Syringocephalus

Syringocephalus , also known as syringoencephalomyelia, is a very rare entity and refers to a syrinx that extends into the cerebrum .

Clinical presentation

Patients with this condition demonstrate a wide variety of focal neurological symptoms depending on where the syrinx is located .

Pathology

When present, syringocephalus is seen in continuity with a long syrinx that demonstrates syringomyelia, syringobulbia, syringopontia, and syringomesencephaly . Most commonly the syrinx extends into the centrum semiovale, however has less commonly been reported to extend into the basal ganglia, internal capsule, and cerebral cortex instead .

There are numerous causes and associations, and these are discussed in more depth in the general article on syrinx.

Radiographic features

Syringocephalus has the same radiographic characteristics on all imaging modalities as any other syrinx . See syrinx for an in-depth discussion of these characteristics.

Treatment and prognosis

When symptomatic, neurosurgical intervention may be required .