**7. Motor neuron disease presenting to non neurological specialists**

**Respiratory:** Progressive respiratory Failure

**Ear, nose, and throat:** Dysphagia.

**Orthopaedics/ neurosurgery:** Foot drop or other symptoms suggestive of compressive radiculopathy


**Elderly care:** Difficulty walking. (Talbot 2002).

(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

**Orthopaedics/ neurosurgery:** Foot drop or other symptoms suggestive of compressive

**ALS PMA PLS PBP** 

60 – 70 % 10 % 1 % 20%

Present Absent Present Absent

Limb muscles absent

3:2 3:4 3:1

Present

1/1000,000- Brugman and wokkle 2004

10-20/1000,000- Brugman and wokkle 2004

(Talbot 2002)

(Pseudo bulbar)

Usually absent (Raaphorst et al 2010)



1:1

Bulbar, Speech and limb

Present in bulbar muscles

Absent

**7. Motor neuron disease presenting to non neurological specialists** 

1. Age of onset 50-70 years 40 – 60 years

1,2-1,8/100,000 -----

5-9/100,000 -----

localization Cortical & spinal spinal cord Limb, Bulbar

Present Present in

Present and some group of ALS without cognitive impairment

causing respiratory failure.

radiculopathy

2. Percent of Frequency (Leigh et al 2003)

3. Incidence (Strong and Rosenfeld, 2003)

4. Prevalence (van der, Graaff, 2004)

5. Male to female Ratio ( Leigh et

al 2003)

6. Anatomical

7. Upper Motor Neuron manifestations

8. Lower Motor Neuron manifestation

9. Cognitive impairment

**Respiratory:** Progressive respiratory Failure

**Elderly care:** Difficulty walking. (Talbot 2002).

**Ear, nose, and throat:** Dysphagia.


Table 2. Clinical Subtypes of Motor Neuron Disease
