(Schaltenbrand syndrome)

Rare and often undiagnosed disease that occurring most often in young female adults. Symptoms of orthostatic headache often accompanied by other neurological signs (tinnitus, visual disturbances, external oculomor paralysis, disorder of consciousness) and that are relieved in the supine position.

It results from intracranial hypovolemia caused by leakage of CSF without any known cause of dural breach  (on the contrary for example of headache following a lumbar puncture. Those children and adolescents often suffer from a defect of collagen (Marfan, Ehlers-Danlos or related syndromes).

This syndrome is often triggered by a benign event: cough, defecation, minor trauma. The CSF leak generally occurs at the level of focal abnormalities of the meninges (Tarlov cysts, for example).  Brain and spinal cord MRI are the best way to confirm the diagnosis and sometimes to identify the localizaton of the CSF leak (usually at the thoracic level).

MRI signs: (SEEPS acronym for Subdural fluid collections, Enhancement of the pachymeninges, Engorgement of venous structures, Pituitary hyperemia, Sagging of the brain)

-         aspect of Chiari malformation type I: herniation of the brainstem and cerebellum

-         venous hyperemia with diffuse enhancement of the meninges.

-        small volume cerebral ventricles (lit-like)

-          subdural hematoma in case of consciousness disorder (20%)

Treatment: blood patch that sometimes must be repeated; epidural injection of biological glue at the level of leakage; rarely surgical repair of the leaking meningeal diverticle; drainage of subdural hematoma.

Warning: cases of transient headache (a few weeks) caused by intracranial hypertension have been described following a blood patch done to correct intracranial hypotension.

Anesthetic implications: 

rare indication of blood patch the success rate of which is lower than in conventional indications (post-dural puncture headache). Even if a blood patch at the lumbar spinal level is often sufficient, it is better to perform it at the level of the meningeal leak if it has been identified. In case of multiple leaks, several successive or simultaneous blood patches can sometimes be necessary.

References : 

• Mokri B. 
  Low cerebrospinal fluid pressure syndromes. 
  Neurol Clin N Am 2004; 22: 55-74.
• Mokri B. 
  Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks. 
  Mayo Clin Proc 2002; 77: 124-6.
• Buguet-Brown M-L, Le Gulluche Y, Vichard A, De Greslan T. 
  Spontaneous intracranial hypotension : a recent indication for epidural blood patch. 
  Br J Anaesth 2006; 96: 668-9
• Bordes J, Faivre A, Meaudre E, Asencio Y, Benefice S. 
  Hypotension intracrânienne spontanée traitée par blood patch lombaire. 
  Ann Fr Anesth Réanim 2007 ; 26 : 613-4.
• Belena JM, Nunez M, Yuste J, Plaza-Nieto JF, Jimenez-Jimenez FJ, Serrano S. 
  Spontaneous intracranial hypotension syndrome treated with a double epidural blood patch. 
  Acta Anaesthesiol Scand 2012; 56: 1332-5.
• Schievink WI, Maya MM, Louy C, Moser FG, Sloninsky L. 
  Spontaneous intracranial hypotension in childhood and adolescence. 
  J Pediatr 2013; 162: 504-10.
• Collange O, Wolff V, Cebula H, Pradignac A, Meyer A et al.
  Spontaneous intracranial hypotension: an etiology for consciousness disorder and coma. 
  A&A Case Reports 2016; 7: 207-11.
• Qasem F, McCallum C, Armstrong P.
  Epidural blood patch treatment for headache caused by a ruptured Tarlov cyst.
  Can J Anesth 2017; 64: 983-4.
• Gowreesunker P, Blanjean A.
  Spontaneous intracranial hypotension and a comparison of available treatment options.
  Acta Anaesth Belg 2019; 70: 213-6

Updated: August 2022