ALS and DEmntia

• EMENTIA

ALS
Amyotrophiclateral sclerosis
( Lou Gehrig's)

• Anterior horn cell diseases

• Many motor nerve diseases principally affect the anterior horn cell body; these are usually neurodegenerative (e.g. motor neurone disease) or hereditary (e.g. spinal muscular atrophy) or infection like
• polio.

(MND) Motor neuron disease

Motor neuron disease eg.(amyotrophic lateral sclerosis) is a progressive neuronal degenerative disease that leads to severe disability and death begins between the ages of 30 and 60 years , It is characterized by degeneration of anterior horn cells in the spinal cord, motor nuclei of the lower cranial nerves in the brainstem, and corticospinal and corticobulbar pathways. So there is features of combination of upper motor and lower motor type (characterized clinically by wasting , weakness and fasciculation of the affected muscles with hypereflexia . without accompanying sensory,cerebellar ,sphenictor or ocular muscles involvement s .

Investigation:

EMG , TFT, Cervicomedullary MRI.

Treatment:

Supportive measures no treatment till now.






• Dementia
It is defined as an acquired deterioration in cognitive abilities that impairs the successful performance of activities of daily living. Memory is the most common cognitive ability lost
with dementia.

Causes of Dementia

The classification of dementing illnesses into reversible and irreversible disorders is a useful approach to differential diagnosis.
Reversible Causes
Hypothyroidism
Thiamine deficiency
Vitamin B12 deficiency
Subdural hematoma

Chronic infection

Brain tumor

Irreversible/DegenerativeDementias

Alzheimer's
Huntington's
Vascular dementia

Alzheimer's Disease

Approximately 10% of all persons over the age of 70 have significant memory loss, and in more than half the cause is AD.
Clinical Manifestations
The most common complaint, often made by a career, spouse or other family member rather than by the patient, is of problems with memory. Patients become repetitive in questioning, forgetting that they asked the same question recently, the patient is unable to work, is easily lost and confused, Language becomes impaired—first naming, then comprehension, and finally fluency. In the late stages of the disease, some persons remain ambulatory but wander aimlessly. Loss of judgment and reasoning is inevitable.

Vascular Dementia

Dementia associated with cerebrovascular disease ,Cerebrovascular disease appears to be a more common cause of dementia, due to the increased prevalence of intracranial atherosclerosis. Individuals who have had several strokes may develop chronic cognitive deficits, commonly called multi-infarct dementia. The strokes may be large or small (sometimes lacunar) and usually involve several different brain regions.

.

Treatment of AD

The management of AD is challenging and gratifying, despite the absence of a cure or a robust pharmacologic treatment. The primary focus is on long-term amelioration of associated behavioral and neurologic problems, as well as providing caregiver support.
Building rapport with the patient, family members, and other caregivers is essential to successful management. In the early stages of AD, memory aids such as notebooks and posted daily reminders can be helpful.


Pharmacological
Cholinesterase inhibitors
The cholinesterase inhibitors (AChEIs) have been specifically developed as symptomatic treatment for AD.

There are currently three AChEIs licensed for the treatment of mild to moderate AD:

donepezil (Aricept)
galantamine (Reminyl)
rivastigmine (Exelon).

Memantine

potentially preventing glutamate-mediated neurotoxicity. It is a N-methyl-d-aspartate (NMDA)-receptor antagonist.