Published in Pediatrics on October 01, 1969
Correlations between genotype, ultrastructural morphology and clinical phenotype in the neuronal ceroid lipofuscinoses. Neurogenetics (2005) 1.88
Neurophysiological identification of a late infantile form of 'neuronal lipidosis'. J Neurol Neurosurg Psychiatry (1973) 1.29
Infantile type of so-called neuronal ceroid-lipofuscinosis. Histological and electron microscopic studies. Acta Neuropathol (1973) 1.27
Inborn errors of metabolism in neurology (Wilson's disease, Refsum's disease and lipidoses). Proc R Soc Med (1971) 1.20
So-called neuronal ceroid lipofuscinosis. Neurophysiological studies in 60 children. J Neurol Neurosurg Psychiatry (1977) 1.19
The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase. Biochem J (1990) 1.11
Significance of muscle biopsies in neuronal ceroid-lipofuscinoses. J Neurol Neurosurg Psychiatry (1975) 1.07
Reassessment of rectal approach to neuropathology in childhood: review of 307 biopsies over 11 years. Arch Dis Child (1975) 1.03
An adult case of canine neuronal ceroid-lipofuscinosis. Acta Neuropathol (1977) 0.97
Juvenile Batten's disease: an ophthalmological assessment of 26 patients. Br J Ophthalmol (1980) 0.96
Additional electron microscopic observations on two cases of Batten-Spielmeyer-Vogt disease. (Neuronal ceroid-lipofuscinosis). Acta Neuropathol (1971) 0.93
Neuronal ceroid lipofuscinosis (Batten's disease). Arch Dis Child (1972) 0.89
Neuronal ceroid-lipofuscin storage in Siamese cats. Can J Comp Med (1974) 0.89
The electron microscopic study of the appendix as early diagnostic means in Batten-Spielmeyer-Vogt disease. Acta Neuropathol (1972) 0.88
The significance of cytoplasmic inclusions in late infantile and juvenile amaurotic idiocy. An ultrastructural study. Acta Neuropathol (1973) 0.85
[Juvenile neuronal ceroid lipofuscinosis. Ophthalmologic findings and differential diagnosis]. Ophthalmologe (2010) 0.85
A dominant form of adult neuronal ceroidlipofuscinosis (Kufs' disease) with an associated occipital astrocytoma: early diagnosis by cortical biopsy. J Neurol Neurosurg Psychiatry (1976) 0.84
[Neuronal ceroid-lipofuscinosis: ultrastructural study of two cerebral biopsies (author's transl)]. Acta Neuropathol (1974) 0.82
[Pigment variant of the adult type of amaurotic idiocy (Kufs) (author's transl)]. Acta Neuropathol (1973) 0.82
Characterization of the expressed genes for subunit c of mitochondrial ATP synthase in sheep with ceroid lipofuscinosis. Biochem J (1993) 0.81
The role of N-glycosylation in folding, trafficking, and functionality of lysosomal protein CLN5. PLoS One (2013) 0.80
Loss of pigment-laden stellate cells: a severe alteration of the isocortex in juvenile neuronal ceroid-lipofuscinosis. Acta Neuropathol (1978) 0.79
So-called neuronal ceroid-lipofuscinosis. Histochemical study with evidence of extractibility of the stored material. Acta Neuropathol (1977) 0.78
Sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses: an immunological study. Acta Neuropathol (1995) 0.78
A case of adult neuronal ceroid-lipofuscinosis with the appearance of membranous cytoplasmic bodies localized in the spinal anterior horn. Acta Neuropathol (1987) 0.78
Analysis of NCL Proteins from an Evolutionary Standpoint. Curr Genomics (2008) 0.76
Batten's disease. Br J Ophthalmol (1980) 0.75
Electrophysiologic testing and its specific application in unsedated children. Trans Am Ophthalmol Soc (1984) 0.75
Neuronal ceroid lipofuscinosis and arthropathy: a family study. J Med Genet (1981) 0.75
Parkinsonism-physiology and pharmacology. Br Med J (1971) 0.75
The Hermansky-Pudlak syndrome: ultrastructure of bone marrow macrophages. Am J Pathol (1973) 0.75
Subacute sclerosing panencephalitis: isolation of measles virus from a brain biopsy. Nature (1969) 4.49
Subacute sclerosing panencephalitis: propagation of measles virus from brain biopsy in tissue culture. Science (1969) 2.43
Isolation of measles virus from brain cell cultures of two patients with subacute sclerosing panencephalitis. Proc Soc Exp Biol Med (1969) 1.77
Enzymuria in gentamicin-induced kidney damage. Antimicrob Agents Chemother (1975) 1.72
Opsoclonus, occult carcinoma, and chemical pathology in dentate nuclei. Arch Neurol (1967) 1.56
Pathology of the torsion dystonias (dystonia musculorum deformans). Neurology (1970) 1.48
A dominant form of neuronal ceroid-lipofuscinosis. Brain (1971) 1.42
Dystonia musculorum deformans. Clinical, genetic and pathoanatomical studies. Psychiatr Neurol Neurochir (1967) 1.34
Deficiency of alpha-L-fucosidase. Science (1972) 1.28
Persistent infection of BSC-1 cells by defective measles virus derived from subacute sclerosing panencephalitis. Infect Immun (1974) 1.28
Early lesion of Pelizaeus-Merzbacher disease: electron microscopic and biochemical study. J Neuropathol Exp Neurol (1973) 1.13
Dancing eyes, dancing feet: infantile polymyoclonia. Brain (1968) 1.09
Minimally invasive coronary artery bypass grafting: a new method using an anterior mediastinotomy. J Card Surg (1995) 1.09
Significance of muscle biopsies in neuronal ceroid-lipofuscinoses. J Neurol Neurosurg Psychiatry (1975) 1.07
Presidential address: Studies in the neuronal ceroid-lipofuscinoses. J Neuropathol Exp Neurol (1974) 1.05
Absence of cerebral myelin sheaths in a case of presumed Pelizaeus-Merzbacher disease. Electron microscopic and biochemical studies. J Neuropathol Exp Neurol (1969) 1.05
On the ultrastructural diversity and essence of residual bodies in neuronal ceroid-lipofuscinosis. Mech Ageing Dev (1979) 1.02
Reflections on the etiology and pathogenesis of subacute sclerosing panencephalitis. Neurology (1968) 1.01
An ultrastructural study of the retina in the Jansky-Bielschowsky type of neuronal ceroid-lipofuscinosis. Am J Ophthalmol (1977) 0.99
Studies on GM1-gangliosidosis, type II. Acta Neuropathol (1974) 0.98
Peripheral motor and sensory neuropathy of early childhood, simulating Werdnig-Hoffmann disease. Neuropadiatrie (1976) 0.95
Serum clonazepam concentrations in children with absence seizures. Neurology (1975) 0.94
Myositis ossificans progressiva. Case report with metabolic and histochemical studies. Metabolism (1966) 0.92
Angiokeratoma corporis diffusum with alpha-L-fucosidase deficiency. Arch Dermatol (1973) 0.91
The fine structure of the retina in neuronal ceroid-lipofuscinosis. Am J Ophthalmol (1974) 0.90
P-phenylenediamine-mediated peroxidase deficiency in English setters with neuronal ceroid-lipofuscinosis. Lab Invest (1974) 0.89
Hallervorden-Spatz syndrome. Arch Neurol (1977) 0.88
Immunoelectrophoretic changes of serum IgG during subacute inflammatory and demyelinating diseases of the central nervous system. Z Immunitatsforsch Allerg Klin Immunol (1967) 0.87
The control of variables in radiopathological studies on mammalian nervous tissue. Int J Radiat Biol Relat Stud Phys Chem Med (1966) 0.85
Spinal fluid cytomorphology. Description of apparatus, technique, and findings. Arch Neurol (1968) 0.85
Free radicals, anticonvulsants, and the neuronal ceroid-lipofuscinoses. Am J Med Genet (1995) 0.84
Effects of high-LET neon (20Ne) particle radiation on the brain, eyes and other head structures of the pocket mouse: a histological study. Int J Radiat Biol Relat Stud Phys Chem Med (1979) 0.83
Ultrastructural changes in the cerebellum after focal deuteron irradiation. Cerebellar irradiation. J Neuropathol Exp Neurol (1967) 0.81
Dystonia: an overview. Adv Neurol (1976) 0.81
Oxygen effect and selectivity of radiolesions in the mammalian neuraxis. Acta Radiol Ther Phys Biol (1966) 0.80
Oral findings in metachromatic leukodystrophy. Oral Surg Oral Med Oral Pathol (1970) 0.79
Early and long-term effects of deuteron irradiation on cells in the cerebellum of adult mice. J Cell Biol (1968) 0.79
Neurodegenerative diseases of infancy and childhood. Ann Neurol (1983) 0.79
Serological studies of measles and subacute sclerosing panencephalitis. Neurology (1968) 0.79
[Subacute sclerosing leukoencephalitis and the measles virus]. Presse Med (1968) 0.78
Batten disease: ocular features, differential diagnosis and diagnosis by enzyme analysis. Birth Defects Orig Artic Ser (1976) 0.78
The neuronal ceroid-lipofuscinoses--Batten-Vogt syndrome: a model for human aging? Adv Gerontol Res (1971) 0.78
Hereditary nonprogressive athetotic hemiplegia: a new syndrome. Neurology (1977) 0.78
Changes in memory, electrophysiology, neurochemistry and neuronal ultrastructure after deuteron irradiation of the brain in C57B1-10 mice. J Neurochem (1968) 0.77
Ultrastructural investigations of peripheral nerves in neuronal ceroid-lipofuscinoses (NCL). J Neurol (1976) 0.77
Hypokalemic periodic paralysis. Children with permanent myopathic weakness. Neurology (1969) 0.77
Retinal pathology in GM 1 gangliosidosis, type II. Am J Ophthalmol (1973) 0.77
Subacute sclerosing panencephalitis. Clinical aspects and prognosis. The Brazilian registry. Arq Neuropsiquiatr (1999) 0.77
Cellular distribution of lesions in Batten disease. Am J Med Genet (1995) 0.76
The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: XII. Results of examination of the calvarium, brain, and meninges. Aviat Space Environ Med (1975) 0.76
Synaptic plasticity in the cerebral cortex of mice: effects of radiation and anesthesia. J Hirnforsch (1983) 0.76
Morphologic approaches to the nosology of nervous system defects. Birth Defects Orig Artic Ser (1971) 0.75
Marfan's syndrome and dystrophia myotonica. N Engl J Med (1966) 0.75
[Hydrosoluble proteins of the nervous system of man and the animal]. Acta Zool Pathol Antverp (1969) 0.75
Dégénérescence systématisée optico-cochléo-dentelée. Acta Neuropathol (1965) 0.75
Exciting prospects in neurology. J Neurol (1984) 0.75
Opsoclonus, occult carcinoma, and chemical pathology in dentate nuclei. Trans Am Neurol Assoc (1967) 0.75
Editorial: Post-therapeutic radiation injuries of the nervous system. Reflections on their prevention. J Neurol (1976) 0.75
A multicenter study of lomefloxacin versus ciprofloxacin in the treatment of complicated urinary tract infections. Int J Antimicrob Agents (1992) 0.75
Variability of exressivity of alpha-fucosidase deficiency. Adv Exp Med Biol (1976) 0.75
[On the pathogenesis of late radiation damage to the spinal cord]. Strahlentherapie Sonderb (1966) 0.75
[On the significance of eosinophilic leukocytes in neurologic diseases]. Wien Z Nervenheilkd Grenzgeb (1969) 0.75
Alterations of basement membranes of cerebral capillaries. J Neuropathol Exp Neurol (1967) 0.75
Transient tyrosinemia. Am J Dis Child (1968) 0.75
The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: IX Results of examination of the nasal mucosa. Aviat Space Environ Med (1975) 0.75
Studies on alpha albumin in nervous tissue. II. Topographical distribution in normal tissue. Brain Res (1970) 0.75
Magnetic resonance imaging in neuronal ceroid lipofuscinosis. J Comput Tomogr (1987) 0.75
What is amaurotic idiocy? Lipids (1969) 0.75
Measles virus and subacute sclerosing panencephalitis. Science (1968) 0.75
Leukocyte abnormalities in familial amaurotic idiocy. N Engl J Med (1966) 0.75
Immunoglobulin synthesis in subacute sclerosing panencephalitis during x-irradiation of the brain. Lancet (1968) 0.75
Studies on the substantia nigra in Batten's disease. Pathol Eur (1968) 0.75
The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: X. Results of ear examination. Aviat Space Environ Med (1975) 0.75
Brain study in balloon-borne monkeys exposed to cosmic rays. Aerosp Med (1970) 0.75
Studies on alpha albumin in nervous tissue. 3. Observations on pathological specimens. Brain Res (1970) 0.75
Drug-induced cytochemical changes in a glioblastoma. Ann Histochim (1966) 0.75
The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: III. Dosimeter design, construction, and implantation. Aviat Space Environ Med (1975) 0.75
Considerations on a classification of neurolipidoses. Pathol Eur (1968) 0.75
Immunoelectrophoretic abnormalities in the anodic field of the IgG precipitation arc during central nervous system disorders. Z Immunitatsforsch Allerg Klin Immunol (1967) 0.75
Ultrastructural changes in fibroblast mitochondria of a patient with HHH-syndrome. J Inherit Metab Dis (1984) 0.75
Modification of acute radiation effect on cerebellar neurons of mice by actinomycin D. Exp Neurol (1968) 0.75
Sandhoff's disease: a case report including family study and fetal enzyme determination. J Indiana State Med Assoc (1976) 0.75
In memoriam. Willibald Scholz. 1889-1971. J Neuropathol Exp Neurol (1972) 0.75
Recent observations in Jakob-Creutzfeldt disease. J Neuropathol Exp Neurol (1971) 0.75
Atrial dissociation associated with carcinomatosis: case report. Mil Med (1978) 0.75
The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: VI. launch, flight, and recovery. Aviat Space Environ Med (1975) 0.75
Spontaneous rhinorrhea due to pituicytoma. Case report. J Neurosurg (1966) 0.75
Studies on alpha albumin in nervous tissue. I. Biochemical investigations. Brain Res (1970) 0.75