This paper describes a previously sufficiently functioning 57 year old man who presented with a recent onset of frontal behaviour. Partial agenesis of corpus callosum was an incidental finding on a computerized tomography scan. The EEG was within normal limits and neuropsychological testing did not reveal any interhemispheric disconnection. A SPECT-Scan revealed bilateral hypoperfusion, consistent with Alzheimer's dementia. Normal functioning up to 50 years of age and a later manifestation of Alzheimer's disease along with agenesis of carpus callosum is of clinical interest as such an association has not been published.
References
1.
JeretJ.S., SerurD., WisniewskiK.E.Clinicopathological findings associated with agenesis of the corpus callosum.Brain Dev1987; 9: 255–263.
2.
KendallB.E.Dysgenesis of corpus callosum.Neuroradiology1983; 125: 239–256.
3.
FerrerI., CusiM.V., LiarteA.A Golgi study of the polymicrogyric cortex in Aicardi syndrome.Brain Dev1986; 8(5): 518–525.
4.
KolodnyE.H.Dysgenesis of the corpus callosum: a marker for inherited metabolic disease?Neurology1989; 39: 847–848.
5.
SerurD., JeretJ.S., WisniewskiK.Agenesis of the corpus callosum: clinical, neuroradiological and cytogenetic studies.Neuropediatrics1988; 19: 87–91.
6.
BruyerR., DupuisM., OphovenE.Anatomical and behavioral study of a case of asymptomatic callosal agenesis.Cortex1985; 21(3): 417–430.
7.
MenkesJ.H., PhillipartM., ClarkD.B.Hereditary partial agenesis of corpus callosum.Arch Neurol1964; 11: 198–208.
8.
PinedaM., GonzalesA., FabriguesJ.Familial agenesis of the corpus callosum with hypothermia and apneic spells.Neuropediatric J1984; 15: 63–67.
9.
TorielloH.V., CareyJ.C.Corpus callosum agenesis, facial anomalies, Robin sequence and other anomalies: a new autosomal recessive syndrome?Am J Med Genet1988; 31: 17–23.
10.
AndermannE.Familial agenesis of the corpus callosum with sensorimotor neuropathy. In: MyrianthopoulosN.C., ed., Handbook of clinical neurology.Neurogenic Directory, Northern Holland, Amsterdam, 1971.
11.
GrubbenC., de CockP., BorghraefM.Severe pre- and post-natal growth retardation, developmental delay with hypotonia and marked hypotrophy of the distal extremities, dental anomalies, and eczematous skin. A new autosomal recessive entity.Clin Genet1992; 41(1): 16–21.
12.
JeretJ.S., SerurD., WisniewskiR.Frequency of agenesis of the corpus callosum in the developmentally disabled population as determined by computerized tomography.Pediatr Neurosci1986; 12: 101–103.
13.
LynnR.B., BuchananD.C., FenichelG.M.Agenesis of the corpus callosum.Arch Neurol1980; 37(77): 444–445.
14.
ViciC.D., SabettaG., GambararaM.Agenesis of the corpus callosum, combined immunodeficiency, bilateral cataract, and hypopigmentation in two brothers.Am J Med Genet1988; 29(1): 1–8.
15.
KaplanP./linked recessive inheritance of agenesis of the corpus callosum.J Med Genet1983; 20(2): 122–124.
16.
KimS., TamakiN., MatsumotoS.Corpus Callosum agenesis and associated malformations.No To Hattatsu Brain Dev1989; 21(1): 14–18.
17.
LuefG., BirbamerG., MarosiM.Agenesis of the corpus callosum and epilepsy in two brothers. Neurophysiological and MRI features.Wiener Klinische Wochenschrift1992; 104(10): 301–304.
18.
MathieuJ., BedardF., PrévostC.Motor and sensory neuropathies with or without agenesis of corpus callosum: a radiological study of 64 cases.Can J Neurolog Sci1990; 17(2): 103–108.
19.
HamanoK., MatsubaraT., ShibataS.Aicardi syndrome accompanied by auditory disturbance and multiple brain tumors.Brain Dev1991; 13(6): 438–441.
20.
Shen-SchwarzS., HillL.M., SurtiU.Deletion of terminal portion of 6q: report of a case with unusual malformations.Am J Med Genet1989; 32(1): 81–86.
21.
Delozier-BlanchetC.D., PitmonD., SchorderetD.Cridu-Chat syndrome and two other deformed children in a family carrying a pericenric inversion or insertion of chromosome 5.J Genet Hum1985; 33(5): 371–380.
22.
de FranceH.F., BeemerF.A., SendersR.C.Partial trisomy 11q due to paternal t(11q; 18p); further delineation of the clinical picture.Clin Genet1984; 25(3): 295–299.
23.
ZamboniG., BernardiF., DanesinoC.Agenesis of the corpus callosum with mosaicism 46,XY/47,XY, extra ring chromosome.J Med Genet1980; 17(1): 59–61.
24.
AbeK., MitsudomeA., OgataH.A case of Aicardi syndrome with moderate psychomotor retardation.No To Hattatsu Brain Dev1990; 22(4): 376–380.
25.
OhtsukiH., HaebaraH., TakahashiK.Aicardi's syndrome — report of an autopsy.Neuropediatrics1981; 12(3): 279–286.
26.
SchinzelA., SchmidW.Halux duplication, postaxial Polydactyly, absence of the corpus callosum, severe mental retardation, additional anomalies in two unrelated patients: a new syndrome.Am J Med Genet1980; 6: 241–249.
27.
MoeschlerJ.B., PoberB.R., HolmesL.B.Acrocallosal syndrome: new findings.Am J Med Genet1989; 32(3): 306–310.
28.
LegiusE., FrynsJ.P., CasaerP.Schinzel acrocallosal syndrome: a variant example of the Greig syndrome?Annal Genet1985; 28(4): 239–240.
29.
KunzeJ., KaufmannH.J.Greig cephalopolysyndactyly syndrome. Report of a sporadic case.Helvetica Paediatrica Acta1985; 40(6): 489–495.
30.
MarchallR.E., SmithD.W.Frontodigital syndrome. A dominant inherited disorder with normal intelligence.J Pediatrics1970; 77: 129–133.
DegreefG., LantosG., BogertsB.Abnormalities of the septum pellucidum on MR scans in first-episode schizophrenic patients (see Comments).Am J Neuroradiol1992; 13(3): 835–840.
33.
RubakowskiJ.The antidepressant effect of carbamazepine in callosal agenesis. Acasereport.Pharmacopsychiatry1992; 25(3): 154–156.
34.
TanaghowA., LewisJ., JonesG.H.Anterior tumors of the corpus callosum with atypical depression.Br J Psychiatry1989; 155: 854–858.
35.
VelekM., WhiteL.E.Jr, WilliamsJ.P.Psychosis in a case of corpus callosum agenesis.Alabama Med1988; 58(6): 27–29.
36.
LewisS.W., ReveleyM.A., DavidA.S.Agenesis of the corpus callosum and schizophrenia: a case report.Psychol Med1988; 18(2): 341–347.