CNS Superficial Siderosis Mimicking a Motor Neuron Disease.

Superficial siderosis of the central nervous system (SS-CNS) is a rare condition characterized by a hemosiderin accumulation along the subpial surfaces and arises from an intermittent chronic bleeding in the subarachnoid space usually as a result of a chronic subarachnoid hemorrhage by trauma, vascular malformations, CNS tumors, or cerebral amyloid angiopathy (CAA). We present a 61-year-old male with a 12-year history of limb weakness, muscle wasting, cramps, clumsiness, progressive unsteady gait, and fine motor impairments. His medical history included the resection of a left parietal meningioma and a myxopapillary ependymoma near the conus terminalis (L3/4) at the age of 51 years. The clinical examination revealed a motor neuron syndrome with a clear bilateral wasting of the hand muscles, a diffuse atrophy of the shoulder and calf muscles, and a weakness of the arms, fingers, hips, and feet. Deep tendon reflexes were symmetrically briskly hyperactive. Standing and walking were only possible with a support. Magnetic resonance imaging of the entire neuroaxis showed progressive severe cerebral, brainstem, and spinal superficial siderosis in form of extensive hypointensities on T2-weighted gradient-echo images and susceptibility-weighted sequences. Despite a successful neurosurgical removal of the tumors and delaed medical treatment with an iron chelator for one year, we observed no clinical recovery or stability in our patient, making this case unique, and suggesting an irreversible neurodegenerative process. This case reinforces the need of including SS-CNS in the list of amyotrophic lateral sclerosis (ALS)-mimics and demonstrates the fundamental use of a complete neuraxial MRI investigation on evaluating possible ALS cases.

[Neuroinflammation as motor of Alzheimer's disease]. / Neuroinflammation als Motor der Alzheimer-Erkrankung.

Sporadic Alzheimer's disease is the most common neurodegenerative disorder and represents a very important public healthcare problem with a devastating economic burden for industrialized countries. Recent knowledge acquired from experimental, epidemiological, radiological and genome-wide association studies (GWAS) underline the role of the innate immune system in the pathophysiology of this disease. This article reviews and discusses the function of the cerebral innate immune system, the newly discovered genes associated with the disease development and the experimental evidence around the role of microglia in the onset and progression of Alzheimer's disease. The discovery of different microglia phenotypes associated with the pathology as well as new molecular players will enable the development of new preventive and therapeutic strategies by modulating neuroinflammation in neurodegenerative diseases.