Transient Ischemic Attack and Ischemic Stroke in Danon Disease with Formation of Left Ventricular Apical Thrombus despite Normal Systolic Function
Danon disease is a rare X-linked dominant skeletal and cardiac muscle disorder presenting with hypertrophic cardiomyopathy, Wolf-Parkinson-White syndrome, skeletal myopathy, and mild intellectual disability. Early morbidity and mortality due to heart failure or sudden death are known in Danon diseas...
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| Format: | Article |
| Language: | English |
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Wiley
2017-01-01
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| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2017/6576382 |
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| author | Takeshi Tsuda Amanda J. Shillingford Jane Vetter Vinay Kandula Badal Jain Joel Temple |
| author_facet | Takeshi Tsuda Amanda J. Shillingford Jane Vetter Vinay Kandula Badal Jain Joel Temple |
| author_sort | Takeshi Tsuda |
| collection | DOAJ |
| description | Danon disease is a rare X-linked dominant skeletal and cardiac muscle disorder presenting with hypertrophic cardiomyopathy, Wolf-Parkinson-White syndrome, skeletal myopathy, and mild intellectual disability. Early morbidity and mortality due to heart failure or sudden death are known in Danon disease, more in males than in females. Here, we present a 17-year-old female adolescent with Danon disease and severe concentric hypertrophy with normal left ventricular (LV) systolic function, who has been complaining of intermittent headache and weakness for about 3 years, initially diagnosed with hemiplegic migraine. Subsequently, her neurological manifestation progressed to transient ischemic attack (TIA) and eventually to ischemic stroke confirmed by CT scan with 1-day history of expressive aphasia followed by persistent left side weakness and numbness. Detailed echocardiogram for the first time revealed a small LV apical thrombus with unchanged severe biventricular hypertrophy and normal systolic function. This unexpected LV apical thrombus may be associated with a wide spectrum of neurological deficits ranging from TIA to ischemic stroke in Danon disease. Possibility of cerebral ischemic events should be suspected in Danon disease when presenting with neurological deficits even with normal systolic function. Careful assessment for LV apical thrombus is warranted in such cases. |
| format | Article |
| id | doaj-art-92a48d093355412ea8c7d284370fbb13 |
| institution | Kabale University |
| issn | 2090-6803 2090-6811 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-92a48d093355412ea8c7d284370fbb132025-02-03T01:23:08ZengWileyCase Reports in Pediatrics2090-68032090-68112017-01-01201710.1155/2017/65763826576382Transient Ischemic Attack and Ischemic Stroke in Danon Disease with Formation of Left Ventricular Apical Thrombus despite Normal Systolic FunctionTakeshi Tsuda0Amanda J. Shillingford1Jane Vetter2Vinay Kandula3Badal Jain4Joel Temple5Nemours Cardiac Center, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USANemours Cardiac Center, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USANemours Cardiac Center, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USADepartment of Medical Imaging, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USADivision of Neurology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USANemours Cardiac Center, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USADanon disease is a rare X-linked dominant skeletal and cardiac muscle disorder presenting with hypertrophic cardiomyopathy, Wolf-Parkinson-White syndrome, skeletal myopathy, and mild intellectual disability. Early morbidity and mortality due to heart failure or sudden death are known in Danon disease, more in males than in females. Here, we present a 17-year-old female adolescent with Danon disease and severe concentric hypertrophy with normal left ventricular (LV) systolic function, who has been complaining of intermittent headache and weakness for about 3 years, initially diagnosed with hemiplegic migraine. Subsequently, her neurological manifestation progressed to transient ischemic attack (TIA) and eventually to ischemic stroke confirmed by CT scan with 1-day history of expressive aphasia followed by persistent left side weakness and numbness. Detailed echocardiogram for the first time revealed a small LV apical thrombus with unchanged severe biventricular hypertrophy and normal systolic function. This unexpected LV apical thrombus may be associated with a wide spectrum of neurological deficits ranging from TIA to ischemic stroke in Danon disease. Possibility of cerebral ischemic events should be suspected in Danon disease when presenting with neurological deficits even with normal systolic function. Careful assessment for LV apical thrombus is warranted in such cases.http://dx.doi.org/10.1155/2017/6576382 |
| spellingShingle | Takeshi Tsuda Amanda J. Shillingford Jane Vetter Vinay Kandula Badal Jain Joel Temple Transient Ischemic Attack and Ischemic Stroke in Danon Disease with Formation of Left Ventricular Apical Thrombus despite Normal Systolic Function Case Reports in Pediatrics |
| title | Transient Ischemic Attack and Ischemic Stroke in Danon Disease with Formation of Left Ventricular Apical Thrombus despite Normal Systolic Function |
| title_full | Transient Ischemic Attack and Ischemic Stroke in Danon Disease with Formation of Left Ventricular Apical Thrombus despite Normal Systolic Function |
| title_fullStr | Transient Ischemic Attack and Ischemic Stroke in Danon Disease with Formation of Left Ventricular Apical Thrombus despite Normal Systolic Function |
| title_full_unstemmed | Transient Ischemic Attack and Ischemic Stroke in Danon Disease with Formation of Left Ventricular Apical Thrombus despite Normal Systolic Function |
| title_short | Transient Ischemic Attack and Ischemic Stroke in Danon Disease with Formation of Left Ventricular Apical Thrombus despite Normal Systolic Function |
| title_sort | transient ischemic attack and ischemic stroke in danon disease with formation of left ventricular apical thrombus despite normal systolic function |
| url | http://dx.doi.org/10.1155/2017/6576382 |
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