Table 2.
Fifty-two Individuals with ECDS and PRS Reported in the Literature
| Reference | Cutaneous involvement |
Neurologic involvement |
Neuroimaging findings† |
|---|---|---|---|
| Asher 1982 (27) |
Right PRS | Migraine headaches Sensory defects Nerve palsies Abnormal deep tendon reflexes Vision loss |
CT: normal MRI: N/A |
| Asher 1982(27) |
Left PRS | None | CT: contralateral parietal non-enhancing area of increased density MRI: N/A |
| David 1990(28) |
Left ECDS | Headaches Seizures |
CT: ipsilateral retrobulbar soft tissue mass with proptosis; ipsilateralparieto-occipital calcification with enhancement MRI: N/A |
| Fry 1992(29) | Right PRS | Hemiparesis | CT: normal MRI: N/A |
| Fry 1992(29) | Right PRS | Headaches | CT: ipsilateral frontal calcification MRI: ipsilateral frontal T2 HI |
| Fry 1992(29) | Right PRS | Behavioral disorder Seizures |
CT: ipsilateral frontal calcification MRI: ipsilateral frontal T2 HI |
| Fry 1992(29) | Right PRS | None | CT: normal MRI: ipsilateral frontal T2 HI |
| Fry 1992(29) | Left PRS | None | CT: ipsilateral frontal calcification MRI: ipsilateral hemispheric multifocal T2 HI |
| Fry 1992(29) | Left PRS | None | CT: N/A MRI: normal |
| Liu 1994(30) | Left ECDS | None | CT: ipsilateral frontal calcification MRI: ipsilateral frontal neuromigrational abnormality; ipsilateral frontal calcification and T2 HI |
| Liu 1994(30) | Left ECDS | None | CT: N/A MRI: normal |
| Liu 1994(30) | Left PRS | N/A | CT: normal MRI: normal |
| Liu 1994(30) | N/A | None | CT: ipsilateral frontal calcification MRI: ipsilateral frontal neuromigrational abnormality; ipsilateral frontal calcification and T2 HI |
| Liu 1994(30) | N/A | None | CT: ipsilateral parietal hypoattenuating lesion MRI: N/A |
| Liu 1994(30) | N/A | None | CT: N/A MRI: normal |
| Cory 1995(31) | Left PRS | Seizures | CT: ipsilateral cingulate gyrus calcification; ipsilateral frontal and parietal subcortical white matter flow densities MRI: ipsilateral cingulate gyrus T1 and T2 calcification; ipsilateral corpus callosal genu, subcortical white matter of cingulate gyrus, deep frontal white matter, frontoparietal subcortical white matter T2 HI; encephalomalacia; ipsilateral frontoparietal pial enhancement |
| Derex 1995(32) |
Left PRS | Seizures Mental retardation |
CT: ipsilateral parietal porencephalic cyst; left frontal and parietal calcification MRI: ipsilateral parietal porencephalic cyst |
| Schievink 1996(33) |
Left PRS | Vision loss Nerve palsies Sensory defects |
CT: ipsilateral cerebral atrophy; ipsilateral basal ganglia, lateral ventricle, and parietal calcifications; contralateral cavernous sinus aneurysm MRI: N/A |
| Goldberg- Stern 1997(19) |
Left PRS | Abnormal deep tendon reflex Seizures |
CT: ipsilateral frontal densities MRI: ipsilateral frontal T2 HI; ipsilateral frontoparietal gyral or sulcal enhancement |
| Menni 1997(34) |
Right ECDS and PRS |
Hemiparesis | CT: normal MRI: ipsilateral hemispheric T2 HI |
| Taylor 1997(35) |
Right ECDS and PRS |
Seizures Vision loss |
CT: ipsilateral temporal calcification MRI: ipsilateral temporal mixed signal abnormality |
| Higashi 2000(36) |
Left ECDS | Headaches Seizures Dizziness |
CT: N/A MRI: ipsilateral frontal T2 HI |
| Yano 2000(37) | Left ECDS and PR |
Seizures | CT: ipsilateral frontal and parietal calcifications; ipsilateral white matter hypodensity MRI: ipsilateral frontal and parietal T2 HI |
| Aynaci 2001(38) |
Left PRS | Adie’s pupil | CT: ipsilateral parietal calcification MRI: ipsilateral parietal subcortical T2 HI MRA: normal |
| Moko 2003(39) |
Left PRS | Migraine headaches | CT: N/A MRI: ipsilateral cerebral T2 HI |
| Moko 2003(39) | Left PRS | None | CT: N/A MRI: normal |
| Shah 2003(13) | Right PRS | Migraine headaches Seizures Hemiparesis Abnormal deep tendon reflexes |
CT: N/A MRI: ipsilateral frontoparietal atrophy; ipsilateral frontal T2 HI MRA: normal |
| Appenzeller 2004(3) |
Right ECDS | None | CT: normal MRI: abnormal gyral pattern; blurring of gray-white matter |
| Appenzeller 2004(3) |
Right ECDS | None | CT: normal MRI: abnormal gyral pattern; blurring of gray-white matter; calcification |
| Finley 2004(40) |
Right PRS | None | CT: N/A MRI: ipsilateral orbital atrophy MRA: normal |
| Hulzebos 2004(41) |
Right PRS | None | CT: N/A MRI: ipsilateral orbital atrophy |
| Sandhu 2004(42) |
Left PRS | Seizures | CT: N/A MRI: ipsilateral frontal subdural hygroma |
| Sathornsumete e 2005(43) |
Right ECDS and PRS |
Seizures Dysarthria Dysphagia Nerve palsies Abnormal deep tendon reflexes |
CT: N/A MRI: ipsilateral cerebral atrophy; hippocampal asymmetry; ipsilateral subdural fluid collection; ipsilateral frontal T2 HI |
| Holl-Weiden 2006(16) |
Left ECDS | Headaches | CT: N/A MRI: ipsilateral frontoparietal and occipital T2 HI |
| Okumura 2006(44) |
Left PRS | None | CT: ipsilateral cerebral and contralateral parieto-occipital hypoattenuating lesions MRI: ipsilateral cerebral and contralateral parieto-occipital T2 HI |
| Paprocka 2006(15) |
Left PRS | Seizures Hemiparesis Mental retardation |
CT: N/A MRI: ipsilateral frontoparietal atrophy; ipsilateral parietal T2 hyperintensity; contralateral occipital T2 hyperintensity MRA: normal MRS: decreased NAA and creatine; presence of lipid and lactate (ipsilateral> contralateral) SPECT: ipsilateral frontoparietal decreased radiopharmaceutical uptake |
| Sommer 2006(1) |
Right PRS | Seizures Migraine headaches |
CT: N/A MRI: normal |
| Sommer 2006(1) |
Right PRS | None | CT: N/A MRI: normal |
| Sommer 2006(1) |
Left ECDS and PRS |
Seizures Migraine headaches |
CT: N/A MRI: normal |
| Sommer 2006(1) |
Right PRS | None | CT: N/A MRI: normal |
| Sommer 2006(1) |
Right PRS | Developmental delay | CT: N/A MRI: normal |
| Carreno 2007(11) |
Left PRS | Seizures | CT: N/A MRI: ipsilateral cerebral atrophy |
| Verhels 2008(45) |
Right ECDS and PRS |
Seizures Developmental regression |
CT: ipsilateral cortical and subcortical calcifications MRI: ipsilateral hippocampal atrophy |
| Chiang 2009(46) |
Right ECDS | Seizures | CT: normal MRI: ipsilateral cerebral atrophy; ipsilateral temporo-occipital T2 HI |
| Menascu 2009(21) |
Left ECDS and PRS |
Migraine headaches Abnormal deep tendon reflexes Fine motor impairment |
CT: N/A MRI: ipsilateral fronto-parietal and temporal T2 HI MRA: normal MRS: normal |
| Sartori 2009(47) |
Left ECDS and PRS |
Seizures Behavioral disorder |
CT: ipsilateral basifrontal and temporal calcification MRI: ipsilateral basifrontal cortical and subcortical T2 and FLAIR HI |
| Qureshi 2010(48) |
Left PRS | Vision loss Seizures Hemiparesis |
CT: narrowing of ipsilateral carotid canal MRI: ipsilateral cerebral atrophy; ipsilateral orbital atrophy MRA: diffuse narrowing of ipsilateral ICA and PCA |
| Takenouchi 2010(49) |
Right PRS | Hemiparesis Migraine headaches Movement disorder |
CT: N/A MRI: ipsilateral cerebral and thalamic atrophy and T2 and FLAIR HI MRA: normal |
| Fain 2011(18) | Right ECDS and PRS |
Seizures Headaches Hemiparesis Sensory defects |
CT: N/A MRI: ipsilateral sulci effacement; ipsilateral cerebral T2 and FLAIR HI; ipsilateral punctate hypointensities (may represent micro hemorrhage or calcification, possibly a cavernous malformation) |
| Fain 2011(18) | Right ECDS | Seizures Sensory defects |
CT: ipsilateral parietal high density lesion MRI: ipsilateral parietal GRE hypointensity suggestive of blood products or calcification, possibly a cavernous malformation |
| Longo 2011(20) |
Left PRS | Seizures | CT: N/A MRI: ipsilateral T2 HI and T1 hypointensity |
HI = hyperintensity/hyperintensities
†
Soft tissue and calvarial changes were not uniformly reported by all authors and are not included in this table.