By Roger A. Barker, Neil Scolding, Dominic Rowe, Andrew J. Larner
The moment version of A-Z of Neurological perform builds at the past variation withrevised and up-to-date details in a excessive density yet simply available structure to supply a short and prepared reference for busy clinicians of all levels of expertise. Entries for particular neurological stipulations are uniformly dependent indicating: Pathophysiology; medical positive factors; Investigations and analysis; Differential prognosis; therapy and analysis. Key references are stated all through and all entries are pass referenced.
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Additional info for A-Z of Neurological Practice: A Guide to Clinical Neurology
B-Microglobulin amyloidosis: associated with renal dialysis. Rarely associated with neurological complications. • Hereditary: –– A-Bri, A-Dan: familial British dementia (FBD) and familial Danish dementia (FDD), respectively. 26 A-Z of Neurological Practice –– Amyloid b-peptide: some forms of intracerebral hemorrhage (cerebral amyloid angiopathy), Alzheimer’s disease. –– Apolipoproteins AI, AII: non-neuropathic. –– Cystatin: some forms of intracerebral hemorrhage (cerebral amyloid angiopathy).
Clinical features • Developmental delay; microcephaly, virtually absent speech, feeding difficulties • Mental retardation: mild to severe; +/- autism Angiostrongyliasis 29 • Happy demeanor (constant), unprovoked laughter (may not be prominent) • Movement disorder: ambulation age 5–6 years, distinctive gait with jerky ataxia and lower extremity hypertonia (the “happy puppet”) • Seizure disorder: tonic, atonic > tonic–clonic epileptic attacks • Moderate dysmorphism: prognathism Investigation Neurogenetic testing: for 15q11-q13 deletion of maternal orgin.
Brain. 2009;132:2688–2698 Le Ber I, Moreira MC, Rivaud-Péchoux S, et al. Cerebellar ataxia with oculomotor apraxia type 1: clinical and genetic studies. Brain. 2003;126:2761–2772 48 A-Z of Neurological Practice Atlantoaxial dislocation, subluxation Dislocation or subluxation of the atlantoaxial articulation often results from failure of the stabilizing function of the odontoid peg of C2. This may occur in • • • • • • • • Cervical trauma Congenital malformation Rheumatoid arthritis Down syndrome Ankylosing spondylitis Foramen magnum lesions Grisel syndrome Morquio’s disease The resulting cord compression results in myelopathy or medullary compression, sometimes even in sudden death.
A-Z of Neurological Practice: A Guide to Clinical Neurology by Roger A. Barker, Neil Scolding, Dominic Rowe, Andrew J. Larner