G. Samandouras

Amyloid in neurosurgical and neurological practice

The amyloidoses are a diverse group of diseases characterized by the deposition of specific proteins with distinct affinity to the dye Congo red, collectively called amyloid. The amyloidogenic proteins have acquired an abnormal, highly ordered, beta-pleated sheet configuration with a propensity to self-aggregate. The amyloid may be distributed in different organs with a remarkable diversity. Two broad categories of amyloidoses are recognised: The systemic (consisting of the primary or light chain form, the secondary or reactive form and the familial or hereditary form) and the localised that target specific organs. A tropism of amyloid proteins to the neural tissue produces certain patterns of central nervous system diseases: cerebral amyloid angiopathy, a substrate of spontaneous intracerebral haemorrhage; mature neuritic plaques found in Alzheimer disease and a subset of prion diseases; a topographically restricted accumulation of extracellular proteins giving rise to tumour-mimicking masses, the amyloidomas; and finally, spinal extradural amyloid collections that occasionally are found in the context of rheumatoid arthritis. In this review article we present original illustrative cases of amyloid diseases of the central nervous system that may be encountered in neurosurgical and neurological practice. Molecular aspects and clinical management problems are discussed.

Intraoperative, imaging and pathological features in spinal cord vasculitis

The surgical intraoperative findings in spinal cord vasculitis have not been previously described. The imaging appearances can mimic intramedullary spinal cord tumours. We present a patient with spinal cord vasculitis and mixed intraoperative findings of spinal vascular lesion and intramedullary tumour. Based on the absence of a confirmatory intraoperative smear, vessel obliteration and tumour debulking was avoided, preventing a catastrophic surgical complication. The limited literature and management options are also reviewed.

Serial LPs Without Serial Imaging in Idiopathic Intracranial Hypertension: A Lesson Learned

With obscure etiology, confusing terminology, and controversial management, idiopathic intracranial hypertension (IIH), most commonly known as benign intracranial hypertension, remains a common and challenging clinical problem of the general and neurological practice. The authors present a case of a patient who was diagnosed with IIH and treated with serial lumbar punctures for 4 years prior to her final diagnosis of a large intracranial tumor. This case emphasizes that IIH, an enigmatic disease that affects young females and can potentially lead to blindness, should be diagnosed after an exhausting exclusion of secondary causes of intracranial hypertension.

Spinal cord infarction caused by malignant intramedullary glioma: the traps of epidemiology and travel history.

A 19-year-old patient of Pakistani origin presented with paraplegia and a sensory level at T4. The patient was well until 3 weeks prior to his admission when he developed back and left leg pain. One week later he developed paraparesis that over the next 10 days progressed to paraplegia and loss of sphincter function. On examination he was drowsy. He had power 0 MRC in his lower extremities and loss of all sensory modalities below T4. His symptoms started whilst he was on holiday in Pakistan where he spent 6 weeks. Routine laboratory investigations were unremarkable and CSF studies obtained after a lumbar puncture were negative for infectious disease including TB, viruses, brucella, amoebic and Lyme disease. Imaging demonstrated normal chest radiographs. MRI of his brain was normal, while MRI of his spine revealed an intramedullary spinal lesion extending from T7 –T10 (Fig. 1). At the centre of the lesion a necrotic core was evident, while the spinal cord rostrally demonstrated extensive associated oedema (Fig. 1).