Osmotic demyelination syndrome in a patient with Noonan syndrome and anterior hypopituitarism

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Abstract

Summary

Severe hyponatremia and osmotic demyelination syndrome (ODS) are opposite ends of a spectrum of emergency disorders related to sodium concentrations. Management of severe hyponatremia is challenging because of the difficulty in balancing the risk of overcorrection leading to ODS as well as under-correction causing cerebral oedema, particularly in a patient with chronic hypocortisolism and hypothyroidism. We report a case of a patient with Noonan syndrome and untreated anterior hypopituitarism who presented with symptomatic hyponatremia and developed transient ODS.

Learning points:

Patients with severe anterior hypopituitarism with severe hyponatremia are susceptible to the rapid rise of sodium level with a small amount of fluid and hydrocortisone.
These patients with chronic anterior hypopituitarism are at high risk of developing ODS and therefore, care should be taken to avoid a rise of more than 4–6 mmol/L per day.
Early recognition and rescue desmopressin and i.v. dextrose 5% fluids to reduce serum sodium concentration may be helpful in treating acute ODS.

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Most cited references 14

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Osmotic demyelination syndrome following correction of hyponatremia.

Richard Sterns, J Riggs, S Schochet (1986)

The treatment of hyponatremia is controversial: some authorities have cautioned that rapid correction causes central pontine myelinolysis, and others warn that severe hyponatremia has a high mortality rate unless it is corrected rapidly. Eight patients treated over a five-year period at our two institutions had a neurologic syndrome with clinical or pathological findings typical of central pontine myelinolysis, which developed after the patients presented with severe hyponatremia. Each patient's condition worsened after relatively rapid correction of hyponatremia (greater than 12 mmol of sodium per liter per day)--a phenomenon that we have called the osmotic demyelination syndrome. Five of the patients were treated at one hospital, and accounted for all the neurologic complications recorded among 60 patients with serum sodium concentrations below 116 mmol per liter; no patient in whom the sodium level was raised by less than 12 mmol per liter per day had any neurologic sequelae. Reviewing published reports on patients with very severe hyponatremia (serum sodium less than 106 mmol per liter) revealed that neurologic sequelae were associated with correction of hyponatremia by more than 12 mmol per liter per day; when correction proceeded more slowly, patients had uneventful recoveries. We suggest that the osmotic demyelination syndrome is a preventable complication of overly rapid correction of chronic hyponatremia.

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Diagnosis and treatment of hyponatremia: a systematic review of clinical practice guidelines and consensus statements

Evi V Nagler, Jill Vanmassenhove, Sabine N van der Veer … (2014)

Background Hyponatremia is a common electrolyte disorder. Multiple organizations have published guidance documents to assist clinicians in managing hyponatremia. We aimed to explore the scope, content, and consistency of these documents. Methods We searched MEDLINE, EMBASE, and websites of guideline organizations and professional societies to September 2014 without language restriction for Clinical Practice Guidelines (defined as any document providing guidance informed by systematic literature review) and Consensus Statements (any other guidance document) developed specifically to guide differential diagnosis or treatment of hyponatremia. Four reviewers appraised guideline quality using the 23-item AGREE II instrument, which rates reporting of the guidance development process across six domains: scope and purpose, stakeholder involvement, rigor of development, clarity of presentation, applicability, and editorial independence. Total scores were calculated as standardized averages by domain. Results We found ten guidance documents; five clinical practice guidelines and five consensus statements. Overall, quality was mixed: two clinical practice guidelines attained an average score of >50% for all of the domains, three rated the evidence in a systematic way and two graded strength of the recommendations. All five consensus statements received AGREE scores below 60% for each of the specific domains. The guidance documents varied widely in scope. All dealt with therapy and seven included recommendations on diagnosis, using serum osmolality to confirm hypotonic hyponatremia, and volume status, urinary sodium concentration, and urinary osmolality for further classification of the hyponatremia. They differed, however, in classification thresholds, what additional tests to consider, and when to initiate diagnostic work-up. Eight guidance documents advocated hypertonic NaCl in severely symptomatic, acute onset ( 48 h) or asymptomatic cases, recommended treatments were NaCl 0.9%, fluid restriction, and cause-specific therapy for hypovolemic, euvolemic, and hypervolemic hyponatremia, respectively. Eight guidance documents recommended limits for speed of increase of sodium concentration, but these varied between 8 and 12 mmol/L per 24 h. Inconsistencies also existed in the recommended dose of NaCl, its initial infusion speed, and which second line interventions to consider. Conclusions Current guidance documents on the assessment and treatment of hyponatremia vary in methodological rigor and recommendations are not always consistent. Electronic supplementary material The online version of this article (doi:10.1186/s12916-014-0231-1) contains supplementary material, which is available to authorized users.

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Desmopressin to Prevent Rapid Sodium Correction in Severe Hyponatremia: A Systematic Review

Thomas E. MacMillan, Terence Tang, Rodrigo Cavalcanti (2015)

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Author and article information

Journal

Journal ID (nlm-ta): Endocrinol Diabetes Metab Case Rep

Journal ID (iso-abbrev): Endocrinol Diabetes Metab Case Rep

Journal ID (hwp): EDM

Title: Endocrinology, Diabetes & Metabolism Case Reports

Publisher: Bioscientifica Ltd (Bristol )

ISSN (Electronic): 2052-0573

Publication date (Electronic): 20 August 2020

Publication date Collection: 2020

Volume: 2020

Electronic Location Identifier: 20-0039

Affiliations

[1 ]Department of Endocrinology , Changi General Hospital, Singapore

Author notes

Correspondence should be addressed to T Chua; Email: tzyharn.chua@ 123456mohh.com.sg

Article

Publisher ID: EDM200039

DOI: 10.1530/EDM-20-0039

PMC ID: 7487176

SO-VID: e17a2cfe-21f2-47a1-b148-1053731d67f0

License:

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License..

History

Date received : 18 May 2020

Date accepted : 29 July 2020

Categories

Subject: Adult

Subject: Male

Subject: Asian - Other

Subject: Singapore

Subject: Pituitary

Subject: GH

Subject: Thyroxine (T4)

Subject: Triiodothyronine (T3)

Subject: TSH

Subject: Cortisol

Subject: ACTH

Subject: FSH

Subject: LH

Subject: Testosterone

Subject: IGF1

Subject: Noonan syndrome

Subject: Hypopituitarism

Subject: Hyponatraemia

Subject: Pituitary adenoma

Subject: Microadenoma

Subject: Hypopituitarism

Subject: Hyponatraemia

Subject: Dizziness

Subject: Headache

Subject: Insomnia

Subject: Nausea

Subject: Abdominal pain

Subject: Vomiting

Subject: Hypotension

Subject: Kyphoscoliosis

Subject: Gynaecomastia

Subject: Ears - low set

Subject: Neck - short

Subject: Hypogonadism

Subject: Short stature

Subject: Neck - loose skin (nape)

Subject: Hypothyroidism

Subject: Seizures

Subject: Hypotonia

Subject: Aphasia

Subject: Osteoporosis

Subject: Sodium

Subject: MRI

Subject: Blood pressure

Subject: Serum osmolality

Subject: Urine osmolality

Subject: GH

Subject: FT3

Subject: FT4

Subject: TSH

Subject: Cortisol

Subject: Cortisol (9am)

Subject: Cortisol (serum)

Subject: ACTH

Subject: FSH

Subject: LH

Subject: Testosterone

Subject: IGF1

Subject: Bone mineral density

Subject: X-ray

Subject: Chloride

Subject: Haemoglobin

Subject: 25-hydroxyvitamin-D3

Subject: Sex hormone binding globulin

Subject: Fluid repletion

Subject: Saline

Subject: Hydrocortisone

Subject: Glucocorticoids

Subject: Glucose

Subject: Desmopressin

Subject: Levothyroxine

Subject: Valproic acid

Subject: Neurology

Subject: Unique/Unexpected Symptoms or Presentations of a Disease

Keywords: adult,male,asian - other,singapore,pituitary,gh,thyroxine (t4),triiodothyronine (t3),tsh,cortisol,acth,fsh,lh,testosterone,igf1,noonan syndrome,hypopituitarism,hyponatraemia,pituitary adenoma,microadenoma,dizziness,headache,insomnia,nausea,abdominal pain,vomiting,hypotension,kyphoscoliosis,gynaecomastia,ears - low set,neck - short,hypogonadism,short stature,neck - loose skin (nape),hypothyroidism,seizures,hypotonia,aphasia,osteoporosis,sodium,mri,blood pressure,serum osmolality,urine osmolality,ft3,ft4,cortisol (9am),cortisol (serum),bone mineral density,x-ray,chloride,haemoglobin ,25-hydroxyvitamin-d3,sex hormone binding globulin,fluid repletion,saline,hydrocortisone,glucocorticoids,glucose,desmopressin,levothyroxine,valproic acid,neurology,unique/unexpected symptoms or presentations of a disease,august,2020

Data availability:

Keywords: adult, male, asian - other, singapore, pituitary, gh, thyroxine (t4), triiodothyronine (t3), tsh, cortisol, acth, fsh, lh, testosterone, igf1, noonan syndrome, hypopituitarism, hyponatraemia, pituitary adenoma, microadenoma, dizziness, headache, insomnia, nausea, abdominal pain, vomiting, hypotension, kyphoscoliosis, gynaecomastia, ears - low set, neck - short, hypogonadism, short stature, neck - loose skin (nape), hypothyroidism, seizures, hypotonia, aphasia, osteoporosis, sodium, mri, blood pressure, serum osmolality, urine osmolality, ft3, ft4, cortisol (9am), cortisol (serum), bone mineral density, x-ray, chloride, haemoglobin , 25-hydroxyvitamin-d3, sex hormone binding globulin, fluid repletion, saline, hydrocortisone, glucocorticoids, glucose, desmopressin, levothyroxine, valproic acid, neurology, unique/unexpected symptoms or presentations of a disease, august, 2020

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