El Paso, TX. Chiropractor, Dr. Alexander Jimenez focuses on degenerative and demyelinating diseases of the nervous system, their symptoms, causes and treatment.
Degenerative & Demyelinating Diseases
Motor Neuron Diseases
- Motor weakness without sensory changes
- Amyotrophic lateral sclerosis (ALS)
- ALS Variants
- Primary lateral sclerosis
- Progressive bulbar palsy
- Inherited conditions that cause anterior horn cell degeneration
- Werdnig-Hoffmann disease in infants
- Kugelberg-Welander disease in children and young adults
Amyotrophic Lateral Sclerosis (ALS)
- Affects patients 40-60 years of age
- Damage to:
- Anterior horn cells
- Cranial nerve motor nuclei
- Corticobulbar and corticospinal tracts
- Lower motor neuron findings (atrophy, fasciculations) AND upper motor neuron findings (spasticity, hyperreflexia)
- Survival ~three years
- Death results from weakness of the bulbar and respiratory musculature and resultant superimposed infection
ALS Variants
- Usually eventually evolve into typical ALS pattern
- Primary Lateral Sclerosis
- Upper motor neuron signs begin first, but patients do eventually have lower motor neuron signs as well
- Survival can be ten years or longer
- Progressive Bulbar Palsy
- Selectively involves the head and neck musculature
Inherited Motor Neuron Conditions
- Anterior horn cell degeneration
- Werdnig-Hoffmann disease
- Affects infants
- Kugelberg-Welander disease
- Affects children and young adults
Church, Archibald. Nervous and Mental Diseases. W.B. Saunders Co., 1923.
Alzheimer Disease
- Characterized by neurofibrillary tangles (aggregates of hyperphosphorylated tau protein) & beta-amyloid plaques
- Generally occurring after age 65
- Hereditary risk factors
- Mutations in the beta amyloid gene
- Epsilon 4 version of apolipoprotein
Diagnosis
- Pathologic diagnosis is the only way to definitively diagnose the condition
- Imaging may be able to rule out other causes of dementia
- Functional imaging studies may be further developed to become diagnostically useful in the future
- CSF studies examining for tau proteins and beta amyloid may become useful as diagnostic tests in the future
Amyloid Plaques & Neurofibrillary Tangles
http://sage.buckinstitute.org/wp-content/uploads/2015/01/plaque-tanglesRNO.jpg
Brain Areas Affected by Alzheimer Disease
-
Hippocampus
- Loss of recent memory
-
Posterior temporo-parietal association area
- Mild anomia & constructional apraxia
-
Nucleus basalis of Meynert (cholinergic neurons)
- Changes in visual perception
Progression
- As more and more cortical areas become involved, the patient will develop more severe cognitive deficits, however paresis, sensory loss, or visual field defects are features.
Treatment Options
-
Medications that inhibit central nervous system acetylcholinesterase
- Donepezil
- Galantamine
- Rivastigmine
-
Aerobic Exercise, 30 minutes daily
-
PT/OT care to maintain activities of daily living
- Antioxidant and anti-inflammatory therapies
- In advanced stages, may require full time, in home care
Vascular Dementia
- Cerebral arteriosclerosis leading to stroke
- Patient will have documented stroke history or signs of prior stroke (spasticity, paresis, pseudobulbar palsies, aphasia)
- May be associated with Alzheimer Disease if due to amyloid angiopathy
Frontotemporal Dementia (Pick’s Disease)
-
Familial
-
Affects the frontal and temporal lobes
- May be seen on imaging if advanced degeneration in these areas
-
Symptoms
- Apathy
-
Disordered behavior
- Agitation
- Socially inappropriate behavior
- Impulsivity
-
Language difficulties
-
Generally no memory or spatial difficulties
-
Pathology reveals Pick bodies within the neurons
-
Results in death in 2-10 years
Pick Bodies/Cytoplasmic Inclusions
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Treatment
-
Antidepressants
- Sertraline
- Citalopram
-
Discontinue medications that can cause memory impairment or confusion
- Sedatives
- Benzodiazepines
-
Exercise
- Lifestyle modification
- Behavioral modification therapy
Parkinson Disease
-
May occur at any age, but rare before age 30, and increases prevalence increases in older populations
-
Familial tendency but can also without family history
-
Can be induced by certain environmental factors
- Exposure 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)
- Compounds which produce excessive free radicals
-
Affects substantia nigra pars compacta
- Dopaminergic neurons
-
On pathology, the presence of Lewy Bodies
- Accumulation of alpha-synuclein
Lewy Bodies
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Symptoms of Parkinsonism
-
Rigidity (all planes)
- Passive ROM
- Active movement
- May be of cogwheel nature due to tremor symptoms
-
Bradykinesia
- Slowness of movement
- Inability to initiate movement
- Freezing
-
Resting tremor (“pill-rolling”)
- Created by oscillation of opposing muscle groups
-
Postural defects
- Anteriorly flexed (stooped) posture
- Inability to compensate for perturbations, resulting in retropulsion
-
Mask-like facies
-
Mild to moderate dementia
- Later in progression, due to lewy body accumulation
Pathology
-
Deficiency of dopamine in the striatum (caudate and putamen) of the basal ganglia
-
Dopamine normally has the effect of stimulating the direct circuit through the basal ganglia, while inhibiting the indirect pathway
Carbidopa/Levodopa
-
Most common treatment is a combination drug
-
Levodopa
- A dopamine precursor that crosses the blood-brain barrier
-
Carbidopa
- Dopamine decarboxylase inhibitor that does not cross the BBB
- Amino acids will reduce effectiveness (competition) and so medication should be taken away from protein
Prolonged Treatment With Carbidopa/Levodopa
-
The patient’s capacity to store dopamine declines with medication use and therefore the improvements from the medications will last for shorter and shorter periods the longer the medication is used
-
Over time can result in proliferation of dopamine receptors
- Peak-dose dyskinesia
-
Long term use puts stress on the liver
-
Other side effects can include nausea, hypotension and hallucinations
Other Treatment Options
-
Medications
- Anticholinergics
- Dopamine agonists
- Dopanime breakdown inhibitors (Monoamine oxidase or catechol-O-methyl transferase inhibitors)
- High dose glutathione
-
Brain balancing functional neuro-rehab exercises
- Vibration
- Retropulsive stimulation
- Repeated reflex stimulation
- Targeted CMT/OMT
Multiple System Atrophy
-
Symptoms of Parkinson Disease paired with one or more of the following:
- Pyramidal signs (Striatonigral degeneration)
- Autonomic dysfunction (ShyDrager syndrome)
- Cerebellar finding (Olivopontocerebellar atrophy)
-
Generally not responsive to standard Parkinson Disease treatments
Progressive Supranuclear Palsy
-
Fast progressing degeneration involving tau proteins in many areas including the rostral midbrain
-
Symptoms usually start around ages 50-60
-
Gait difficulty
-
Significant dysarthria
-
Voluntary vertical gaze difficulty
-
Retrocollis (dystonic extension of the neck)
-
Severe dysphagia
-
Emotional lability
-
Personality changes
-
Cognitive difficulty
-
Does not respond well to standard PD treatment
Diffuse Lewy Body Disease
- Progressive dementia
- Severe hallucinations and possible paranoid delusions
- Confusion
- Parkinsonian symptoms
Multiple Sclerosis
-
Multiple white matter lesions (plaques of demyelination) in the CNS
- Variable in size
- Well-circumscribed
- Visible on MRI
-
Optic nerve lesions are common
-
Peripheral nerves are not involved
-
Uncommon in children under 10, but usually presents before age 55
-
Viral infection may trigger an inappropriate immune response with antibodies to a common virus-myelin antigen
-
Infectious and immune mechanisms contribute
Types Of MS
-
Primary progressive MS (PPMS)
-
Secondary progressive MS (SPMS)
-
Relapsingremitting multiple sclerasis (RRMS)
- Most common type
- Can develop acutely, spontaneous appear to resolve and return
- Eventually becomes like SPMS
Optic Nerve Involvement
-
In 40% of MS cases
-
Pain with eye movements
-
Visual field defect (central or paracentral scotoma)
-
Funduscopic examination
- May reveal papilledema if the plaque involves the optic disk
- May not appear unusual if plaques are behind the optic disk (retrobulbar neuritis)
Medial Longitudinal Fasciculus Involvement
-
Demyelination of the MLF results in internuclear ophthalmoplegia
- During lateral gaze there is paresis of the medial rectus and nystagmus of the contralateral eye
-
Convergence remains normal
Other Possible MS Symptoms
-
Myelopathy
- Spastic hemiparesis
- Impaired sensory tracts (DC-ML)
- Paresthesias
-
Cerebellar involvement
- Ataxia
- Dysarthria
-
Vestibular system involvement
- Imbalance
- Mild vertigo
- Nystagmus
-
Tic douloureux (trigeminal neuralgia)
-
Lhermitte’s symptom
- Shooting or tingling sensation referred to the trunk and limbs during neck flexion
- Fatigue
- Hot bath often exacerbates symptoms
Differentials To Consider
-
Multiple emboli and vasculitis
- May appear as white matter damage on MRI
-
Central nervous system sarcoidosis
- Can produce reversible optic neuritis and other CNS signs
-
Whipple disease
- Inflammatory lesions
- Usual eye movements
-
Vitamin B12 deficiency
- Dementia
- Spasticity
- Dorsal column
-
Meningovascular syphilis
- Multifocal CNS damage
-
CNS Lyme disease
- Multifocal disease
Differential Diagnosis: Diagnostic Studies
-
Blood tests can help to distinguish
- Complete blood count
- Antinuclear antibodies (ANA)
- Serum test for syphilis (RPR, VDRL, etc.)
- Fluorescent treponemal antibody test
- Lyme titer
- ESR
- Angiotensin converting enzyme level (to r/o sarcoidosis)
Diagnostic Studies Of MS
-
MRI with and without contrast
- 90% of MS cases have detectable MRI findings
-
CSF findings
- Elevation of mononuclear white blood cells
- Oligoclonal IgG bands
-
Increased globulin to albumin ratio
- This is also seen in 90% of MS cases
-
Increased myelin basic protein levels
Prognosis
- Average survival after diagnosis is ~ 15 to 20 year
- Death is usually from superimposed infection and not due to the effects of the disease itself
Sources
Alexander G. Reeves, A. & Swenson, R. Disorders of the Nervous System. Dartmouth, 2004.
Swenson, R. Degenerative Diseases of the Nervous System. 2010.