Archives of Neurology

Berg, Leonard

doi: 10.1001/archneur.1985.04210090001001pmid: 4026604

Abstract Consider with me the question in the title, even though it is unanswerable at present. My goal is to present relevant data in a balanced manner and encourage others to devote their investigative talents to providing a definitive answer. However, readers should be warned of my personal bias that the answer is "no." SIGNIFICANCE If Alzheimer's disease (AD) represents an exaggeration of normal aging, these corollaries follow: (1) Normal aging and AD have the same causes and mechanisms. (2) There are only quantitative differences between the two. (3) Given sufficient longevity, everyone will develop AD. (4) The study of one may lead to the solution of both. (5) When caretakers of an elderly, demented person seek our advice only after the illness is advanced and explain, "We thought it was just old age," they are closer to the truth than we thought. (6) Normal agingg of the brain represents very References 1. McKhann G, Drachman D, Folstein M, et al: clinical diagnosis of Alzheimer's disease . Neurology 1984;34:939-944.Crossref 2. Chui HC, Bondareff W, Zarow C, et al: Stability of neuronal number in the human nucleus basalis of Meynert with age . Neurobiol Aging 1984;5:83-88.Crossref 3. Scheibel ME, Lindsay RD, Tomiyasu U, et al: Progressive dendritic changes in the aging human limbic system . Exp Neurol 1976;53:420-430.Crossref 4. Buell SJ, Coleman PD: Quantitative evidence for selective dendritic growth in normal human aging but not in senile dementia . Brain Res 1981;214:23-41.Crossref 5. Kemper T: Neuroanatomical and neuropathological changes in normal aging and in dementia , in Albert ML (ed): Clinical Neurology of Aging . New York, Oxford University Press, 1984, pp 9-52. 6. Tomlinson BE: Plaques, tangles and Alzheimer's disease . Psychol Med 1982;12:449-459.Crossref 7. Research Workshop: Diagnosis of Alzheimer disease. Arch Neurol, in press. 8. DeKosky ST, Bass NH: Aging, senile dementia and the intralaminar microchemistry of cerebral cortex . Neurology 1982;32:1227-1233.Crossref 9. Coben LA, Danziger WL, Berg L: Frequency analysis of the resting awake EEG in mild senile dementia of Alzheimer type . Electroencephalogr Clin Neurophysiol 1982;55:372-380.Crossref 10. Creasy H, Rapaport SI: The aging human brain . Ann Neurol 1985;17:2-10.Crossref 11. Storandt M, Botwinick J, Danziger WL: A longitudinal study of the cognitive performances of patients with mild senile dementia of the Alzheimer type and matched healthy controls, in Poon LW (ed): Handbook for the Clinical Memory Assessment of Older Adults. Washington, DC, American Psychological Association, in press.

Schoenberg, Bruce S.;Anderson, Dallas W.;Haerer, Armin F.

doi: 10.1001/archneur.1985.04210090004002pmid: 4026605

Abstract • The purpose of this investigation was to document the prevalence of severe dementia among different racial groups residing in the same community—Copiah County, Mississippi. Both household and institutionalized populations were included. For either sex, the prevalence ratios of all severe dementia and clinically diagnosed severe senile dementia of the Alzheimer's type were at least as large among blacks as among whites. For either race, the corresponding prevalence ratios were greater in females. For each race and sex, the corresponding prevalence ratios increased with advancing age. Finally, in the population studied, approximately 1% of individuals 40 years old or older had severe dementia. This figure increased to 7% for individuals 80 years old or older. References 1. Anderson DW, Schoenberg BS, Haerer AF: Racial differentials in the prevalence of major neurological disorders: Background and methods of the Copiah County Study . Neuroepidemiology 1982;1:17-30.Crossref 2. Kahn RL, Goldfarb AI, Pollack M, et al: Brief objective measures for the determination of mental status in the aged . Am J Psychiatry 1960;117:326-328. 3. Mortimer JA, Schuman LM, French LR: Epidemiology of dementing illness , in Mortimer JA, Schuman LM (eds): The Epidemiology of Dementia . New York, Oxford University Press, 1981, pp 3-23. 4. Kay DWK, Bergmann K, Foster EM, et al: Mental illness and hospital usage in the elderly: A random sample followed up . Compr Psychiatry 1970;11:26-35.Crossref 5. Akesson HO: A population study of senile and arteriosclerotic psychoses . Hum Hered 1969; 19:546-566.Crossref 6. Roth M: Epidemiological studies , in Katzman R, Terry RD, Bick KL (eds): Alzheimer's Disease: Senile Dementia and Related Disorders . New York, Raven Press, 1978, pp 337-339. 7. Broe GA, Akhtar AJ, Andrews GR, et al: Neurological disorders in the elderly at home . J Neurol Neurosurg Psychiatry 1976;39:362-366.Crossref 8. Schoenberg BS: Neurologic disease in the elderly: Epidemiologic considerations . Semin Neurol 1981;1:5-12.Crossref 9. Siegel JS: Some demographic aspects of aging in the United States , in Ostfeld AM, Gibson DC (eds): Epidemiology of Aging , United States Department of Health, Education, and Welfare publication (NIH)77-711. Bethesda, Md, National Institutes of Health, 1977, pp 17-82. 10. Hill AB: Principles of Medical Statistics , ed 9. New York, Oxford University Press, 1971.

Cutler, Neal R.;Haxby, James;Kay, Arthur D.;Narang, Prem K.;Lesko, Lawrence J.;Costa, Jonathan L.;Ninos, Mary;Linnoila, Markku;Potter, William Z.;Renfrew, James W.;Moore, Angela M.

doi: 10.1001/archneur.1985.04210090008003

Askanas, Valerie;Gallez-Hawkins, Ghislaine

doi: 10.1001/archneur.1985.04210090013004pmid: 3896205

Abstract • In two- to five-week tissue cultures of biopsied adult human skeletal muscle, combined addition to the culture medium of insulin, fibroblast growth factor, and epidermal growth factor synergistically increased creatine kinase activity 17-fold, increased acetylcholine receptors tenfold, and accelerated muscle differentiation. This study provides the first demonstration of the beneficial influence of these peptides on human muscle. It also establishes a new culture medium, resulting in the following: (1) much better longterm growth and differentiation of biopsied adult human muscle; and (2) by allowing elimination of embryo extract and reduction of serum, an important step toward developing a fully defined medium for culturing biopsied adult human normal and pathologic muscle tissue. References 1. Askanas V: Human muscle and Schwann's cells in tissue culture as a tool in studying pathogenesis and treatment of neuromuscular disorders , in Serratrice G, Desnuelle C, Pellissier JF, et al (eds): Neuromuscular Diseases . New York, Raven Press, 1984. 2. Askanas V, Engel WK: Normal and diseased human muscle in tissue culture , in Vinken PJ, Bruyan GW (eds): Handbook of Clinical Neurology . New York, North Holland Publishing Co, 1979. 3. Dollenmeier P, Turner DC, Eppenberger HM: Proliferation and differentiation of chick skeletal muscle cells cultured in a chemically defined medium . Exp Cell Res 1981;135:46-61.Crossref 4. Florini JR, Roberts SB: A serum-free medium for the growth of muscle cells in culture . In Vitro 1979;15:983-992.Crossref 5. Hayashi I, Kobylecki J: Growth of myoblasts in hormone-supplemented serum-free medium , in Soto GH, Pardee AB, Sirbasku DA (eds): Growth of Cells in Hormonally Defined Media . Cold Spring Harbor, NY, Cold Spring Harbor Conference on Cell Proliferation, 1982. 6. Miranda AF, Mongini T: Duchenne muscle culture: Current status and future trends , in Serratrice G, Desnuelle C, Pellissier JF (eds): Neuromuscular Diseases . New York, Raven Press, 1984, pp 365-371. 7. Tahmoush AJ, Askanas V, Nelson PG, et al: Electrophysiologic properties of aneurally cultured muscle from patients with myotonic muscular atrophy . Neurology 1983;33:311-316.Crossref 8. Askanas V, Engel WK: A new program for investigating adult human skeletal muscle grown aneurally in tissue culture . Neurology 1975;28:58-67.Crossref 9. Vogel Z, Sytkowski AJ, Nierenberg MW: Acetylcholine receptors of muscle grown in vitro . Proc Nail Acad Sci USA 1982;69:3180-3184.Crossref 10. Turner DC, Maier V, Appenberger HN: Creatine kinase and aldolase isoenzyme transitions in cultures of chick skeletal muscle cells . Dev Biol 1974;37:63-89.Crossref 11. Lowry OH, Rosebrough NY, Farr AL, et al: Protein measurement with the folin phenol agent . J Biol Chem 1951;193:265-275. 12. Hess YW, Murdock KY, Natho GYW, et al: Creatine phosphokinase: A spectrophotometric assay with improved sensitivity . Am J Clin Pathol 1968;50:89-97. 13. Florini LR, Ewton DZ: Insulin acts as a somatomedin analog in stimulating myoblast growth in serum-free medium . In Vitro 1981; 17:763-768.Crossref 14. Mandel JL, Pearson ML: Insulin stimulates myogenesis of rat myoblast line . Nature 1979; 251:618-620.Crossref 15. Sandra A, Przybylski RY: Antogeny of insulin binding during chick skeletal myogenesis in vitro . Dev Biol 1979;68:546-556.Crossref 16. Linkhart TA, Clegg CH, Hauschka SD: Control of mouse commitment to terminal differentiation by mitogens . J Supramolec Str 1980; 14:483-498.Crossref 17. Gospodarowicz D, Weseman J, Moran JS, et al: Effect of fibroblast growth factor on the division and fusion of bovine myoblasts . J Cell Biol 1970;70:395-405.Crossref 18. Cohen S: Isolation of a mouse submaxillary gland protein accelerating incisor eruption eyelid opening in the newborn animal . J Biol Chem 1962;237:1555-1562. 19. Carpenter G: Epidermal growth factor: Biology and mechanism of action . Birth Defects 1980;16:61-72. 20. Gospodarowicz D: Localization of a fibroblast growth factor and its effect alone and with hydrocortisone on 3T3 cell growth . Nature 1974: 249:123-127.Crossref 21. King GL, Kahn CR, Rechler MM, et al: Direct demonstration of separate receptors for growth and metabolic activities of insulin and multiplication stimulating activity (an insulinlike growth factor) using antibodies to the insulin receptor . J Clin Invest 1980;66:130-140.Crossref 22. Gospodarowicz D, Moran JS: Growth factors in mammalian cell culture . Ann Rev Biochem 1976;45:431-558.Crossref 23. Nilsen-Hamilton M, Hamilton RT, Allen WR, et al: Synergistic stimulation of S6 ribosomal protein phosphorylation and DNA synthesis by epidermal growth factor and insulin in quiescent 3T3 cells . Cell 1982;31:237-242.Crossref 24. Kato Y, Hinaki Y, Ingue H, et al: Differential and synergistic actions of somatomedin-like growth factors, fibroblast growth factor, and epidermal growth factor in rabbit costal chondrocytes . Eur J Biochem 1983;129:685-690.Crossref 25. Linkhart A, Lim RW, Hauschka SD: Regulation of normal and variant mouse myoblast proliferation and differentiation by specific growth factors , in Sato CH, Pardee AB, Sirbasku DA (eds): Growth of Cells in Hormonally Definat Media . Cold Spring Harbor, NY, Cold Spring Harbor Conference on Cell Proliferation, 1982. 26. Cohen S, Carpenter G: Human epidermal growth factor: Isolation and chemical and biolog ical properties . Proc Natl Acad Sci USA 1975. 72:1317-1321.Crossref

Gross, Kenneth B.;Skrivanek, Joseph A.;Carlson, Kenneth C.;Kaufman, David M.

doi: 10.1001/archneur.1985.04210090017005pmid: 4026606

Abstract • A 46-year-old man, the son of normal nonconsanguinous parents, had a rare progressive neurological illness that began in midlife and was characterized by seizures, buccolingual dyskinesia, orofacial tics, choreiform movements, areflexia, and neurogenic muscle atrophy. Acanthocytosis was present. The serum creatine kinase level was elevated and normobetalpoproteinemia was noted. A brother had a similar disorder. These clinical and laboratory characteristics are consistent with those of previously reported cases of neuroacanthocytosis, ie, familial amyotrophic chorea with acanthocytosis (FACWA). Features not previously reported in cases of FACWA and noted in our index patient are as follows: (1) an increased level of free sialic acid was detected in the serum; (2) haloperidol administered in high doses decreased the orofacial tics; (3) absence of the McLeod blood group phenotype was noted; and (4) Hispanic-Puerto Rican ancestry was documented. References 1. Aminoff MJ: Acanthocytosis and neurological disease . Brain 1972;95:749-760.Crossref 2. Bird TD, Cederbaum S, Valpey RW, et al: Familial degeneration of the basal ganglia with acanthocytosis: A clinical, neuropathological and neurochemical study . Ann Neurol 1978;3:253-258.Crossref 3. Critchley EMR, Clark DB, Wikler A: Acanthocytosis and neurological disorder without abetalipoproteinemia . Arch Neurol 1968;18:134-140.Crossref 4. Critchley EMR, Nicholson JT, Betts JJ, et al: Acanthocytosis, normolipoproteinemia and multiple tics . Postgrad Med J 1970;46:698-701.Crossref 5. Estes JW, Morley TJ, Levine IM, et al: A new hereditary acanthocytosis syndrome . Am J Med 1967;42:868-881.Crossref 6. Kito S, Itoga E, Hiroshege Y, et al: A pedigree of amyotrophic chorea with acanthocytosis . Arch Neurol 1980;37:514-517.Crossref 7. Sakai T, Mawatari S, Iwashitu H, et al: Choreoacanthocytosis: Clues to clinical diagnosis . Arch Neurol 1981;38:335-338.Crossref 8. Warren L: The thiobarbituric acid assay of sialic acids . J Biol Chem 1959;234:1971-1975. 9. Cederbaum S, Haywood D, Aigner R, et al: Progressive chorea, dementia and acanthocytosis: A genocopy of Huntington's chorea, abstracted . Clin Res 1971;19:177. 10. Levine IM, Estes JW, Looney JM: Hereditary neurological disease with acanthocytosis . Arch Neurol 1968;19:403-409.Crossref 11. Yamamoto T, Genjiro H, Shimazaki K, et al: Movement disorders of familial neuroacanthocytosis syndrome . Arch Neurol 1982;39:298-301.Crossref 12. Bassen FA, Kornzweig AL: Malformation of erythrocytes in a case of atypical retinitis pigmentosa . Blood 1950;5:381-387. 13. Becroft DMO, Costello JM, Scott PJ: Abetalipoproteinemia (Bassen-Kornzweig syndrome): Report of a case . Arch Dis Child 1965; 40:40-46.Crossref 14. Cooper RA, Durocher JR, Leslie MH: Decreased fluidity of red cell membrane lipids in abetalipoproteinemia . J Clin Invest 1977;60:115-121.Crossref 15. Schwartz JF, Rowland LP, Eder H, et al: Bassen-Kornzweig syndrome: Deficiency of serum β-lipoprotein . Arch Neurol 1963;8:438-454.Crossref 16. Symmans WA, Shepherd CS, Marsh WL, et al: Hereditary acanthocytosis associated with McLeod phenotype of the Kell blood group system . Br J Haematol 1979;42:575-583.Crossref 17. Biemer JJ: Acanthocytosis: Biochemical and physiological considerations . Ann Clin Lab Sci 1980;10:238-249. 18. Galey WR, Evan AP, Van Nice PS, et al: Morphology and physiology of the McLeod erythrocyte . Vox Sung 1978;34:152-161.Crossref 19. Schwartz SA, Marsh WL, Symmans A, et al: 'New' clinical features of McLeod syndrome, abstracted . Transfusion 1982;22:404. 20. Scheinberg P: Disorders of movement, tone and coordination , in Modern Practical Neurology , ed 2. New York, Raven Press, 1981, pp 87-112. 21. Maury P: Increased excretion of two sialic acid-containing trisaccharides in the urine of patients with rheumatoid arthritis . Eur J Clin Invest 1978;8:405-409.Crossref 22. Maury CPJ, Sjoblom C, Wegelius O: Urinary excretion of sialic acid-containing saccharides in systemic lupus erythematosus . Arthritis Rheum 1981;24:1137-1141.Crossref 23. Papadopoulos NM, McLane JE, O'Doherty D, et al: Cerebrospinal fluid neuraminic acid in parkinsonism and schizophrenia . J Nerv Ment Dis 1959;128:450-452.Crossref 24. Papadopoulos NM: Sialic acid in cerebral tissue in neurological disease . Excerpta Medica Int Congress Series 1961;38:84. 25. Gross KB, Skrivanek JA, Emeson EE: Ganglioside abnormality in amyotrophic chorea with acanthocytosis . Lancet 1982;2:772.Crossref

Trauner, Doris A.

doi: 10.1001/archneur.1985.04210090021006pmid: 4026607

Abstract • Olivopontocerebellar atrophy is a hereditary disorder that has variable clinical manifestations. Five types have been described, as well as a sixth that contains sporadic cases. This report describes a family with three affected members who demonstrate a composite of types III and V. Their features include progressive spasticity, ataxia, dementia, visual loss with retinal pigmentation, dysarthria, ophthalmoplegia, and chorea. This family might represent an additional category of the disease. In the two family members who developed chorea, baclofen resulted in marked improvement with abolition of the choreiform movements. Response has been sustained for several years in the mother and for eight months in the daughter. Neither has experienced any return of chorea while receiving treatment. When attempts were made to discontinue baclofen, choreiform movements returned promptly and with their original severity. Baclofen, a γ-aminobutyric acid analogue, may be useful in the treatment of other forms of chorea as well. References 1. Menzel P: Beitrag zur Kenntniss der hereditarer Ataxic und Kleinhirnatrophie . Arch Psychiatrie 1891;22:160-166.Crossref 2. Konigsmark BW, Weiner LP: The olivopontocerebellar atrophies: A review . Medicine 1970;49:227-241.Crossref 3. Havener WH: Cerebellar-macular abiotrophy . Arch Ophthalmol 1951;45:40-43.Crossref 4. Carpenter S, Schumacher GA: Familial infantile cerebellar atrophy associated with retinal degeneration . Arch Neurol 1966;14:82-94.Crossref 5. Weiner LP, Konigsmark BW, Stoll J, et al: Hereditary olivopontocerebellar atrophy with retinal degeneration . Arch Neurol 1967;16:364-376.Crossref 6. Jampel RS, Okazaki H, Bernstein H: Ophthalmoplegia and retinal degeneration associated with spinocerebellar ataxia . Arch Ophthalmol 1961;66:123-135.Crossref

Rolak, Loren A.

doi: 10.1001/archneur.1985.04210090023007pmid: 4026608

Abstract • Flight of colors (FOC), the rapidly changing series of colored afterimages perceived When a bright light briefly strikes the eye, is impaired or absent in patients with, lesions affecting central visual fields, especially optic neuropathies (ONS). The effectiveness of a bedside test of FOC using a pocket flashlight was compared with that of pattern-reversal visual evoked responses (PRVERs) in examining 74 subjects): 20 controls, seven patients With ON not due to multiple sclerosis (MS), 26 patients with MS, and 21 patients with possible MS and no clinical ON. The FOC test correctly identified 95 of 99 normal eyes and 45 of 49 eyes with ON, and accurately diagnosed 140 (95%) of 148 eyes overall. In 84 eyes examined by PRVER and FOC, the results agreed in 73 cases (87%), including those of subclinical ON. References 1. Matthews WB, Small DG, Small M, et al. Pattern reversal evoked visual potential in the diagnosis of multiple sclerosis . J Neurol Neurosurg Psychiatry 1977;40:1009-1014.Crossref 2. Feldman M, Todman L, Bender MB: Flight of colors in lesions of the visual system . J Neurol Neurosurg Psychiatry 1974;37:1265-1272.Crossref 3. Mourik J, van Donselaar CA, Minderhoud JM: Disturbed flight of colors in multiple sclerosis . Lancet 1978;1:105. 4. Swart S, Millac P: A comparison of flight of colors with visually evoked responses in patients with multiple sclerosis . J Neurol Neurosurg Psychiatry 1980;43:550-551.Crossref 5. Berry W: The flight of colors in the after image of a bright light . Psychol Bull 1922;19:307-337.Crossref 6. McAlpine D, Lumsden C, Acheson E: Multiple Sclerosis: A Reappraisal , ed 2. London, Churchill Livingston, 1972. 7. Coltheart M: The persistences of vision Philos Trans R Soc Lond Biol 1980;290:57-69.Crossref

Graff-Radford, Neill R.;Schelper, Robert L.;Ilinsky, Igor A.;Damasio, Hanna

doi: 10.1001/archneur.1985.04210090025008pmid: 4026609

Abstract • A 70-year-old man had a stroke and became unconscious. High-resolution computed tomography (CT) with 5-mm cuts disclosed bilateral thalamic infarctions, larger on the left than the right. He died one week later, and a postmortem examination was performed. By plotting the CT on templates constructed to show the different vascular territories of the thalamus, the infarctions were predicted to be in the territories of the interpeduncular profunda arteries. Comparing sagittal reconstructions to the Schaltenbrand and Wahren atlas, the following thalamic nuclei were thought to be involved: dorsomedial, parafascicular, and centrum medianum bilaterally; and reticular, ventroanterior, and ventrolateral on the left. Pathologic study confirmed these findings. We believe that it is possible to predict the vascular territory of thalamic infarctions by plotting the CT on templates showing the different vascular territories of the thalamus. Sagittal reconstructions of CT scans also permit the determination of thalamic nuclei involved in a lesion. References 1. Graff-Radford NR, Eslinger PJ, Damasio AR, et al: Non-hemorrhagic infarction of the thalamus: Behavioral, anatomical and physiologiical correlates . Neurology 1984;34:14-23.Crossref 2. Schlesinger B: The Upper Brain Stem in the Human: Its Nuclear Configuration and Vascular Supply . New York, Springer Verlag, 1971. 3. Archer CR, Ilinsky IA, Goldfader PR, et al: Aphasia in thalamic stroke: CT stereotactic localization . J Comput Assist Tomogr 1981;5:427-432.Crossref 4. Schaltenbrand G, Wahren W: Atlas for Stereotaxy of the Human Brain . Stuttgart, West Germany, Georg Thieme Verlag, 1977. 5. Castaigne P, Lhermitte F, Buge A, et al: Paramedian, thalamic and midbrain infarcts: Clinical and neuropathological study . Ann Neurol 1981;10:127-148.Crossref

Hammond, Edward J.;Wilder, B. Joe;Goodman, Ira J.;Hunter, Stephen B.

doi: 10.1001/archneur.1985.04210090031009pmid: 4026610

Abstract • Although there have been extensive anatomical and physiological studies in animals, the actual neural sources, or even the laterality, of some components of auditory brain-stem evoked potentials in humans are uncertain. We studied these responses in a 56-year-old patient who had a clearly demarcated pontine hemorrhage on the right side. The patient Was somnolent, with dense left hemiplegia and signs of involvement of right cranial nerves V, VI, and VII. Stimulation of the left ear (ie, contralateral to the lesion) evoked a normal series of waves with clearly resolved positive components Peaking at 2.0, 3.3, 4.8 (wave IV), and 6.0 ms (wave V). Stimulation of the right ear (ie, ipsilateral to the lesion) evoked only waves I, III, and IV. These results suggest that a pathway ipsilateral to the stimulated ear is necessary and sufficient for generation of auditory wave V and that wave IV is generated in bilateral pathways. References 1. Achor LJ, Starr A: Auditory brainstem responses in the cat: II. Effects of lesions . Electroencephalogr Clin Neurophysiol 1980;48:174-190.Crossref 2. Buchwald JS, Huang CM: Far-field acoustic response: Origins in the cat . Science 1975;189:382-384.Crossref 3. Chiappa KH: Evoked Potentials in Clinical Medicine: Pattern-Shift Visual, Brainstem Auditory and Short-Latency Somatosensory . New York, Raven Press, 1983. 4. Brown RH, Chiappa KH, Brooks EB: Brainstem auditory evoked responses in 22 patients with intrinsic brainstem lesions: Implications for clinical interpretations . Electroencephalogr Clin Neurophysiol 1981;51:38P. 5. Parker SW, Chiappa KH, Ojemann RG: Auditory pathway dysfunction in patients with posterior fossa meningiomas . Neurology 1983; 33( (suppl 2) ):206.Crossref 6. Oh SJ, Kuba T, Soyer A, et al: Lateralization of brainstem auditory evoked potentials . Neurology 1981;31:14-18.Crossref 7. Starr A, Squires K: Distribution of auditory brainstem potentials over the scalp and nasopharynx in humans . Ann NY Acad Sci 1982; 388:427-442.Crossref 8. Wada SI, Starr A: Generation of auditory brain stem responses (ABRs): III. Effects of lesions of the superior olive, lateral lemniscus, and inferior colliculus on the ABR in guinea pig . Electroencephalogr Clin Neurophysiol 1983; 56:352-366.Crossref 9. Morest DK: Structural organization of the auditory pathways , in Tower D (ed): The Nervous System . New York, Raven Press, 1975, vol 3, pp 19-29. 10. Adams JC: Ascending projections to the inferior colliculus . J Comp Neurol 1979;183:519-538.Crossref 11. Strominger NL, Nelson LR, Dougherty WJ: Second order auditory pathways in the chimpanzee . J Comp Neurol 1977;172:349-366.Crossref

Morgan, Seth A.;Emsellem, Helene A.;Sandler, Jeffrey R.

doi: 10.1001/archneur.1985.04210090033010pmid: 4026611

Abstract • A patient with absence of the septum pellucidum, optic hypoplasia, congenital nystagmus, hemiatrophy, and seizures fulfilled clinical and radiological criteria for diagnosis of both septo-optic dysplasia and the syndrome of absent septum pellucidum with porencephalies. The anatomical and clinical similarities between these two syndromes suggest a common embryological basis. Their simultaneous presence in this case further supports this explanation. Clinically mild forms of both septo-optic dysplasia and the syndrome of absent septum pellucidum with porencephalies are now detected with the aid of computed tomographic scanning in patients with unexplained hemiatrophy, congenital nystagmus, seizures, and short stature. References 1. Brook CGD, Sanders MD, Hoare RD: Septooptic dysplasia . Br Med J 1972;3:811-813.Crossref 2. DeMorsier G: Etudes sur les dysraphies cranioencéphaliques: III. Agénésie du septum lucidum avec malformation du tractus optique: La dysplasie septo-optique . Schweiz Arch Neurol Neurochir Psychiatr 1956;77:267-292. 3. DeMorsier G: Median craniocephalic dysraphias and olfaclogenital dysplasia . World Neurol 1962;3:485-503. 4. Aicardi J, Goutiere F: The syndrome of absence of the septum pellucidum with porencephalies and other developmental defects . Neuropediatrics 1981;12:319-329.Crossref 5. Acers TE: Optic nerve hypoplasia: Septooptic-pituitary dysplasia syndrome . Trans Am Ophthalmol Soc 1981;79:425-457. 6. Ellenberger C, Runyan TE: Holoprosencephaly with hypoplasia of the optic nerves, dwarfism and agenesis of the septum pellucidum . Am J Ophthalmol 1970;70:960-967. 7. Gross H, Simányi M: Porencephaly , in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology . Amsterdam, North-Holland Publishing Co, 1977, vol 30, pp 681-692. 8. Rush JA, Bajandas FJ: Septo-optic dysplasia (deMorsier syndrome) . Am J Ophthalmol 1978;86:202-205. 9. Bannwarth A: Über den Nachweis von Gehirnmissbildungen durch das Röngenbild und über seine klinische Bedeutung . Arch Psychiatr Nervenkr 1939;110:314-364.Crossref 10. Trzebicki J, Golabek R: Anomalies du septurn pellucidum . Ann Radiol 1969;12:493-497.