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Cerebral Salt-Wasting Syndrome

Cerebral Salt Wasting Syndrome

Cerebral salt-wasting syndrome constitutes a medical condition characterized by hypovolemia and hyponatremia, secondary to various pathologies affecting the central nervous system (CNS).


Presentation

Cerebral salt-wasting syndrome (CSWS) is a medical entity that leads to hyponatremia and hypovolemia due to dehydration, as a result of an acute or chronic and persistent underlying CNS disorder, including trauma, tumors, and other pathologies.

Patients present with the clinical manifestations of hyponatremia and hypovolemia. In general, a case of very mild hyponatremia tends to produce no noticeable symptoms; however, it has been observed that even non-severe hyponatremia can lead to devastating clinical manifestations [1]. A serum sodium concentration lower than 135 mEq/L, but over 115 mEq/L usually leads to nausea and malaise. Values less than 135 mEq/L lead to profound neurological symptomatology due to cerebral edema, including a diminished level of consciousness and lethargy, cephalalgia, epileptic phenomena and, eventually, even a comatose state [2]. There is a positive correlation between the decrease in sodium levels and the aggravation of symptoms: as the concentration of sodium in the serum of the patient declines, symptoms are exacerbated.

Hypovolemia leads to symptoms that entail dried mucous membranes (oral or nasal cavity), dry skin that lacks elasticity, sunken anterior fontanelle, and tachycardia. Profound hypovolemia causes hypotension. A vital clinical characteristic of patients affected by cerebral salt-wasting syndrome is that the aforementioned manifestations tend to respond positively to volume and sodium replacement [3] [4]. Massive polyuria that can be reversed with the simultaneous administration of vasopressin and cortisone acetate, hypotension, the absence of cardiac symptoms and hypovolemia, constitute a potential clinical picture of a patient with central diabetes insipidus and CSWS, according to a recent study [5].

Weight Loss
  • She had a history of significant weight loss for the last six months. On examination she was drowsy and on admission Glasgow coma scale was 7/15.[jpma.org.pk]
  • Intravascular volume depletion Historical features suggesting hypovolemia include thirst, abrupt weight loss, decreasing urinary frequency, and negative fluid balance. 11.[slideshare.net]
  • Significant Weight Loss Weight loss is often used as a surrogate for malnutrition.[northshore.org]
  • In CSW, expect signs and symptoms of dehydration, including dry mucous membranes, poor skin turgor, tachycardia, orthostatic hypotension, weight loss, and a negative fluid balance (with output exceeding intake).[americannursetoday.com]
  • Because of reduced extracellular fluid may have dehydration performance such as low central venous, orthostatic hypotension, tachycardia, dry mucous membrane, jugular vein collapse, body weight loss, et al . [ 7 ].[omicsonline.org]
Orthostatic Hypotension
  • Advanced symptoms include muscle cramps , lightheadedness, dizziness or vertigo , feelings of anxiety or panic (not mentally induced), increased heart rate or slowed heart rate , low blood pressure and orthostatic hypotension sometimes resulting in fainting[en.wikipedia.org]
  • But now when you assess him, you find that he can't follow simple commands, has orthostatic hypotension, and is oliguric with dry mucous membranes.[journals.lww.com]
  • In CSW, expect signs and symptoms of dehydration, including dry mucous membranes, poor skin turgor, tachycardia, orthostatic hypotension, weight loss, and a negative fluid balance (with output exceeding intake).[americannursetoday.com]
  • Because of reduced extracellular fluid may have dehydration performance such as low central venous, orthostatic hypotension, tachycardia, dry mucous membrane, jugular vein collapse, body weight loss, et al . [ 7 ].[omicsonline.org]
  • Adverse effects reported with short-term use are typically minimal, but may include serious effects such as hypokalaemia, orthostatic hypotension, and unexpectedly rapid serum sodium correction.[jnaccjournal.org]
Confusion
  • This syndrome is often confused with dilutional hyponatremia secondary to inappropriate ADH secretion. Accurate diagnosis and management are mandatory for to improve the course of the disease.[ncbi.nlm.nih.gov]
  • In this article, it was aimed to report a patient with CSWS which progresses with low percentage in intensive care unit patients with head trauma, is frequently confused with inappropriate antidiuretic hormone syndrome and progresses mortally when not[ingentaconnect.com]
  • To add to the confusion, in both SIADH and in CSW the vasopressin levels are elevated. The trick is demonstrating that the volume is reduced.[derangedphysiology.com]
  • On july 9th he became confused. Cerebral CT was performed with no significant changes. His natremia dropped to 128 mEq/l, with development of poliuria despite maintenance of desmopressin doses.[endocrine-abstracts.org]
Seizure
  • She presented 16 days postoperatively, and following two grand mal seizures was found to be profoundly hyponatraemic (sodium 101 nmol/l).[ncbi.nlm.nih.gov]
  • A 2-year-old male patient presented to our hospital due to a seizure attack. He had been neglected and refused to take food for a long time (body weight 3rd percentile).[ncbi.nlm.nih.gov]
  • In addition, nearly half of the patients with CSWS (47%) had post-operative hyponatremic seizures.[ncbi.nlm.nih.gov]
  • […] symptoms frequently associated with dysautonomia include: headaches , pallor , malaise , facial flushing, constipation or diarrhea , nausea , acid reflux , visual disturbances, numbness, nerve pain, trouble breathing, chest pains, loss of consciousness and seizures[en.wikipedia.org]
  • Infants, however, lack the ability to dilute their urine to this extent and are at increased risk of water intoxication and hyponatremic seizures after administration of free water.[clinicaladvisor.com]
Lethargy
  • Values less than 135 mEq/L lead to profound neurological symptomatology due to cerebral edema, including a diminished level of consciousness and lethargy, cephalalgia, epileptic phenomena and, eventually, even a comatose state.[symptoma.com]
  • Symptoms include lethargy, agitation, headache, altered consciousness, seizures,and coma . The severity of symptoms typically reflects the magnitude and rapidity of the decrease in serum sodium concentration.[slideshare.net]
  • Emergency Treatment of Hyponatremia The acute onset ( serum sodium values can be associated with central nervous system dysfunction resulting in lethargy, loss of consciousness, psychosis, and seizures (especially in younger children).[clinicaladvisor.com]
  • Hyponatremia signs and symptoms—confusion, lethargy, decreased level of consciousness, seizures, and coma—are common, too.[americannursetoday.com]
  • Symptoms include lethargy, agitation, headache, altered consciousness, seizures and coma. [17] , [18] Serum osmolality Normal serum osmolality is 285-295 mosmosm/L. This is found to be decreased in SIADH but is either normal or decreased in CSWS.[jnaccjournal.org]
Neglect
  • He had been neglected and refused to take food for a long time (body weight 3rd percentile).[ncbi.nlm.nih.gov]
  • A 79-year-old Japanese woman with hypertension and arrhythmia was admitted to our hospital for mild consciousness disturbance, conjugate deviation to right, left unilateral spatial neglect and left hemiparesis.[ncbi.nlm.nih.gov]

Workup

CSWS is a syndrome difficult to diagnose, due to the lack of a specific set of tests to detect any pathognomonic result. The primary difficulty is its differentiation from the syndrome of inappropriate antidiuretic hormone secretion (SIADH), due to a significant similarity between these two distinct entities. The workup involved in the diagnostic procedure encompasses:

Serum sodium concentration

Reveals hyponatremia, namely a serum sodium concentration < 135 mEq/L.

Urinary output

CSWS is expected to lead to increased urine flow and dilute urine, whereas SIADH leads to a low urine flow and concentrated urine.

Urinary sodium concentration

CSWS leads to a high urinary sodium concentration, that exceeds 40 mEq/L, followed by a negative net sodium balance.

Uric acid

Fractional excretion of uric acid (FEUA) is increased in patients with the cerebral salt-wasting syndrome and hyperuricemia is detected. These findings tend to remain, despite the reversion or hyponatremia. On the contrary, the same findings that are observed in SIADH tend to regress after the correction of serum sodium [6] [7].

Phosphate

Cerebral salt-wasting syndrome leads to an increase in the fractional excretion of phosphate (FEP), in contradistinction to SIADH [7].

Decreased Central Venous Pressure
  • A review of renal/CSWS reveals three studies involving hyponatremic neurosurgical patients who had decreased blood volume, decreased central venous pressure, and inappropriately high urinary sodium concentrations in the majority of them, suggesting that[ncbi.nlm.nih.gov]
Hyponatremia
  • The appropriate treatment of CSW syndrome is opposite the usual treatment of hyponatremia caused by SIADH.[ncbi.nlm.nih.gov]
  • Hyponatremia should be prevented if possible and treated promptly when discovered in patients with CNS disease as even mild hyponatremia could lead to neurological deterioration.[ncbi.nlm.nih.gov]
  • These two different sources of hyponatremia require different treatments.[ncbi.nlm.nih.gov]
  • Hyponatremia associated with neuroleptic malignant syndrome has thus far been described as a syndrome of inappropriate secretion of antidiuretic hormone.[ncbi.nlm.nih.gov]
  • Patients present with the clinical manifestations of hyponatremia and hypovolemia.[symptoma.com]
Streptococcus Pneumoniae
  • Streptococcus pneumoniae was isolated in the culture of the cerebrospinal fluid and, after identifying the antibiogram (intermediate sensitivity to cephotaxime), the dosage of cephotaxime was increased and rifampicin was added.[elsevier.es]
  • The results were shown sputum culture and sensitivity of Streptococcus pneumoniae .[omicsonline.org]

Treatment

  • The specific treatment for CSWS was to increase volume delivery according to the characteristics of the patient. Except for one case, none of the remaining patients required more than 72 hours of treatment to correct hyponatremia.[ncbi.nlm.nih.gov]
  • The appropriate treatment of CSW syndrome is opposite the usual treatment of hyponatremia caused by SIADH.[ncbi.nlm.nih.gov]
  • Treatment of cerebral salt wasting consists of hydration and salt replacement. This article uses a case report to discuss the importance of recognition of this syndrome, and treatment concerns are reviewed.[ncbi.nlm.nih.gov]
  • […] for CSWS and SIADH can be drastically different, which makes the distinction critical for post-operative treatment.[ncbi.nlm.nih.gov]
  • We also revise the aetiology, mechanisms, differential diagnosis and treatment of hyponatraemia after pituitary surgery.[ncbi.nlm.nih.gov]

Prognosis

  • This case emphasizes the poor prognosis that may result from the rapid correction of profound hyponatraemia.[ncbi.nlm.nih.gov]
  • Of 11 patients, 5 had a good prognosis 3 months later (Extended Glasgow Outcome Scale score 6), 1 had an Extended Glasgow Outcome Scale score of 4, 2 died in the hospital of brain hernia, and 3 developed a vegetative state.[ncbi.nlm.nih.gov]
  • Objectives INTRODUCTION Definition PATHOPHYSIOLOGY Etiology EPIDEMIOLOGY History Physical Examination Workup DIAGNOSIS DIFFERENTIAL DIAGNOSIS TREATMENT Prognosis SUMMARY 3.[slideshare.net]
  • As long as accurate and timely diagnosis, appropriate treatment, the prognosis is good. About CSWS pathogenesis remains to be further studied.[omicsonline.org]
  • The report included 81 individuals with tuberculous meningitis, 39.5% of whom suffered ischemic stroke; 50% of these stroke patients had cerebral salt wasting. [12] Prognosis Cerebral salt-wasting syndrome usually develops in the first week following[emedicine.medscape.com]

Etiology

  • The etiology of this hyponatremia is often diagnosed as syndrome of inappropriate diuretic hormone (SIADH). Fluid restriction is usually the first line of treatment. However, this can exacerbate vasospasm and produce resultant ischemia.[ncbi.nlm.nih.gov]
  • Although the exact etiology of CSWS is still not completely elucidated, it is believed that the hypothalamus plays a pivotal role in the genesis of this disorder.[ncbi.nlm.nih.gov]
  • The most common etiologies were severe central nervous system infection (n 7, 44%) and hypoxic-ischemic event (n 4, 25%). In 15 patients, diabetes insipidus was diagnosed during the first 3 days after acute central nervous system injury.[ncbi.nlm.nih.gov]
  • An etiologic role has been given to ANH and to other, as yet undiscovered, central nervous system natriuretic factors.[ncbi.nlm.nih.gov]
  • After exclusion of other etiologies, a diagnosis of CSW was established and the patient started appropriate treatment with normalization of sodium levels.[journalmc.org]

Epidemiology

  • Objectives INTRODUCTION Definition PATHOPHYSIOLOGY Etiology EPIDEMIOLOGY History Physical Examination Workup DIAGNOSIS DIFFERENTIAL DIAGNOSIS TREATMENT Prognosis SUMMARY 3.[slideshare.net]
  • Kojima et al have described an animal model of cerebral salt-wasting syndrome that may allow better clarification of the condition’s etiology. [8] Epidemiology Occurrence in the United States Exact incidence data for this disorder are not available.[emedicine.medscape.com]
  • .: Role of nutritional status in predicting quality of life outcomes in cancer--a systematic review of the epidemiological literature. Nutr J 11: 27, 2012.[northshore.org]
Sex distribution
Age distribution

Pathophysiology

  • All 3 syndromes affect both sodium and water balance; however, they have differences in pathophysiology, diagnosis, and treatment.[ncbi.nlm.nih.gov]
  • Differences in the pathophysiology of this disorder mandate a unique therapeutic approach which may be essential to avoiding serious electrolyte disturbances.[nature.com]
  • "Cerebral salt wasting: pathophysiology, diagnosis, and treatment". Neurosurg Clin N Am . 21 (2): 339–52. doi : 10.1016/j.nec.2009.10.011 . PMID 20380974 . Peters JP, Welt LG, Sims EA, Orloff J, Needham J (1950).[en.wikipedia.org]
  • PATHOPHYSIOLOGY The SNS promotes Na , uric acid &water reabsorption in the proximal tubule, as well as renin release.[slideshare.net]
  • However, an exact value and pathophysiologic mechanisms of decreased serum uric acid level in pediatric patients remain elusive.[frontiersin.org]

Prevention

  • […] the prevention of hyponatremia in hospitalized patients with CNS disease and a more hypertonic fluid may be required.[ncbi.nlm.nih.gov]
  • Differentiating between hypernatremia (central neurogenic diabetes insipidus) and the 2 hyponatremia syndromes (syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome) is critical for preventing worsening neurological[ncbi.nlm.nih.gov]
  • It should be prevented by fluid restriction. In the latter, we found adequately low ADH levels, when the hypo-osmolarity is taken into account.[ncbi.nlm.nih.gov]
  • Close monitoring of salt and fluid balance is essential to prevent severe neurologic and hemodynamic complications.[ncbi.nlm.nih.gov]
  • The hyponatremia or natricuresis of the children improved and neurologic and circulatory sequelae could be prevented.[ncbi.nlm.nih.gov]

References

Article

  1. Grant P, Ayuk J, Bouloux PM, et al. The diagnosis and management of inpatient hyponatraemia and SIADH. Eur J Clin Invest. 2015 Aug; 45(8): 888–894.
  2. Sherlock M, O'Sullivan E, Agha A, et al. Incidence and pathophysiology of severe hyponatraemia in neurosurgical patients. Postgrad Med J. 2009 Apr. 85(1002):171-5.
  3. Cerdà-Esteve M, Cuadrado-Godia E, Chillaron JJ, et al. Cerebral salt wasting syndrome: review. Eur J Intern Med 2008; 19: 249–54
  4. El-Amin Abdel-Latif M, Chan PWK, Yu-Teik Goh A, Chai-See Lum L. Cerebral salt wasting syndrome following atlantoaxial fracture dislocation in Down syndrome. BMJ Case Rep. 2009.
  5. Wu X, Zhou X, Gao L, et al. Diagnosis and Management of Combined Central Diabetes Insipidus and Cerebral Salt Wasting Syndrome After Traumatic Brain Injury. World Neurosurg. 2016 Apr. 88:483-7.
  6. Moritz ML. Syndrome of inappropriate antidiuresis and cerebral salt wasting syndrome: are they different and does it matter?. Pediatr Nephrol. 2012 May. 27(5):689-93. [Medline].
  7. Maesaka JK, Miyawaki N, Palaia T, Fishbane S, Durham JH. Renal salt wasting without cerebral disease: diagnostic value of urate determinations in hyponatremia. Kidney Int. 2007 Apr. 71(8):822-6.

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Last updated: 2018-06-21 18:57