A 52-year-old woman presented with a 10-year-history of memory loss and executive dysfunction. Examination disclosed hypopigmented whorls and linear streaks following the lines of Blaschko in different body segments (Figure 1), typical of Hypomelanosis of Ito (HI). Brain MRI showed marked enlarged Virchow-Robin spaces (Figure 2). HI or incontinentia pigmenti achromians (MIM#300337) represents a...

Six patients with an aortoiliac vascular disease and a peripheral neurological deficit are presented. Clinical and electromyographic findings revealed lumbosacral plexus, sciatic and femoral nerve lesions. A correlation is made between the level of the vascular lesion (aortic, aortoiliac or distally) and the type of peripheral nerve deficit observed. In a patient complaining of pain, weakness, ...

Wegener's granulomatosis (WG) is a systemic disease of unknown origin characterized by necrotizing granulomatous vasculitis that involves primarily the upper and the lower respiratory tracts and the kidneys. A 44-year-old woman presenting with unusual neurological manifestation is reported. The patient with multiple pulmonary round shadows and hilar lymphadenopathy on the chest X-ray pictures, ...

Systemic lupus erythematous (SLE) is frequently encountered in clinical practice; a widespread immunological response can involve any organ system, sometimes leading to rare and diagnostically challenging presentations. We describe a 38-year-old female who presented with symmetric numbness and tingling of the hands and feet, and cervical pain. Imaging studies were not diagnostic of any serious ...

Niemann-Pick disease (NP-C) is a lysosomal storage disease in which impaired intracellular lipid transport leads to accumulation of cholesterol and glycosphingolipids in various neurovisceral tissues. It is an autosomal recessive disorder, caused by mutations in the NPC1 or NPC2 genes. The clinical spectrum is grouped by the age of onset and onset of neurological manifestation: pre/perinatal; e...

Abstract Background Erdheim–Chester disease (ECD) is a rarely encountered idiopathic systemic form of non-Langerhans cell histiocytosis. The clinical manifestations ECD are highly heterogeneous, ranging from unifocal forms to life-threatening multisystem involvement. Patients with CNS involvement often do not show remission. Case presentation We present case 60-year-old male patient worsening c...

Anti-N-methyl-D-aspartate receptors encephalitis (ANMDARE) is a well-defined, life threatening, but treatable disorder that often occurs as a paraneoplastic manifestation of ovarian teratomas in adult women. We report a child with this disorder who displayed a subacute onset of delirium, seizures, and autonomic instability. Antibodies against NMDA receptor were detectable in the serum and in th...

Multiple hydatid cysts of the brain are uncommon and may be either primary or secondary. A 15-year-old boy with a huge mass of intracranial hydatid cysts (95x85x80 mm) is presented. The first manifestation was headache and vomiting, which was followed by symptoms of raised intracranial pressure. The patient underwent an urgent operation due to rapidly deteriorating neurological status, and 19 h...

HTLV-I-associated myelopathy (HAM/TSP) is the most common neurological manifestation of HTLV-I, causing progressive weakness, sensory disturbance, and sphincter dysfunction. Although motor disorders have been well described, few studies have associated cognitive disorders and HTLV-I infection. In areas endemic for HTLV-I infection, the differential diagnosis between HAM/TSP and other myelopathy...