Discussion w7-8 652

Q-1

Parkinson’s disease (PD) is a progressive, chronic, and incurable neurodegenerative disorder affecting 1% of >65 years of age (Abyad, 2020). Two main neuropathological findings in PD include the loss of neurons in the substantia nigra pars compacta and basal ganglia resulting in decreased dopamine production and the development and accumulation of Lewy bodies. An earlier feature of PD is resting tremor also described as “pill-rolling”. Other primary motor symptoms of parkinsonism include bradykinesia which can be evaluated by hand movements including pronation-supination and toe/foot tapping, and rigidity or stiffness in the limbs and trunk which is also known as cogwheeling (Levin et al., 2016). A combination of rigidity and bradykinesia results in secondary motor symptoms, in which patients may present with dysarthria, dysphagia, gait freezing, hypersalivation, and mask-like expression. Additionally, nonmotor symptoms include orthostatic hypotension, urinary retention, constipation, erectile dysfunction, depression, anxiety, apathy, obsessive-compulsive disorder, and Lewy body dementia (Levin et al., 2016). Risk factors include family history and environmental risks such as well water, pesticides, industrial chemicals, and farming. Also, toxins and certain medications, such as antipsychotics, can lead to extrapyramidal side effects and motor manifestations resulting in secondary parkinsonism. Therefore, patients should decrease exposure to environmental risks and limit the use the drugs.

If a patient presents with tremor at rest, rigidity, and/or bradykinesia, Parkinson’s disease should be suspected and a neurologist should be consulted. Clinical diagnosis is based on neurological exam and assessment findings. The Movement Disorder Society-Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) can be utilized which evaluates various aspects of PD including non-motor and motor symptoms and complications. Thus, a thorough history and physical examination are essential for PD diagnosis as specific labs and imaging studies are typically unremarkable. An MRI can be useful in narrowing the differential and excluding other conditions, such as hydrocephalus or mass/lesions. However, to help confirm the diagnosis of PD is a therapeutic response to Levodopa treatment as it decreases side effects and improves CNS (Abyad, 2020).

Symptoms typically occur between ages 60-80 years and most prevalent in geriatrics; therefore, symptoms and disease may be overlooked resulting in undiagnosed/misdiagnosed in adults. Adults may have symptoms of dystonia, dyskinesia, tremors, and rigidity whereas geriatrics will also experience problems with balance and coordination, confusion, and memory loss. In later stages of PD, patients are at risk for aspiration pneumonia, falls, and cognitive decline as 80% of patients will typically develop dementia increasing morality (Abyad, 2020). Therefore, depending on symptoms, PT/OT should be consulted to increase mobility, speech therapy to maximize communication and swallow therapy to prevent aspiration.

References

Abyad, A. (2020). Parkinson’s disease: An update on pathophysiology, epidemiology, diagnosis and management. Part 2: Etiology and pathophysiology. Middle East Journal of Family Medicine, 18(6), 71–80. https://doi.org/10.5742MEWFM.2020.93824

Levin, J., Kurz, A., Arzberger, T., Giese, A., & Höglinger, G. U. (2016). The differential diagnosis and treatment of atypical parkinsonism. Deutsches Ärzteblatt International, 113(5), 61–69. https://doi.org/10.3238/arztebl.2016.0061

Q-2

Multiple sclerosis (MS) is a chronic inflammatory disease characterized by CNS lesions that may lead to severe physical or cognitive disability as well as neurologic defects (Ghasemi, Razavi & Nikzad, 2017). The etiology of MS has been considered a multifactorial disease which includes a genetic predisposition combined with environmental influences such as exposure to infectious agents, vitamin deficiencies, and smoking. Classically presents in white women with ages between 20-40 years old, with temporary visual or sensory loss (Ghasemi, Razavi & Nikzad, 2017). May present with subtle changes in vision, ambulation, and reflexes on examination that provide evidence of previous attacks, which may not have been noticed by the patient. Graying or blurring of vision in one eye can be described as looking through petroleum jelly. May have pain in moving that eye and describe the loss of color discrimination, particularly reds. Patients often describe odd sensations of a patch of wetness or burning, or hemi-body sensory loss or tingling (Ghasemi, Razavi & Nikzad, 2017).

For this patient, I will order an MRI of the brain, MRI of the spinal cord; blood tests such as CBC, CMP, TSH, and Vitamin B12. MRI is the most definitive test for MS. I will further consult a neurologist to interpret the images since they are very familiar with typical MS findings on MRI and in the context of the individual's history and examination. MRI brain with gadolinium-containing contrast should be performed on all patients with clinical presentation suspicious for MS. MRI views should include sagittal fluid-attenuated inversion recovery (FLAIR) images, which most easily distinguish between MS lesions and non-specific white matter changes (Cerqueira et al., 2016). MRI of the cervical spinal cord is also recommended for all patients to rule out cervical spondylosis. Laboratory evaluations in MS are recommended to exclude MS mimics or diseases that may contribute to MS symptoms, such as thyroid disease, vitamin B12 deficiency, and diabetes mellitus (Cerqueira et al., 2016).

Patients diagnosed with MS will highly likely develop UTI, depression, visual impairment, and impaired mobility. Patients with urinary tract infections may present only with neurologic worsening (Cerqueira et al., 2016). Urinary tract infections should be treated appropriately and preventive measures considered, such as increased hydration, control of constipation, and prophylactic antibiotics. Depression is due to multifactorial causes such as sleep disturbance and situational response. Therefore, consultation with a mental healthcare provider may be helpful. Visual complications of MS are a primary manifestation of the disease. Patients should be seen by an experienced ophthalmologist, preferably a neuro-ophthalmologist, to make sure that symptoms are truly MS-related (Cerqueira et al., 2016). Impaired mobility is treated with disease-modifying therapy that is directed in preventing progression, with variable effectiveness. Patients should be seen by an experienced physical therapist that can assist with the prescription of appropriate devices including ankle-foot orthosis and 4-wheeled walkers with hand brakes, which are preferred for MS patients due to stability issues. There are no clear strategies for primary prevention other than encouraging at-risk individuals not to smoke and possibly taking a multivitamin containing vitamin D (Cerqueira et al., 2016).

The onset of MS is infrequent and atypical after 50 years - late-onset multiple sclerosis (LOMS) and more so over 60 years - very late-onset multiple sclerosis (VLOMS) (Sanai et al., 2016). Ambulation and other physical capacities decline more rapidly in the aged MS patients. Likewise, in the psychological perspective, MS-associated psychological symptoms occur more frequently in the elderly as compared to younger patients (Sanai et al., 2016). Within an elderly MS patient group, the study showed 52.8% reported having frequent depressed feelings and moods, and 30.2% had suicidal ideation. Interestingly, while depression and fatigue remain common in older-aged MS patients, the prevalence of anxiety actually decreases (Sanai et al., 2016).

References:

Cerqueira, J. J., Compston, D. A., Geraldes, R., Rosa, M. M., Schmierer, K., Thompson, A., Palace, J. (2018). Time matters in multiple sclerosis: Can early treatment and long-term follow-up ensure everyone benefits from the latest advances in multiple sclerosis? Journal of Neurology, Neurosurgery & Psychiatry, 89(8), 844-850. doi:10.1136/jnnp-2017-317509

Ghasemi, N., Razavi, S., & Nikzad, E. (2017). Multiple Sclerosis: Pathogenesis, Symptoms, diagnoses and Cell-Based Therapy. Retrieved April 14, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5241505/

Sanai, S. A., Saini, V., Benedict, R. H., Zivadinov, R., Teter, B. E., Ramanathan, M., & Weinstock-Guttman, B. (2016). Aging and multiple sclerosis. Multiple Sclerosis Journal, 22(6), 717-725. doi:10.1177/1352458516634871

 

Q-3

Amyotrophic Lateral Sclerosis (ALS)

Typical presentation: - Amyotrophic lateral sclerosis (ALS)  is a progressive neurodegenerative disease characterized by gradual loss of upper and lower motor neurons and their pathways usually without affecting the extraocular and sphincter muscles ending in programmed cell death in selective neuronal subpopulations. The typical form of ALS with upper and lower motor neuron involvement and progressive bulbar paralysis with bulbar muscle involvement with difficulty in speaking, swallowing, articulation. Progressive muscle atrophy, where the only lower motor neuron is affected and primary lateral sclerosis with only upper motor neuron damage are rare, familiar forms of ALS (FALS) is usually associated with dementia (frontotemporal lobar dementia), behavioral disorders, cognitive dysfunction, and impairment of executive functions (Stetkarova, & Ehler, 2021). Other non-motor symptoms of GI (sialorrhea, constipation, diarrhea), autonomic (pain, dyspnea, urinary incontinence), psychiatric (depression, anxiety, sleep problems, fatigue, suicidal tendency, cognitive-behavioral impairment), vascular (dyslipidemia), itching, and pressure sores, psychosis and schizophrenia or mood disorders.

Appropriate diagnostic testing and screening: - Clinical presentation of central and peripheral motor neuron involvement in one or more segmental anatomical areas and functional impairment, nerve conduction/needle electromyography studies (EMG) Motor evoked potentials after transcranial magnetic stimulation is to identify impairment of upper motor neurons (Stetkarova, & Ehler, 2021). MRI of the brain reveals early degeneration of upper motor neurons. A biochemical-specific protein called TDP-43. Blood work such as CBC, CMP, CK, Muscle MRI, and muscle biopsy, ESR, CSF studies to exclude other neuro disorders, Chest x-ray, and urine analysis. Genetic tests and screening to rule out other neurological disorders. SMN detection (genetic testing)

Admission orders: - Admission status based on patient’s airway status. If the patient unable to maintain his oxygenation need supplementary oxygenation and as the respiratory muscles get involved, the respiratory status deteriorated and may need ventilatory support and need ICU management.

-Maintain fluid and electrolytes with IVF or in long-term tube feeding requiring gastrostomy feeding.

-Skin and pressure ulcer prevention measures, care, and management of skin.

-Managing incontinence and complications secondary to incontinence and constipation by using natural fibers, stool softener, or laxative in severe constipation.

-Management of diagnostic tests and imaging and education support and counseling with the diagnosis of ALS regarding the disease, various diagnostic tools, prognosis, end of life care and symptomatic management and finding the resource and support groups on ALS. Monitor for the elevation of WBC, fever, and other signs of infection.

Referrals and Consultations: - Referral to pomologist, neurologist, ENT surgeon to place tracheostomy and gastrostomy placement either by interventionist or surgeon. Rehabilitation services such as PT/OT/ST in the initial part of diagnosis and social service, nutritionist and case management for planning the appropriate therapy as the patient condition deteriorates, and end-of-life care when the disease gets advanced.

Potential Risks and Complications: - People with ALS are prone to breast cancer or lymphoma, noticeable weight loss in 5-10 years, motor weakness leading to split hand syndrome, gait disorders, and bulbar signs associated with vocalization (Stetkarova, & Ehler, 2021).

Interventions and preventive care and geriatric considerations: - No definitive treatment for ALS and many drugs and therapies are still being tried. Drugs such as riluzole and edaravone, are used to slow disease progression (Cruz, 2018), and also stem cell and Sodium phenylbutyrate-taurursodiol were also been tried. Care and management of ALS are directed towards the quality of life and functional independence and physical integrity. Supportive therapy such as airway management by ventilator assistance, nutrition by feeding tube, meeting hygienic, elimination and ADL needs and prevention of complications such as ventilator-associated pneumonia, malnutrition, maintaining fluid and electrolyte balance, and prevention of pressure sores and other complications. Education about nature and counseling the patient and family poor prognosis of the disease due to no successful therapy has a big role in expectation and quality of life and prepare them for transitioning as the patient’s conditions progress. Preventive care is by immunization against common pathogens such as flu vaccine, pneumococcal vaccine, meningitis vaccine, Now Covid vaccine and prevention of therapy-related complication such as pneumonia, pressure injury, infections, contractures, malnutrition and mental counseling to cope up with the disease and major life changes with poor prognosis and nature of the disease. ALS is underrecognized in elderly patients related to frequent presentation with symptoms of dysphagia, frailty, or general weakness for other reasons and ALS is to be considered as a differential diagnosis since it is common than formerly assumed.

Reference.

Stetkarova, I., & Ehler, E. (2021). Diagnostics of Amyotrophic lateral sclerosis: Up to date. Diagnostics, 11(2), 231. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7913557/

Broussalis, E., Grinzinger, S., Kunz, A. B., Killer‐Oberpfalzer, M., Haschke‐Becher, E., Hartung, H. P., & Kraus, J. (2018). Late age onset of amyotrophic lateral sclerosis is often not considered in elderly people. Acta Neurologica Scandinavica, 137(3), 329-334. Retrieved from https://onlinelibrary.wiley.com/doi/abs/10.1111/ane.12869

Cruz, M. P. (2018). Edaravone (Radicava): a novel neuroprotective agent for the treatment of amyotrophic lateral sclerosis. Pharmacy and Therapeutics, 43(1), 25. Retrieved from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5737249/