Abstract Details

Superficial Siderosis is a rare neurodegenerative disorder that results from hemosiderin deposition in the brain and spinal cord. Extensive investigations to localise a bleeding source is often unyielding. We report a case that highlights the importance of early diagnosis for clinical and therapeutic implications.  

A 69 year old male presented with a 6-month history of gait deterioration resulting in multiple falls, leaving him wheelchair bound. There was no previous history of trauma or surgery. On examination, he was myelopathic with generalised hypertonia, hyperreflexia and upgoing plantars. A dysarthric speech was noted, with generalised cerebellar ataxia and a broad based spastic gait.

MRI of the brain and spine revealed a rim of T2 hypointensity coating the surfaces of the leptomeninges, ventricles and spinal cord, classical for Superficial Siderosis. The most prominent hemosiderin deposition was seen in the lower brainstem, cerebellum and spinal cord. An epidural fluid filled sac was noted from in the mid-thoracic region. In light of this, a CT myelogram was pursued and localised a dural tear from C6 to T7. MRA and a six-vessel cerebral and spinal angiogram did not reveal any source of intracranial haemorrhage, aneurysm or arterio-venous malformation. He subsequently underwent a right T2/3 interlaminar decompression with fenestration and sealing of the epidural cyst and cavity. 

At 6 months post-operative follow-up, the patient had marked improvement in gait and was able to walk independently with a walking stick and no falls. Interval MRI of the spine confirmed successful fixation with stable siderosis changes compared to previously and no radiological features of disease progression.

Comprehensive imaging of the neuroaxis leads to early diagnosis of Superficial Siderosis, and aids in localisation of the bleeding source. This facilitates prompt surgical intervention, directly translating to improvement in motor function and potentially halting further neurodegenerative progression.