Health Condition 1: null- All patients of monomelic amyotrophy (MMA) seen in Neurology department in the outpatient or admitted will be included in the study.
Conditions
Interventions
None listed
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: All patients of monomelic amyotrophy (MMA) seen in Neurology department in the outpatient or admitted will be included in the study. The diagnosis is based on the following criteria[6]: (i) age at onset >18 years; (ii) disease duration of >4 years from the time of onset of weakness; (ii) evidence of lower motor neuron (LMN) involvement on neurological examination (weakness, atrophy and fasciculations); and (iv) electrophysiological evidence of LMN involvement on needle EMG examination.
Exclusion criteria
Exclusion criteria: Any of the following: 1 familial history of lower motor neuron disease (LMND); 2 deletion in the SMN1 gene or an expansion of CAG-repeats ( >40) in the androgen receptor gene; 3 history of diseases that may mimic LMND (acute poliomyelitis, spinal radiculopathy, diabetic amyotrophy, thyrotoxicosis or hyperparathyroidism); 4 clinical signs of UMN involvement [pseudobulbar symptoms, brisk jaw jerk, hyperreflexia or extensor plantar response]; 5 objective sensory signs on neurological examination; 6 tracheostomy or intermittent ventilatory assistance; 7 structural lesions (tumors, intervertebral disk herniation, vascular lesions, syringomyelia) on MRI or myelography of the spinal cord; and 8 motor conduction block on extensive standardized nerve conduction studies
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The primary outcome studied will be the clinical progression, if any, measured by MRC (Medical Research Council) grading of power in the affected area, The Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS)Timepoint: The time points are 3 months, 6 months and 1 year | — |
Secondary
| Measure | Time frame |
|---|---|
| The Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS)Timepoint: 6 months and one year | — |
Countries
India
Contacts
All India Institute of Medical Sciences