Disorders of Upper and Lower Motor Neurons
Fig. 21.1 ALS. In (a) and (b) T2-weighted images demonstrate slight hyperintensity along cortico-spinal tracts (arrows). In (c), note a slight hypointensity in both Rolando’s cortex (arrowheads) No specific biomarker…
Neurofibromatosis Type 1 and Type 2
Fig. 26.1 NF1. Coronal and axial T2-weighted images (a–d) show multiple, asymmetric plexiform neurofibromas of the bilateral brachial plexuses and of bilateral lumbar plexuses. Note the confluent aspect of the…
Disorders of Cerebrospinal Fluid Circulation
Fig. 23.1 SIH. Axial T2-weighted image and coronal T1-weighted image after contrast medium administration (a) and (b) demonstrated bilateral subdural fluid collections (arrows) and diffuse dural thickening and enhancement along…
Mitochondrial Disorders
Disease/syndrome Clinical features Genetics Prognosis Defects of mtDNA Chronic progressive external ophthalmoplegia syndrome (CPEO) Mimicking KSS plus AD; AR Progressive disorder of variable severity, and phenotype. Onset: late teens-early 20s…
Neurological Pain
1 Continuing pain, which is disproportionate to any inciting event 2 At least one symptom in three (clinical diagnostic criteria) or four (research diagnostic criteria) of the following categories: (a)…
Inflammatory Myopathies
© Springer-Verlag Italia 2015Angelo Sghirlanzoni, Giuseppe Lauria and Luisa Chiapparini (eds.)Prognosis of Neurological Diseases10.1007/978-88-470-5755-5_39 39. Inflammatory Myopathies Lucia Morandi1 (1) UO Muscle Pathology–Neuroimmunology, Neurological Institute “Carlo Besta”, Milan, Italy Lucia Morandi Email: lucia.morandi@istituto-besta.it KeywordsDermatomyositisIdiopathic inflammatory myopathies…
Muscular Dystrophies
Disease Gene Typical onset Progression Heart involvement Respiratory involvement LGMD1A TTID Adulthood Slow Rare Rare LGMD1B LMNA Variable Slow Frequent Possible LGMD1C CAV3 Childhood Slow Possible No LGMD1D DNAJB6 Adulthood…
Autoimmune Ion Channel Disorders of the Peripheral Nervous System
MG LEMS: autonomic dysfunction, absent deep tendon reflexes, rare ocular involvement Congenital myasthenic syndromes: onset in childhood, absence of autoantibodies, no effect of immunotherapy Motor neuron disorders: fasciculations, muscle atrophy,…
