PubRank

T de Barsy

Author PubWeight™ 42.17‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Commentary. Lysosomotropic agents. Biochem Pharmacol 1974 12.28
2 Brain glucose metabolism in postanoxic syndrome. Positron emission tomographic study. Arch Neurol 1990 2.06
3 Fluvoxamine: an antidepressant with low (or no) epileptogenic effect. Lancet 1990 1.39
4 Rodent and human acid -glucosidase. Purification, properties and inhibition by antibodies. Investigation in type II glycogenosis. Eur J Biochem 1972 1.30
5 A gene on chromosome 11q23 coding for a putative glucose- 6-phosphate translocase is mutated in glycogen-storage disease types Ib and Ic. Am J Hum Genet 1998 1.28
6 Pompe's disease: an inborn lysosomal disorder with storage of glycogen. A study of brain and striated muscle. Acta Neuropathol 1973 1.24
7 Immunoblot analyses of glycogen debranching enzyme in different subtypes of glycogen storage disease type III. J Pediatr 1990 1.22
8 Progressive cardiac failure following orthotopic liver transplantation for type IV glycogenosis. Eur J Pediatr 1992 1.09
9 Acid maltase deficiency in non-identical adult twins. A morphological and biochemical study. J Neurol 1976 1.06
10 On an autosomal dominant form of retinal-cerebellar degeneration: an autopsy study of five patients in one family. Acta Neuropathol 1994 1.01
11 Non-epileptic seizures: delayed diagnosis in patients presenting with electroencephalographic (EEG) or clinical signs of epileptic seizures. Seizure 2002 0.91
12 Enzyme replacement in Pompe disease: an attempt with purified human acid alpha-glucosidase. Birth Defects Orig Artic Ser 1973 0.90
13 Polysaccharide (amylopectin-like) storage myopathy histochemical ultrastructural and biochemical studies. Acta Neuropathol Suppl 1981 0.87
14 A morphologic and biochemical study of the fate of antibody-bearing liposomes. Lab Invest 1976 0.87
15 Neuronal ceroid-lipofuscinosis: preferential metabolic alterations in thalamus and posterior association cortex demonstrated by PET. J Neurol Neurosurg Psychiatry 1990 0.86
16 Bilateral tonic pupils and polyneuropathy in Sjögren's syndrome: a common pathophysiological mechanism? Eur Neurol 1991 0.86
17 The autosomal form of phosphorylase kinase deficiency in man: reduced activity of the muscle enzyme. Biochem Biophys Res Commun 1980 0.84
18 [Occurrence and significance of certain fibrillary glioses of the brain stem in very young children]. J Hirnforsch 1969 0.83
19 The spectrum of the so-called rigid spine syndrome: nosological considerations and report of three female cases. J Neurol 1986 0.83
20 Biochemical and ultrastructural study of leucocytes in type II glycogenosis. Arch Int Physiol Biochim 1975 0.82
21 Myoadenylate deaminase deficiency in a patient with facial and limb girdle myopathy. J Neurol 1981 0.81
22 Bradycardia, an epileptic ictal manifestation. Seizure 1995 0.81
23 Myoadenylate deaminase deficiency: absence of correlation with exercise intolerance in 452 muscle biopsies. J Neurol 1987 0.81
24 [Bradycardia: an unrecognized complication of some epileptic crises]. Rev Neurol (Paris) 1998 0.80
25 The retina in Lafora disease: light and electron microscopy. Albrecht Von Graefes Arch Klin Exp Ophthalmol 1980 0.80
26 The cellular distribution of lipososmes in the liver of newborn rats. Biochem Soc Trans 1975 0.78
27 Retarded and aberrant splicings caused by single exon mutation in a phosphoglycerate kinase variant. Arch Biochem Biophys 1996 0.78
28 Type III glycogenosis with multicore structures. Muscle Nerve 1980 0.78
29 [A study of the abnormal polysaccharide in a child with type IV glycogen storage disease (author's transl)]. Arch Fr Pediatr 1981 0.78
30 Nucleolar activation and vacuolation in embryo radicle cells during early germination. J Cell Sci 1985 0.77
31 Brain metabolism in mitochondrial encephalomyopathy: a PET study. J Comput Assist Tomogr 1988 0.77
32 [Extraneural metastases of medulloblastomas: 2 cases]. Neurochirurgia (Stuttg) 1967 0.76
33 Brain glucose metabolism in postanoxic syndrome due to cardiac arrest. Acta Neurol Belg 1994 0.76
34 [Cardiomuscular lysosomal glycogenosis in adults without known enzyme deficiency. A cause of familial myocardiopathy and lysosomal glycogen overload with normal acid maltase]. Arch Mal Coeur Vaiss 1988 0.75
35 Uncommon case of type II glycogenosis. Acta Neuropathol 1979 0.75
36 [Sporadic proximal spinal amyotrophy (Kugelberg-Welander) with a late onset. Its place in the framework degenerations]. Encephale 1967 0.75
37 [Liver fructose-1-phosphate and fructose-1,6-diphosphate aldolase deficiency in hereditary fructose intolerance (author's transl)]. Klin Padiatr 1981 0.75
38 [Amount in cerebrospinal fluid of gammaglutamyl peptidase and leucine aminopeptidase]. Acta Neurol Psychiatr Belg 1966 0.75
39 [Longitudinal study of mental, electroencephalographic and biological evolutiions of an atypical chronic form of subacute sclerosing leukoencephalitis]. Encephale 1967 0.75
40 Normal metabolism and disorders of carbohydrate metabolism. Baillieres Clin Endocrinol Metab 1990 0.75
41 [Polysaccharide amylopectin-type storage myopathy]. Rev Neurol (Paris) 1992 0.75
42 Muscle carnitine deficiency in old age. Case report and therapeutic results. Clin Neurol Neurosurg 1985 0.75
43 Proceedings: Glycogen phosphorylase and its converter enzymes in cultured human fibroblasts. Hoppe Seylers Z Physiol Chem 1974 0.75
44 [Intolerance to exercise caused by carnitine palmitoyltransferase deficiency]. Rev Neurol (Paris) 1990 0.75
45 Acid maltase deficiency in adults. A study of five cases. Acta Neurol Belg 1986 0.75
46 Event-related potentials and cognitive functions in epileptic treated patients. Clin Electroencephalogr 1990 0.75
47 [On diffuse round cell sclerosis Krabbe type)(clinical and genetic notes)]. Arch Fr Pediatr 1967 0.75
48 [Lethal pseudotumoral syndrome following suprarenal deprivation. Picture of fatal cerebral hypertension, in the course of a lipidic nephrosis with abrupt stop of cortisone treatment]. Acta Neuropathol 1968 0.75
49 [Two observations of subacute maningoencephalitis of undetermined origin with extensive symmetrical cutaneous necrosis]. Helv Paediatr Acta 1967 0.75
50 [Certain aspects of axial and cerebellar lesions in infantile amaurotic idiocy GM2 type]. Pathol Biol (Paris) 1970 0.75
51 [Acute encephalomyopathy and persistent cerebellar syndrome after lithium salt and haloperidol poisoning]. Rev Neurol (Paris) 1997 0.75
52 Inheritance of the S113L mutation within an inbred family with carnitine palmitoyltransferase enzyme deficiency. Hum Genet 1996 0.75
53 [Type II glycogenosis. Example of congenital lysosomal disease]. Acta Neurol (Napoli) 1974 0.75
54 A European head injury evaluation chart. Scand J Rehabil Med Suppl 1992 0.75
55 [Psychometric studies in Friedreich's ataxia]. Acta Neurol Belg 1985 0.75
56 [Hallervorden-Spatz disease in a brother and a sister (hereditary-familial fragility of the extrapyramidal apparatus)]. J Genet Hum 1968 0.75
57 Idiopathic recurrent myoglobinuria and persistent weakness. Neurology 1983 0.75