血管異常を伴う進行性顔面半側萎縮症の1例

CASE REPORT:PROGRESSIVE FACIAL HEMIATROPHY ASSOCIATED WITH CEREBROVASCULAR ABNORMALITY

I．はじめに

　進行性顔面半側萎縮症は1825年Parryにより初めて報告され，1871年EulenburgによりHemiatrophiafaciei progressivaと命名された^1）。以来欧米に於ては多数の報告例があり，既に1932年Archambault andFromm^2）は400余の報告例を集め，その成因に就いて検討している。本邦に於ての報告例は明治27年の和辻の報告を第1例とし^3），125例に達する^4）。しかしその成因に関する学説は様々であり，未だに定説をみない。最近我々は脳血管写上，血管異常を認めた本症の1例を経験したので，ここに報告すると共に若干の文献的考察を加えつつ，本症の成因に関する我々の考えを述べてみたい。

A case of progressive facial hemiatrophy associatedwith the congenital cerebrovascular anomaly andvarious neurological symptoms was reported inthis paper. The 14-year-old girl was first noticedto demonstrate facial hemiatrophy of the right sideat the age of three years. The atrophy had beenprogressive although no other abnormality wasnoticed until she became age of 14 when she de-veloped sudden episode of general convulsion. Thepatient was admitted to the Juntendo UniversityHospital for a general neurological examination onAugust 27, 1973. Examinations and findings onadmission: The distribution of the facial atrophywas limited to the right side of the face and scalpwhere one could see the deformity of the nostriland forehead and also V-shaped alopecia in thefrontal region of the head. On general neurologicalexamination, no abnormal finding could be foundexcept for the following, that is, optic fundi werecharacterized by bitemporal pallor and diffusenarrowing of retinal arteries. On the ophthalmicperimetry, irregular shaped defects of visual fieldwere demonstrated. Cerebrospinal fluid pressurewas 180 mmH₂O, although its chemical compositionwas normal. EEG showed delta waves in the leftfrontal area only by induced hyperventilation. X-ray studies of the skull demonstrated the decreasedcranial volume on the right side which seemed tobe caused by atrophy of the cranial vault. Carotidangiography revealed the slight shift of the anteriorcerebral artery to the left side. One tiny branchof the anterior temporal artery was connected withbeaded abnormal vessels. PEG disclosed the dis-placement of anterior portion of lateral ventricletoward left.

From these data, it can be explained that in-creased intracranial pressure, EEG abnormality andconvulsive seizure were produced by the decreasedsize of cranial cavity. On the other hand, otherfindings such as visual field defect or abnormaloptic fundi etc, were hard to be explained by thesame category. If we take a count of the vascularanomaly shown in CAG as a part of the patho-genesis of progressive facial hemiatrophy, we mayinclude aforementioned visual abnormalities in thecategory of the embrional developmental anomalies.

One may assume that an unknown prenatal dis-order might inflict upon the diencephalon and theprimitive trigeminal and ophthalmic nerves, bothare in the anatomically close vicinity at the earlyembriological stage. Although this is largelyspeculative, complicated clinical manifestation ap-peared widely in both mesodermal and ectodermalelements seems to be reasonably explained withthis theory.