[Familial spontaneous intracranial hypotension].
BACKGROUND
Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache and diffuse dural gadolinium enhancement on magnetic resonance imaging. Spontaneous intracranial hypotension is caused by spinal cerebrospinal fluid leaks and evidence for an underlying generalized connective tissue disorder is found in about two thirds of patients. There had been no familial cases reported to date.


METHODS
We describe the case report of three healthy female patients with spontaneous intracranial hypotension who belong to the same family. They have no cutaneous or articular stigmas of connective tissue disease.


CONCLUSIONS
These familial cases of spontaneous SIH suggest that certain underlying genetic susceptibility, probably linked to lower resistance of the spinal meninges, could favor the development of spontaneous intracranial hypotension.
Larrosa
D
D
Servico de Neurologia, y Centro de Investigación Biomédica en Red sobre enfermedades Neurodegeneratives, Hospital Universitaro Marqués de Valdecilla, Santander.
Vázquez
J L
JL
Mateo
I
I
Infante
J
J
D005260:Female; D006801:Humans; D019585:Intracranial Hypotension / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008578:Meninges / Q000378:metabolism; D008875:Middle Aged; D055815:Young Adult