| Id |
Subject |
Object |
Predicate |
Lexical cue |
| TextSentencer_T1 |
0-92 |
Sentence |
denotes |
COL4A1 mutations as a monogenic cause of cerebral small vessel disease: a systematic review. |
| TextSentencer_T2 |
93-116 |
Sentence |
denotes |
BACKGROUND AND PURPOSE: |
| TextSentencer_T3 |
117-191 |
Sentence |
denotes |
A number of single gene disorders can cause cerebral small vessel disease. |
| TextSentencer_T4 |
192-453 |
Sentence |
denotes |
Mutations in the COL4A1 gene encoding the type IV collagen alpha 1 chain, which are already associated with porencephaly and infantile hemiparesis, have been recently recognized as a further monogenic cause of small vessel disease that can present in adulthood. |
| TextSentencer_T5 |
454-462 |
Sentence |
denotes |
METHODS: |
| TextSentencer_T6 |
463-623 |
Sentence |
denotes |
We performed a systematic review of published data from 1966 to January 8, 2010 to characterize the features of small vessel disease seen with COL4A1 mutations. |
| TextSentencer_T7 |
624-632 |
Sentence |
denotes |
RESULTS: |
| TextSentencer_T8 |
633-679 |
Sentence |
denotes |
We identified a total of 52 mutation carriers. |
| TextSentencer_T9 |
680-850 |
Sentence |
denotes |
A history of stroke was reported in 9 subjects (17.3%); in 6 cases it was attributable to subcortical hemorrhage and in 3 cases it was attributable to lacunar infarction. |
| TextSentencer_T10 |
851-970 |
Sentence |
denotes |
Stroke often occurred as first presentation of the disease, with a mean age of onset of 36.1 (SD, 12.95; range, 14-49). |
| TextSentencer_T11 |
971-1082 |
Sentence |
denotes |
Hemorrhages, often recurrent, have been associated with physical trauma and activity and anticoagulant therapy. |
| TextSentencer_T12 |
1083-1254 |
Sentence |
denotes |
Brain imaging showed frequent leukoaraiosis (63.5%), microbleeds that are usually subcortical (52.9%), lacunar infarction (13.5%), and dilated perivascular spaces (19.2%). |
| TextSentencer_T13 |
1255-1340 |
Sentence |
denotes |
Extensive leukoaraiosis was seen in a number of asymptomatic adult mutation carriers. |
| TextSentencer_T14 |
1341-1420 |
Sentence |
denotes |
Asymptomatic intracranial aneurysms were common (44.4% of 18 with angiography). |
| TextSentencer_T15 |
1421-1516 |
Sentence |
denotes |
Migraine (with and without aura) was reported in 10 subjects, with a mean age at onset of 31.7. |
| TextSentencer_T16 |
1517-1623 |
Sentence |
denotes |
Systemic features are also frequent, affecting the eye (10/21, 47.6%), kidney (15.4%), and muscle (15.4%). |
| TextSentencer_T17 |
1624-1828 |
Sentence |
denotes |
CONCLUSIONS: COL4A1 is a further cause of familial vasculopathy and may present with stroke, ischemic as well as hemorrhagic, in adult life and with radiological features of leukoaraiosis and microbleeds. |
| T1 |
0-92 |
Sentence |
denotes |
COL4A1 mutations as a monogenic cause of cerebral small vessel disease: a systematic review. |
| T2 |
93-116 |
Sentence |
denotes |
BACKGROUND AND PURPOSE: |
| T3 |
117-191 |
Sentence |
denotes |
A number of single gene disorders can cause cerebral small vessel disease. |
| T4 |
192-453 |
Sentence |
denotes |
Mutations in the COL4A1 gene encoding the type IV collagen alpha 1 chain, which are already associated with porencephaly and infantile hemiparesis, have been recently recognized as a further monogenic cause of small vessel disease that can present in adulthood. |
| T5 |
454-462 |
Sentence |
denotes |
METHODS: |
| T6 |
463-623 |
Sentence |
denotes |
We performed a systematic review of published data from 1966 to January 8, 2010 to characterize the features of small vessel disease seen with COL4A1 mutations. |
| T7 |
624-632 |
Sentence |
denotes |
RESULTS: |
| T8 |
633-679 |
Sentence |
denotes |
We identified a total of 52 mutation carriers. |
| T9 |
680-850 |
Sentence |
denotes |
A history of stroke was reported in 9 subjects (17.3%); in 6 cases it was attributable to subcortical hemorrhage and in 3 cases it was attributable to lacunar infarction. |
| T10 |
851-970 |
Sentence |
denotes |
Stroke often occurred as first presentation of the disease, with a mean age of onset of 36.1 (SD, 12.95; range, 14-49). |
| T11 |
971-1082 |
Sentence |
denotes |
Hemorrhages, often recurrent, have been associated with physical trauma and activity and anticoagulant therapy. |
| T12 |
1083-1254 |
Sentence |
denotes |
Brain imaging showed frequent leukoaraiosis (63.5%), microbleeds that are usually subcortical (52.9%), lacunar infarction (13.5%), and dilated perivascular spaces (19.2%). |
| T13 |
1255-1340 |
Sentence |
denotes |
Extensive leukoaraiosis was seen in a number of asymptomatic adult mutation carriers. |
| T14 |
1341-1420 |
Sentence |
denotes |
Asymptomatic intracranial aneurysms were common (44.4% of 18 with angiography). |
| T15 |
1421-1516 |
Sentence |
denotes |
Migraine (with and without aura) was reported in 10 subjects, with a mean age at onset of 31.7. |
| T16 |
1517-1623 |
Sentence |
denotes |
Systemic features are also frequent, affecting the eye (10/21, 47.6%), kidney (15.4%), and muscle (15.4%). |
| T17 |
1624-1828 |
Sentence |
denotes |
CONCLUSIONS: COL4A1 is a further cause of familial vasculopathy and may present with stroke, ischemic as well as hemorrhagic, in adult life and with radiological features of leukoaraiosis and microbleeds. |