1 Neurological Disorders Prepared by: Lourdes Cabrera Certified Nurse Practitioner

2 General Approach Problems range from chronic to acute and lethal Consider cardiac or metabolic etiology, adverse medication reactions A complete neuro exam consist of motor,sensory,autonomic,cognitive and behavioral evaluation

3 General approach Focal findings suggest a space occupying lesion of the brain or spinal cord, or a peripheral compressive neuropathy Some symptoms such as generalized weakness or change in cognition may suggest a more global problem such as dementia,delirium or depression Sudden onset of symptoms such as headache unilateral weakness,aphasia,visual changes or changes in LOC, require immediate consult, referral or hospitalization.

4 Alzheimer’s Disease

5 Dr.Alois Alzheimer There are actually two forms of Alzheimer's Disease: one that's commonly diagnosed in the elderly, and the other rarer form, described by Dr. Alois Alzheimer in the late 1800's, affects people much younger. This rarer form is most commonly referred to today as Early onset Alzheimer's Disease

6 Description Dementia: impairment of global intellectual and cognitive function characterized by memory loss,aphasia,agnosia and apraxia with preservation of level of consciousness Alzheimer’s Disease: gradual onset and progressive decline without focal neurological deficits

7 Etiology Mostly unknown More neuritic plaques and neurofibrillary tangles are found on autopsy as compared to non-demented patients 3 genes on different chromosomes have been identified in families with history of Alzheimer's,although not all cases are inherited

8 Alzheimer’s Disease Hallmarks of Alzheimer's disease include neuritic plaques (outside neurons), and neurofibrillary tangles (inside neurons).

9 Incidence and Demographics Affects 5-10%,65 and older.Increases with age Affects 2-4 million Americans Often misdiagnosed or unrecognized especially in early stages

10 Risk Factors Down syndrome Family history

11 Prevention and Screening Those over 65 should have cognitive and functional evaluation every 3 years Use “Clinical guide for recognition of dementia”

12 Assessment: History Detailed history of present illness,including time frame and progression. Any associated neurological symptoms as amaurosis fugax,aphasia,unilateral weakness Past medical history: hypertension,strokes,head trauma Psychiatric history: depression,schizophrenia Medications Social and family history

13 Assessment: Physical Complete physical examination Verify for any evidence of infectious process Signs of physical and mental abuse There are no focal neurological findings

14 Diagnosis: Signs and Symptoms Alzheimer’s disease is divided into clinical stages: 1-early stage 2-intermidiate stage 3-severe stage 4-end stage

15 Early stage Loss of recent memory Inability to learn and retain new information Word finding Mood swings Personality changes Progressive difficulty in performing adl’s (balancing checkbook,remembering where they put things) Hostility,agitation Confabulation (*hallmark) Mostly reported by family members

16 Intermediate stage Unable to learn and recall new information Memory for remote events is affected but not fully lost Require help with bathing,eating,dressing,or toileting Wandering (*hallmark) Sundowning (*hallmark) Physically aggressive Loose all sense of time,place Confused;get lost in home Accident prone

17 Severe and End stage Unable to walk or perform adl’s Totally incontinent Recent and remote memory is completely lost Unable to swallow;risk for aspiration pneumonia Risk for pressure sores Eventually become mute End Stage---coma and death usually from infection

18 Diagnostic Studies Blood work to r/o metabolic causes.Syphilis,HIV and drug toxicity if indicated by history. Head ct/mri for early dementia of less than 2 years duration may show atrophy, infarcts or unexpected lesions EEG is not useful

19 Diagnostic studies Head MRI atrophy of temporal area Brain Scan- hypometabolism

20 Management  Cognitive symptoms: Donepezil (aricept) 5-10mg qd Namenda (memantine) 20mg qd  Psychosis and agitation: Haloperidol (haldol) 0.25mg -2mg tid or hs Risperidone (risperdal) 0.5mg-3mg bid Lorazepam (ativan) 0.5mg-6mg qd in divided doses

21 Management Depression: Paroxetine (paxil) 10-40mg/day Sertraline (zoloft) 25-200mg/day Nortriptyline (pamelor) 30-50mg/day Sleep Disturbances: Zolpidem (ambien)5-10mg qhs Trazadone (desyrel) 25-75mg qhs

22 Hallmarks of Alzheimer’s Disease No focal neurological deficit Neuritic plaques and neurofibrillary tangles Confabulation Wandering Sundowning

23 Parkinson’s Disease

24 Description : Neurodegenerative disease characterized by slow movement,rigidity,flexed posture,loss of postural reflex,freezing and resting tremor Distruction of substancia nigra and nigrostriatal tract occur resulting in damage to dopaminergic neurons,leaving active acetylcholine neurons intact Imbalance of dopamine and acetylcholine result in loss of voluntary movement

25 Substancia Nigra

26 Etiology/ Incidence Unknown,although genetics, toxins as carbon monoxide and illicit drug Methylphenyl-tetrahydropyridine (MPTP) have been implicated Patients who survive encephalitis have developed Parkinson's Most common neurodegenerative disease of older persons Prevalence 60-187 per 100,000; 20,000-50,000 new cases each year Greater in men than woman 3:2 ratio Affects 1% of those over 65

27 Risk factors/ Prevention and Screening Age Hereditary Possible environmental factors Older persons can benefit from periodic assessment of mobility,cognitive and functional status

28 Diagnosis Signs and Symptoms Most common symptoms are tremor and slowed movement Complain that limbs feel stiff and ache,and that fine movements are difficult. Slowed movement causes the characteristic symptoms of difficulty in rising from a chair,or getting in and out of bed “Mask-like” expression Signs and symptoms are more prominent on one side Writing becomes small (micrographia),and spidery,with a tendency to tail off at end of a line

29 Micrographia Hitler Hitler's condition was apparent by the lack of movement in his left arm. Dr. Abraham Lieberman studied about 300 hours of videos on Hitler and declared that the first symptom of Hitler's condition was apparent in 1934 (age 45). Many neurologists believe that Hitler had lost the ability to reason and grasp quickly due to his disease and this in part contributed to the downfall of Germany

30 Diagnostic studies No laboratory test exists to confirm Parkinson's disease Diagnosis is made from clinical evidence Pet Scan reveals decreased glucose metabolism

31 Physical Examination Tremor: 1.Characteristic “rest” tremor in one limb,limbs on one side,or four limbs.May be absent in 20% of patients. Usually decreased by action and increased by emotion. 2.Mouth and lips 3.“Pill-rolling” (*hallmark) Rigidity: 1.Stiffness of limbs can be felt in range of motion 2.“Leadpipe like” mostly on one side and neck 3. “Cogwheeling” (*hallmark) jerky resistance to passive movement.

32 Physical Examination Akinesia: 1.Paucity and slowness of movement (bradykinesia) 2.Difficulty in initiating movement 3.Difficulty in making normally rapid finger movements (i.e. piano playing) 4.“Mask-like” face (*hallmark) immobility of face; smile is gone 5.“Serpintine like” state- the frequency of spontaneous eye blinking is decreased; 4 th cranial nerve palsy

33 Physical Examination Postural changes: 1.Stoop is characteristic 2.Shuffling gait (*hallmark) 3.Festination and poor arm swing 4.Falls are common. Fall stiffly like falling trees 5.Sits with trunk bent forward and motionless, without gestures or animation, although limbs are tremulous 6.Problems with turns and stopping

34 Physical Examination Speech: 1.At first is monotonous,progressing to a characteristic tremulous slurring dysarthia,caused by a combination of akinesia,tremor and rigidity. 2.Dribbling and drooling is frequent 3.Dysphagia develops as the disease worsens

35 Physical Examination There is no sensory loss No motor weakness Deep tendon reflexes are unaffected, no Babinski Brisk reflexes Plantar reflexes remain flexor Cognitive function preserved early in the disease, but dementia can develop Meyerson’s Sign: repetitive tapping on the bridge of the nose produces sustained blink response

36 Management There is no cure.Therapy is aimed at managing symptoms to preserve independence and mobility Carbidopa (sinemet) 110mg tid Pramipexole (miraprex) 0.125mg tid Hoehn and Young Scale can be helpful for staging the disease and guiding pharmacological and supportive therapy

37 Hoehn and Young Scale Stage I: Unilateral involvement Stage II:Bilateral involvement but no postural abnormalities Stage III:Bilateral involvement with mild postural instability,the patient leads an independent life Stage IV: Bilateral involvement with postural instability,the patient requires substantial help Stage V: Severe,fully developed disease,the patient is restricted to bed and chair

38 Parkinson’s disease Hallmark Resting tremor Pill rolling Cog wheeling Mast like Face Shuffling gait Meyerson’s sign

39 Multiple Sclerosis

40 Multiple Sclerosis Description Progressive neurodegenerative disease characterized by demyelization and inflammation of the neuronal sheath in the brain and spinal cord Disease of the White Matter

41 Damaged Myelin

42 Etiology/ Incidence and Demographics Autoimmune disease, possible causes may be genetic,viral,immunologic or environmental Prevalence 250,000-300,000 in the US Prevalence is higher in warm climates Woman ratio 2-3:1;hormones may be implicated as symptoms develop during menstrual cycle and after pregnancy Age of onset 15-55 years

43 Risk Factors 1-3% hereditary Urban dwelling Upper socioeconomic status

44 Signs and Symptoms Paresthesias 1. Numbness: “pins and needles” sensation of hands and feet; arms and legs 2. MS Hug: tight, painful band around the stomach or torso, which is often referred to as a “girdle sensation”(*hallmark) Vertigo Muscle weakness and Spasticity Ataxia Fatigue- heats makes it worse (characteristic) Intentional tremor (*hallmark) Bladder and bowel dysfunction Visual changes Memory problems Trigeminal neuralgia

45 Physical Examination Cranial Nerve Exam: 1. Optic neuritis-decreased visual acuity,abnormal pupillary response,hyperemia,edema of optic disk 2. Cranial nerve III (oculomotor) and Cranial nerve VI-(abducens) palsy= Nystagmus Motor Exam: 1.Decreased strength,increased tone,clonus,positive Babinski 2.Decreased proprioception and vibratory sensation 3.Positive Romberg (*hallmark) 4.Lhermitte’s sign-an electrical sensation down the back into the legs is produced with neck flexion (characteristic)

46 Diagnostic Studies MRI- to visualize characteristic lesions

47 Lumbar puncture Oligoclonal Bands- important indicator in the diagnosis of multiple sclerosis. Approximately 79%- 90% of all patients with multiple sclerosis have permanently observable oligoclonal bands.multiple sclerosis (*hallmark)

48 Management Aimed at delaying progress,managing chronic symptoms and treating acute exacerbations Medications should be coordinated with a neurologist Delay progression: Interferon beta (avonex) 30mcg IM q week Glatiramer acetate (copaxone) 20mg SQ q week Acute exacerbations: Prednisone 60-80mg/day x 1week,taper over 2-3w Spasticity: Baclofen (lioresal) 40mg-80mg/day in divided doses Fatigue: Amantadine (symmetrel) 100mg bid

49 Hallmarks/ Characteristics of MS Paresthesias MS hug Fatigue worse with heat Intentional tremor Positive Romberg Lhermitte’s sign MRI lesions of white matter Oligoclonal bands