| Demographic Data |
| Relation to Proband |
No Data |
| Age at Sampling |
58 YR |
| Sex |
Female |
| Age of Onset(If not a control) |
46 YR |
| Age at Diagnosis(If not a control) |
50 YR |
| Hispanic or Latino/Not Hispanic or Latino |
Not Hispanic/Latino |
| Racial Category |
White |
| Country |
USA |
| Diagnosed By |
No Data |
| |
| Data Elements |
| Clinical Element Type: Motor Neuron Disorders |
| (Baseline) |
| Longitudinal Data |
| Is this data Longitudinal (Follow-Up) Data? |
yes no |
| Family History |
| ALS/other MND |
present absent unknown |
| Parkinson's disease |
present absent unknown |
| Alzheimer's disease |
present absent unknown |
| Other dementia |
present absent unknown |
| Other neurodegenerative disease |
present absent unknown |
| Medical History |
| Alzheimer's disease |
present absent |
| Ataxia |
present absent |
| Autism |
present absent |
| Bipolar (manic-depressive) |
present absent |
| Brain aneurysm |
present absent |
| Cancer |
present absent |
| Dementia |
Alzheimer's
Dementia
absent
|
| Depression |
present absent |
| Diabetes |
present absent |
| Dystonia |
present absent |
| Epilepsy |
present absent |
| Heart disease |
present absent |
| Hypertension |
present absent |
| Multiple sclerosis |
present absent |
| Muscle disease |
present absent |
| Parkinson's |
present absent |
| Schizophrenia |
present absent |
| Suicide/Attempt |
present absent |
| Stroke |
present absent |
| Primary Clinical Diagnosis |
| Primary clinical diagnosis |
Primary Lateral Sclerosis |
| Secondary Neurological Diagnosis |
| Secondary neurological diagnoses |
Frontotemporal dementia
Other (specify)
Not Applicable
|
| Site of Symptom Onset |
| site of symptom onset |
Limb-lower |
| Treatment |
| Current treatment |
Riluzole
PEG
NIPPV
Tracheotomy
Assisted Ventilation > 23 hours
Other (specify)
No Treatment
Notes: GABAPENTIN, FLUOXETINE, CO-Q 10, BACLOFEN, ZANAFLEX, VITAMIN C, VITAMIN E, VITAMIN B COMPLEX, BETA-CAROTENE |
| Signs Supporting Diagnosis |
| Upper Motor Neuron Signs-Bulbar |
definite indeterminate absent not tested |
| Upper Motor Neuron Signs-Cervical/upper limbs |
definite indeterminate absent not tested |
| Upper Motor Neuron Signs-Thoracic/chest |
definite indeterminate absent not tested |
| Upper Motor Neuron Signs-Lumbosacral/lower limbs |
definite indeterminate absent not tested |
| Lower Motor Neuron Signs-Bulbar |
definite indeterminate absent not tested |
| Lower Motor Neuron Signs-Cervical/upper limbs |
definite indeterminate absent not tested |
| Lower Motor Neuron Signs-Thoracic/chest |
definite indeterminate absent not tested |
| Lower Motor Neuron Signs-Lumbosacral/lower limbs |
definite indeterminate absent not tested |
| EMG Studies |
| Bulbar |
acute denervation chronic denervation negative not examined acute/chronic denervation |
| Cervical/upper limbs |
acute denervation chronic denervation negative not examined acute/chronic denervation |
| Thoracic/chest |
acute denervation chronic denervation negative not examined acute/chronic denervation |
| Lumbosacral/lower limbs |
acute denervation chronic denervation negative not examined acute/chronic denervation |
| Genetics |
| SOD-1 mutation |
No Data
Notes: NOT TESTED |
| Other mutation |
No Data
Notes: NOT TESTED |
| Atypical Features of ALS/MND |
| Atypical features of ALS/MND |
sensory
autonomic
cerebellar
cognitive
Parkinsonian
sphincter
ocular
other
|
| Optional data |
| Current ALSFRS-R |
36 |
| FVC |
69 |
| smoking history |
never former smoker current smoker |
| years smoking |
20 YEARS, QUIT IN 1985 |
| Handedness |
Right Left Ambidextrous |
| Clinical Element Type: Motor Neuron Disorders |
| (Longitudinal, Series #2) |
| Longitudinal Data |
| Is this data Longitudinal (Follow-Up) Data? |
yes no |
| Family History |
| ALS/other MND |
present absent unknown |
| Parkinson's disease |
present absent unknown |
| Alzheimer's disease |
present absent unknown |
| Other dementia |
present absent unknown |
| Other neurodegenerative disease |
present absent unknown |
| Medical History |
| Alzheimer's disease |
present absent |
| Ataxia |
present absent |
| Autism |
present absent |
| Bipolar (manic-depressive) |
present absent |
| Brain aneurysm |
present absent |
| Cancer |
present absent |
| Dementia |
Alzheimer's
Dementia
absent
|
| Depression |
present absent |
| Diabetes |
present absent |
| Dystonia |
present absent |
| Epilepsy |
present absent |
| Heart disease |
present absent |
| Hypertension |
present absent |
| Multiple sclerosis |
present absent |
| Muscle disease |
present absent |
| Parkinson's |
present absent |
| Schizophrenia |
present absent |
| Suicide/Attempt |
present absent |
| Stroke |
present absent |
| Primary Clinical Diagnosis |
| Primary clinical diagnosis |
Primary Lateral Sclerosis |
| Secondary Neurological Diagnosis |
| Secondary neurological diagnoses |
Frontotemporal dementia
Other (specify)
Not Applicable
|
| Site of Symptom Onset |
| site of symptom onset |
Limb-lower |
| Treatment |
| Current treatment |
Riluzole
PEG
NIPPV
Tracheotomy
Assisted Ventilation > 23 hours
Other (specify)
No Treatment
Notes: GABAPENTIN, FLUOXETINE, CO-Q 10, BACLOFEN, ZANAFLEX, VITAMIN C, VITAMIN E, VITAMIN B COMPLEX, BETA-CAROTENE |
| Signs Supporting Diagnosis |
| Upper Motor Neuron Signs-Bulbar |
definite indeterminate absent not tested |
| Upper Motor Neuron Signs-Cervical/upper limbs |
definite indeterminate absent not tested |
| Upper Motor Neuron Signs-Thoracic/chest |
definite indeterminate absent not tested |
| Upper Motor Neuron Signs-Lumbosacral/lower limbs |
definite indeterminate absent not tested |
| Lower Motor Neuron Signs-Bulbar |
definite indeterminate absent not tested |
| Lower Motor Neuron Signs-Cervical/upper limbs |
definite indeterminate absent not tested |
| Lower Motor Neuron Signs-Thoracic/chest |
definite indeterminate absent not tested |
| Lower Motor Neuron Signs-Lumbosacral/lower limbs |
definite indeterminate absent not tested |
| EMG Studies |
| Bulbar |
acute denervation chronic denervation negative not examined acute/chronic denervation |
| Cervical/upper limbs |
acute denervation chronic denervation negative not examined acute/chronic denervation |
| Thoracic/chest |
acute denervation chronic denervation negative not examined acute/chronic denervation |
| Lumbosacral/lower limbs |
acute denervation chronic denervation negative not examined acute/chronic denervation |
| Genetics |
| SOD-1 mutation |
No Data
Notes: NOT TESTED |
| Other mutation |
No Data
Notes: NOT TESTED |
| Atypical Features of ALS/MND |
| Atypical features of ALS/MND |
sensory
autonomic
cerebellar
cognitive
Parkinsonian
sphincter
ocular
other
|
| Optional data |
| Current ALSFRS-R |
36 ON 7/17/08 |
| FVC |
74 ON 7/17/08 |
| smoking history |
never former smoker current smoker |
| years smoking |
20 YEARS, QUIT IN 1985 |
| Handedness |
Right Left Ambidextrous |