CEREBRAL HEMIATROPHY - keywords
cerebral hemiatrophy
references to cerebral hemiatrophy
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Abdul Rashid AM, Md Noh MSF (2018) Dyke-Davidoff-Masson syndrome: a case report. BMC Neurol 29;18(1):76.
Bagazgoitia L, García-Peñas JJ, Duat-Rodríguez A, Hernández-Martín A, Torrelo A (2010) Facial capillary malformation and Dyke-Davidoff-Masson syndrome. Pediatr Neurol 43(3):202-4.
Chan GM, Nelson LS (2004): More on blue cohosh and perinatal stroke. N Engl J Med 351: 2239-41; author reply 2239-41.
Erbayraktar S, Tekinsoy B, Acar F, Acar U (2001) Posttraumatic isolated infarction in the territory of Heubner’s and lenticulostriate arteries. Kobe J Med Sic 47: 113-121.
Finkel RS, Zarlengo KM (2004) Blue cohosh and perinatal stroke. N Engl J Med 351, 302-3.
Govaert P, Vanhaesebrouck P, de Praeter C (1992) Traumatic neonatal intracranial bleeding and stroke. Arch Dis Child 67:840-5.
Jan MM, Camfield PR (1998) Outcome of neonatal stroke in full-term infants without significant birth asphyxia. Eur J Pediatr 157, 846-8.
Koelfen W, Freund M, Varnholt V (1995) Neonatal stroke involving the middle cerebral artery in term infants: clinical presentation, EEG and imaging studies, and outcome. Dev Med Child Neurol 37(3):204-12.
Kourtopoulos H, Lindgren S, Oberg L (2000) Ruptured intracranial aneurysms in infancy. Diagnostic difficulties and overall reflections associated with the surgical treatment and the treatment of vasospasm. Acta Neurochir (Wien) 142:1425–1426.
Mazumdar A, Mukherjee P, Miller JH, Malde H, McKinstry RC (2003) Diffusion-weighted imaging of acute corticospinal tract injury preceding Wallerian degeneration in the maturing human brain. AJNR Am J Neuroradiol 24(6):1057-66.Piro E, Piccione M, Marrone G, Giuffrè M, Corsello G (2013) Dyke-Davidoff-Masson syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery. Ital J Pediatr 14;39:32.
Reynolds EW, Riel-Romero RM, Bada HS (2007) Neonatal abstinence syndrome and cerebral infarction following maternal codeine use during pregnancy. Clin Pediatr (Phila) 46(7):639-45.
Sener RN (1995) Hemimegalencephaly associated with contralateral hemispheral volume loss. Pediatric Radiology 25:387–388.
Steinbok P, Haw CS, Cochrane DD, Kestle JR (1995) Acute subdural hematoma associated with cerebral infarction in the full-term neonate. Pediatr Neurosurg 23(4):206-15.
Stewart RM, Williams RS, Lukl P, Schoenen J (1978) Ventral porencephaly: a cerebral defect associated with multiple congenital anomalies. Acta Neuropathologica 42:231–235.
Talvik I, Peet A, Laugesaar R, Lintrop M, Talvik T (2010) Vehicle-associated closed trauma-induced stroke in a 27-day-old girl. Medicina (Kaunas) 46:624-7.
Vosskämper M, Schachenmayr W (1990) Cerebral hemiatrophy: a clinicopathological report of two cases with a contribution to pathogenesis and differential diagnosis. Clinical Neuropathology 9:244–250.
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typical images
Congenital cerebral hemiatrophy is rare. Observations suggest it follows fetal vascular disruption encompassing anomalies of the circle of Willis such as absence of an ACA or MCA.
Early fetal MCA or internal carotid artery occlusion may be an acquired cause of unilateral cerebral tissue loss.
In syndromes that cause asymmetrical disturbance of white matter, for instance on an infective or ischaemic basis, the ventricle will be strongly dilated and calcium deposition may be found together with subcortical cysts.
Extensive unilateral polymicrogyria may also be suspected in some presentations of hemiatrophy.
Dyke-Davidoff-Masson syndrome (DDMS) has been
described mainly in late childhood, adolescence and adult ages (Piro et al. 2013, Abdul Rashid et al. 2018). The salient features are cerebral hemiatrophy, contralateral hemiplegia, facial asymmetry, developmental delay, cognitive impairment and seizures. Congenital and acquired presentations of DDMS are recognized. In the congenital type structural anomalies of brain arteries were considered responsible for hemispheric atrophy. Hyperpneumatization of the sinuses on the affected side and compensatory calvarial thickening have been documented. Facial capillary malformation has been associated in rare instances. Prenatal unilateral ventriculomegaly is an important indirect finding of cerebral tissue loss. Early postnatal brain MRI and contrast enhanced-MRI angiography, shows cerebral left hemiatrophy associated with reduced caliber of the left MCA characteristic of the Dyke-Davidoff-Masson syndrome.
ventral porencephaly
literature examples
cerebral hemiatrophy
Cerebral hemiatrophy has also been mentioned in infants with seizures in the hemisphere contralateral to hemimegalencephaly (with a lissencephalic appearance) and it can be seen in a hemisphere with extensive polymicrogyria (Sener 1995). Facing a difference in size between both cerebral hemispheres, distinction is to be made between hemimegalencephaly and hemiatrophy. In both the lateral ventricle may be dilated on the affected side. The OFC does not always betray the underlying problem, although macrocrania should typically be associated with megalencephaly and microcrania with hemiatrophy. Even when hemimegalencephaly gradually develops into hemiatrophy the differential diagnosis is easy because of the abnormal neuronal proliferation and migration in hemimegalencephaly.
Encephalocraniocutaneous lipomatosis (ECCL) is a neurocutaneous syndrome that comprises unilateral porencephalic cysts with cortical atrophy.
Cerebral hemispheric asymmetry may be also found in septo-optic dysplasia and with ventral porencephaly (Stewart et al. 1978).
small right hemisphere; ventricle border lined with heterotopia
perinatal < iVH
term infant, seizures on day 2; extensive heterotopia in a large peri-insular zone; ventriculomegaly on the atrophic side
courtesy J Arnaes, Burgos
tissue loss——>
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cerebral hemiatrophy: characteristic findings
ventral porus ---------——>
ventral porencephaly and hemiatrophy
<———— ventral porus
term infant, left micropthalmia and left choanal atresia; left ventral porencephaly and cerebral hemiatrophy; no angiographic exams (compare with Stewart et al. 1978)
left cerebral hemiatrophy
<——————— ventral porus
cerebral hemidestruction following IVH at term
The hypothetical existence of spasm
Prolonged arterial spasm wit subsequent infarction, has been linked to:
- spasm in the vicinity of subdural or subarachnoid bleeding associated with arterial stroke has been tentatively reported (hypothetical because never angiographically documented in the newborn) (Govaert et al. 1992, Steinbok et al 1995, Jan and Camfield 1998, Koelfen et al. 1995, Kourtopoulos et al. 2000, Mazumdar et al. 2003, Talvik et al. 2010).
- spasm due to cocaine or other vasoconstrictor (amphetamines, blue cohosh [Chan and Nelson 2004, Finkel and Zarlengo 2004), codein (Reynolds et al. 2007)]
- spasm in a brain artery by blunt skull trauma (Erbayraktar et al. 2001, older child), not yet documented in the newborn.
(female, outborn, term): spontaneous vaginal delivery, apnoea on day 2, focal right clonic seizures; insular arteries with flow on affected side and day 3 MRI with patency of ipsilateral carotid artery and MCA (no proof of macrovascular occlusion); extensive destruction of the left cerebrum around an extensive IVH; hypothetically this can be due to spasm in the arteries around the bleeding, but this remains unsubstantiated
cerebral hemiatrophy literature examples
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