**6. Bad prognostic factors for MND**


The classical concept that MND only affects the motor system is obsolete. MND is considered to be a multisystem neurodegenerative disease. There is increasing clinical evidence for autonomic dysfunction (Baltadzhieva, et al 2006 and Takeda, et al, 1994), sensory abnormalities (Takeda et al, 1994) (Pugdahl et al, 2007) and ophthalmoplegia

Motor Neuron Disease 207

symptoms Present Absent Present Promiment

lability May be Present Present Prominent Absent

Positive- lower motor neuron manifestations

5 years, more long survivors (>10 years)

Cognitive impairment is increasingly being recognized in MND. Subtle subclinical cognitive defects and frontal lobe dysfunction may be demonstrated in up to half of MND patients with detailed neuropsychological testing (Ringholz, et al. 2005). Several genetic mutations of MND have been identified in association with frontotemporal dementia and/or parkinsonism (Valdmanis and Rouleau. 2008). It is well recognized that MND is a multisystem disorder (Geser, et al, 2008) with compromise of regions beyond the motor

Normal Normal Normal Normal

Normal ---- Normal ----



Posterior inferior cerebellar artery occlusion

Fibrillation and Positive sharp waves.

cerebrovascular

Normal

20 years and More 2-3years

diseases -cervical Spondylotic myelopathy



> motor neuron manifestations

> > 3-4 years

**8. Cognitive function impairment in motor neuron disease** 

Table 2. Clinical Subtypes of Motor Neuron Disease

10. Bulbar

11. Mimic syndromes

12. Emotional

b. MRI brain When Bulbar manifestations are isolated symptoms and

signs

14. Prognosis Median

c. MRI spinal cord All Limb onset without bulbar symptoms

> Survival (Leigh et al 2003)

13. Investigotions

a. EMG&CV Positive- lower

(Takeda, et al, 1994) in MND. On the other hand, there are good pathological accounts of the involvement of sympathetic and parasympathetic neurons, (Takeda, et al, 1994), Onuf's nucleus (which innervates the pelvic floor sphincteric muscles), (Takeda, et al, 1994) peripheral sensory nerves, (Isaacs, et al. 2007) and oculomotor nuclei. (Takeda, et al, 1994). However, in practical terms, the presence of prominent ophthalmoplegia, sensory signs or sphincter dysfunction should raise doubts regarding the diagnosis of MND, unless there are a clear alternative explanation. Death usually results from ventilatory muscle weakness causing respiratory failure.
