| Journal of Neurology Research, ISSN 1923-2845 print, 1923-2853 online, Open Access |
| Article copyright, the authors; Journal compilation copyright, J Neurol Res and Elmer Press Inc |
| Journal website http://www.neurores.org |
Case Report
Volume 1, Number 4, October 2011, pages 161-164
Mutant ApoA-1 Amyloidosis in a Family of Five Siblings With Motor Neuron Disease and Dementia
Table
| Patient | Men/Women | Born/died (year) | Clinical symptoms of MND | Duration of MND (months) | Clinical symptoms of dementia | EENG/EMG | Computed tomography of the brain | Cardiovascular events |
|---|---|---|---|---|---|---|---|---|
| MND: Motor neuron disease; EENG: Electroneurography; EMG: Electromyography; ADL: Activity of daily living; ND: Not done. | ||||||||
| HL | M | 1919/1982 | Fasciculations, atrophy, dysarthria. Normal reflexes and absent Babinski sign. | 19 | Personality change, poor hygiene. | Widespread denervation, low motor amplitudes | Cortical and central atrophy | Claudicatio intermittens |
| GL | M | 1923/1993 | Fasciculations, atrophy, increased reflexes and positive Babinski sign | 24 | Difficulties in ADL, poor memory | Widespread denervation, low motor amplitudes | Cortical and central atrophy | Abdominal aortic aneurysm |
| RL | M | 1923/1983 | Fasciculations, atrophy, decreased reflexes and absent Babinski sign. | 20 | No | Widespread denervation, low motor amplitudes | Normal | - |
| SL | M | 1926/1994 | No | - | Poor memory, concentration difficulties | ND | Cortical atrophy, Pons infarction | Operated abdominal aortic aneurysm. Died in myocardial infarction 1994 |
| UG | W | 1930/1997 | No | - | No | ND | Left-sided cerebral infarction | Mitral valve prothesis. Atrial fibrillation. Died in heart insufficiency and cardiac arrest in 1997 |