A

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C

D E

Click on Images for Enlarged View

Findings: In this entity perinatal sonographic examination of the head demonstrates linear or branched-linear foci of echogenicity to follow the course of the lenticulostriate branches of the middle cerebral arteries, in the region of the basal ganglia and/or thalami. Right-sided (Figures A & B) and left-sided (Figures C & D) parasagittal images of the thalami illustrate such findings in this case. Duplex ultrasound may reveal a signal consistent with flow in the medium-sized perforating arteries to these nuclei. Color doppler may reveal an arterial pattern, as in Figure (E); color-flow may also be absent, secondary to vasculopathic arterial stenosis.

Diagnosis:Sonographic lenticulostriate vasculopathy.

Discussion: The entity termed mineralizing vasculopathy has been described in a total of approximately 125 cases of perinatal and infantile head ultrasound examinations in the scientific literature, commensurate with a branched-linear pattern of echogenicity in the region of the basal ganglia and thalami. (1-5) These linear hyperechoic foci are noted to follow the course of the lenticulostriate branches of the middle cerebral arteries; these vessels are normally not sonographically distinct from surrounding brain parenchyma.

The lesions related to the entity were originally proposed to be secondary to a cerebrovasculitic response to congenital infection (most often ascribed to rubella, CMV, and syphilis), or, rarely, to chromosomal aberration (trisomy 13) (1,3,4) At autopsy, several case-specimens were defined histopathologically as containing thickened, hypercellular walls in the perforating, medium-sized arteries to the basal ganglia and/or thalami. Perivascular deposits of amorphous basophilic material were proven to be free of calcium deposition, explaining the lack of corroborative CT findings.(3)

Recent studies have broadened the array of clinical entities associated with the entity of "mineralizing vasculopathy" to include, for example, trisomy 21, non-immune hydrops, traumatic head injuries, diabetic fetopathy, and fetal alcohol syndrome, and have described several cases for which no specific underlying disorder could be found.(2,5) Wang, et al, have recently postulated that a wide range of perinatal, acquired, and nonspecific clinical conditions may result in sonographic lenticulostriate vasculopathy - an imaging term which may be more appropriate, given the lack of consistent histopathological findings.(5)

References:
1. Ben-Ami T, Yousefzadeh D, Backus M, Reichman B, Kessler A,
and Hammerman-Rosenberg C. Lenticulostriate vasculopathy in infants
with infections of the central nervous system: sonographic and
doppler findings. Pediatric Radiology: 575-579, 1990.

2. Hughes P, Weinberger E, and Shaw DWW. Linear areas of echogenicity
in the thalami and basal ganglia of neonates: An expanded association. Radiology 179:103-105, 1991.

3. Teele RL, Hernanz-Schulman M, and Sotrel A. Echogenic vasculature
in the basal ganglia of neonates: a sonographic sign of vasculopathy. Radiology 169:423-427, 1988.

4. Toma P, Mezzano P, and Serra G. The "candlestick sign" on
cerebral ultrasound. Pediatric Radiology: 319, 1991.

5. Wang, HS, Kuo MF, and Chang TC. Sonographic lenticulostriate
vasculopathy in infants: some associations and a hypothesis. AJNR 16:97-102, 1995.

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