1.
[Non-cirrhotic Portal-systemic Encephalopathy with Total Aphasia and Right Hemiplegia Following Transient Right Hemiparesis: A Case Report].
Goto, H, Tanaka, T, Suetsugi, N, Kubotsu, Y, Momozaki, N, Mizuta, T
Brain and nerve = Shinkei kenkyu no shinpo. 2018;(2):155-159
Abstract
A 77-year-old right-handed woman without any liver diseases was admitted to our hospital because of transient right hemiparesis. She developed total aphasia with right hemiplegia on the third hospital day. We suspected that she had a cerebral infarction following a transient ischemic attack. However, brain diffusion-weighted images revealed no abnormal-intensity lesions, and cerebral angiography showed patent arteries. Additionally, her serum ammonia level was elevated. Theta waves without triphasic waves were detected by electroencephalogram. T1-weighted magnetic resonance brain images revealed high-intensity signals in the bilateral globus pallidus. Enhanced abdominal computed tomography showed a portal-systemic shunt from the splenic and inferior mesenteric veins into the left renal vein via the left ovarian vein. The administration of branched-chain amino acids and lactulose improved her clinical symptoms. We confirmed the diagnosis of non-cirrhotic portal-systemic encephalopathy (NCPSE), therefore balloon-occluded retrograde transvenous obliteration of the shunt vessel was performed. The recognition of NCPSE on the examination of a suspected stroke patient is important, as patients with NCPSE can present as stroke mimics. (Received June 26, 2017; Accepted August 22, 2017; Published February 1, 2018).
2.
ATP1A3-related disorders: An update.
Carecchio, M, Zorzi, G, Ragona, F, Zibordi, F, Nardocci, N
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society. 2018;(2):257-263
Abstract
Alternating Hemiplegia of Childhood (AHC), Rapid-onset Dystonia Parkinsonism (RDP) and CAPOS syndrome (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) are three distinct, yet partially overlapping clinical syndromes that have long been thought to be allelic disorders. From 2004 to 2012, both autosomal dominant and de novo mutations in ATP1A3 have been detected in patients affected by these three conditions. Growing evidence suggests that AHC, RDP and CAPOS syndrome are part of a large and continuously expanding clinical spectrum and share some recurrent clinical features, such as abrupt-onset, asymmetric anatomical distribution and the presence of triggering factors, which are highly suggestive of ATP1A3 mutations. In this review, we will highlight the main clinical and genetic features of ATP1A3-related disorders focussing on shared and distinct features that can be helpful in clinical practice to individuate mutation carriers.
3.
Abnormal bone and calcium metabolism in patients after stroke.
Sato, Y
Archives of physical medicine and rehabilitation. 2000;(1):117-21
Abstract
OBJECTIVE To review how mineral stores and endocrine factors affect bone mass in poststroke patients immobilized by hemiparesis. DATA SOURCES Computer databases and published indexes. STUDY SELECTION Case-control studies of hemiparetic poststroke patients examined regarding bone metabolism. DATA EXTRACTION References were obtained from MEDLINE; all data concerning the objective were used. DATA SYNTHESIS Bone loss occurs in affected extremities after stroke. Immobilization from hemiplegia causes hypercalcemia. Insufficiency or deficiency of 25-hydroxyvitamin D (25-OHD) is very common in stroke patients and may be caused by poor dietary intake, decreased sunlight exposure, or both. Compensatory hyperparathyroidism may not occur because hypercalcemia inhibits the parathyroid glands even when 25-OHD is in the insufficient range. However, hyperparathyroidism does occur when 25-OHD is in the deficient range, in which case the parathyroid response to hypovitaminosis D overrides effects of hypercalcemia. Increased bone resorption was observed during the first year after stroke, declining to normal during the second year. During the first year, determinants of bone mineral density (BMD) in hands affected by hemiplegia were age, severity of hemiplegia, duration of paralysis, serum calcium concentration, and 25-OHD concentration. In the second year, BMD determinants on the hemiplegic side were severity of hemiplegia and 25-OHD concentration, whereas 25-OHD concentration was the only BMD determinant on the intact side. Administering 1alpha-hydroxyvitamin D3, vitamin K2, or ipriflavone ameliorated osteopenia on both sides and decreased the frequency of hip fracture on the hemiplegic side. CONCLUSIONS Bone remodeling and determinants of bone mass for the affected and unaffected sides after stroke differ between the first and subsequent years.