg ALA once daily (n = 233) or placebo (n = 227) for 4 years. Primary end point was a composite score (Neuropathy Impairment Score [NIS]-Lower Limbs [NIS-LL] and seven neurophysiologic tests). Secondary outcome measures included NIS, NIS-LL, nerve conduction, and quantitative sensory tests (QSTs).
Change in primary end point from baseline to 4 years showed no significant difference between treatment groups (P = 0.105). Change from baseline was significantly better with ALA than placebo for NIS (P = 0.028), NIS-LL (P = 0.05), and NIS-LL muscular weakness subscore (P = 0.045). More patients showed a clinically meaningful improvement and fewer showed progression of NIS (P = 0.013) and NIS-LL (P = 0.025) with ALA than with placebo. Nerve conduction and QST results did not significantly worsen with placebo. Global assessment of treatment tolerability and discontinuations due to lack of tolerability did not differ between the groups. The rates of serious adverse events were higher on ALA (38.1%) than on placebo (28.0%).
Four-year treatment with ALA in mild-to-moderate DSPN did not influence the primary composite end point but resulted in a clinically meaningful improvement and prevention of progression of neuropathic impairments and was well tolerated. Because the primary composite end point did not deteriorate significantly in placebo-treated subjects, secondary prevention of its progression by ALA according to the trial design was not feasible.
Diabetes care 07/2011; 34(9):2054-60. · 8.09 Impact Factor
-
Roy Freeman,
Wouter Wieling,
Felicia B Axelrod,
David G Benditt,
Eduardo Benarroch,
Italo Biaggioni,
William P Cheshire,
Thomas Chelimsky,
Pietro Cortelli,
Christopher H Gibbons, [......],
Benjamin D Levine, Phillip A Low,
Christopher Mathias,
Satish R Raj,
David Robertson,
Paola Sandroni,
Irwin J Schatz,
Ron Schondorf,
Julian M Stewart,
J Gert van Dijk
Autonomic neuroscience: basic & clinical 03/2011; 161(1-2):46-8. · 1.82 Impact Factor
-
Roy Freeman,
Wouter Wieling,
Felicia B Axelrod,
David G Benditt,
Eduardo Benarroch,
Italo Biaggioni,
William P Cheshire,
Thomas Chelimsky,
Pietro Cortelli,
Christopher H Gibbons, [......],
Benjamin D Levine, Phillip A Low,
Christopher Mathias,
Satish R Raj,
David Robertson,
Paola Sandroni,
Irwin Schatz,
Ron Schondorff,
Julian M Stewart,
J Gert van Dijk
Clinical Autonomic Research 03/2011; 21(2):69-72. · 1.30 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Quantitative Sudomotor Axon Reflex Testing is a useful measure of post ganglionic sudomotor function. The test is based on the iontophoresis of an acetylcholine solution which induces a local sweat response. We have previously described a gel-based vehicle that may provide another option for the iontophoresis of acetylcholine. It was our objective to compare the influence of the vehicle (gel versus solution) on sudomotor recordings and perceived discomfort. Results show gel-based vehicles are very similar to solution-based vehicles during Quantitative Sudomotor Axon Reflex Testing.
Autonomic neuroscience: basic & clinical 12/2010; 158(1-2):123-6. · 1.82 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The periaqueductal gray (PAG) consists of distinct columns that participate in the integrated control of autonomic function. We sought to determine whether the PAG is affected in multiple system atrophy (MSA), a disorder characterized by prominent autonomic failure. Brains were obtained at autopsy from 13 MSA patients (10 M, 3 F, age 61±3 years) and 13 controls (8 M, 5 F, age 67±4 years). Transverse formalin-fixed 50 μm sections were obtained throughout the PAG and immunostained for the vesicular transporter 2 (VGLUT-2), nitric oxide synthase (NOS), or α-synuclein and co-stained with thionin. Some sections were processed for myelin or astrocyte staining. Stereological quantitation was performed separately in the ventrolateral, lateral, dorsolateral, and dorsomedial columns of the PAG. In MSA cases, there was a decrease in the total estimated number of VGLUT-2 immunoreactive neurons in the ventrolateral, lateral, and dorsomedial and to a lesser extent dorsolateral PAG compared to controls (ventrolateral PAG: 16,299±1612 vs. 27,906±2480 respectively, p<0.01; lateral PAG: 11,004±1401 vs. 16,078±1140 respectively, p<0.05; and dorsomedial PAG: 8847±1052 vs. 15,412±1097 respectively, p<0.001). The number of NOS immunoreactive neurons in the dorsolateral PAG was similar to controls. In all columns, the number of non-immunolabelled Nissl-stained cells was similar between groups. There was accumulation of glial cytoplasmic inclusions in all PAG columns in MSA. Our findings indicate involvement of the PAG columns in MSA, which may contribute to autonomic disturbances in this disorder.
Autonomic neuroscience: basic & clinical 12/2010; 158(1-2):111-7. · 1.82 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The α-synucleinopathy multiple system atrophy (MSA) and diseases defined by pathological 43-kDa transactive response DNA-binding protein (TDP-43) or fused in sarcoma (FUS) aggregates such as amyotrophic lateral sclerosis and frontotemporal lobar degeneration show overlapping clinico-pathological features. Consequently, we examined MSA for evidence of TDP-43 or FUS pathology utilizing immunohistochemical studies in autopsy material from 29 MSA patients.
TDP-43 pathology was generally rare, and there were no FUS lesions. The TDP-43 lesions were located predominantly in medio-temporal lobe and subcortical brain areas and were comprised mainly of dystrophic processes and perivascular (and subpial) lesions.
The multisystem clinical symptoms and signs of MSA, and in particular the neurobehavioural/cognitive and pyramidal features, appear not to result from concomitant TDP-43 or FUS pathology, but rather from widespread white matter α-synuclein positive glial cytoplasmic inclusions and neurodegeneration in keeping with a primary α-synuclein-mediated oligodendrogliopathy. The gliodegenerative disease MSA evidently results from different pathogenetic mechanisms than neurodegenerative diseases linked to pathological TDP-43.
Neuropathology and Applied Neurobiology 10/2010; 37(4):358-65. · 3.80 Impact Factor
-
Peter J Dyck,
Carol J Overland, Phillip A Low,
William J Litchy,
Jenny L Davies,
P James B Dyck,
Peter C O'Brien,
James W Albers,
Henning Andersen,
Charles F Bolton,
John D England,
Christopher J Klein,
J Gareth Llewelyn,
Michelle L Mauermann,
James W Russell,
Wolfgang Singer,
A Gordon Smith,
Solomon Tesfaye,
Adrian Vella
[show abstract]
[hide abstract]
ABSTRACT: The purpose was to test whether physicians can validly and reproducibly diagnose diabetic sensorimotor polyneuropathy (DSPN). Twelve physicians assessed 24 patients with diabetes mellitus (DM) on consecutive days (576 examinations) with physical features and voice disguised. Results were compared to gold standard 75% group diagnosis (dx) and a nerve conduction score (Sigma5 NC nds). Masking of patients was achieved. Reproducibility measured by the kappa coefficient and compared to Sigma5 NC nd varied considerably among physicians: median and ranges: signs 0.8 (0.32-1.0); symptoms 0.79 (0.36-1.0), and diagnoses 0.47 (0.33-0.84), both low and high scores indicating poor performance. There was substantial agreement between 75% group dx and confirmed NC abnormality (abn). As compared to Sigma5 NC, individual physicians' clinical dx was excessively variable and frequently inaccurate. Study physician dx from signs and symptoms were excessively variable, often overestimating DSPN. Specific approaches to improving clinical proficiency should be tested.
Muscle & Nerve 08/2010; 42(2):157-64. · 2.37 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: To compare the clinical presentation, autonomic dysfunction, and pregnancy outcomes in parous and nulliparous women with postural tachycardia syndrome (POTS) and in women with POTS before and after pregnancy.
This study consists of women who had at least 1 pregnancy during which time they met criteria for POTS between May 1993 and July 2009. All patients underwent standard autonomic testing. POTS was defined as a heart rate (HR) increase of greater than 30 beats/min on head-up tilt (HUT) with symptoms of orthostatic intolerance. Patients' charts were reviewed retrospectively to determine pregnancy outcomes.
Clinical characteristics related to POTS did not differ between parous and nulliparous women except for disease duration (parous, 3.7+/-2.6; nulliparous, 2.1+/-2.2; P<.001). Autonomic dysfunction did not differ between groups (change in HR on HUT: parous, 42.6+/-12.0 beats/min; nulliparous, 41.3+/-10.6 beats/min; P=.39). Of 116 total pregnancies, adverse pregnancy outcomes were reported in 9% and maternal complications in 1%. No complication was related to POTS. There was a trend toward modest improvement in autonomic dysfunction before and after pregnancy (change in HR on HUT: before pregnancy, 38.1+/-22.7 beats/min; after pregnancy, 21.9+/-14.9 beats/min; P=.07).
The long-term impact of pregnancy on POTS does not appear to be clinically important. However, there does appear to be a trend toward improvement in the short-term postpartum period. Adverse pregnancy events were similar to those seen in the general public and do not present a barrier to women with POTS who want to have children.
Mayo Clinic Proceedings 07/2010; 85(7):639-44. · 5.70 Impact Factor
