GRADING OF DEEP TENDON REFLEXES

0:

1:

2: 

3:

4:

No reflex elicited

Reflex elicited w/ reinforcement

Reflex normal

Reflex brisk

Sustained clonus elicited

UPPER MOTOR LESION (CNS)

Distal extenson > flexor weakness

Spasticity

Hyperreflexia (clonus)

Babinski +

NO m. atrophy or fasciculations

LOWER MOTOR LESION (PNS)

Flaccid weakness

Atrophy

Fasciculations

Decreased deep tendon reflex (hyporeflexia)

NO Babinski

MUSCLE DISEASE

Neuro findings:

Proximal Symmetric Weakness

Muscle tone nl to mildly dec.

Reflexes nl to mildly dec.

NO sensory loss

DDx: Polymyositis (associated w/ collagen vascular disease).

Need: EMG and muscle biopsy

Tmt: steroids, immunosuppressives

NEUROMUSCULAR JUNCTION

Neuro findings:

Weakness w/ repetitive use

Proximal symmetric weakness

No sensory loss

DDx: Myasthenia Gravis (ptosis)

Need: ACh receptor Abs, MuSK protein Abs, EMG

Tmt: thymectomy, AChase inhibitors, corticosteroids/immunosuppressives

PERIPHERAL NEUROPATHY

Neuro findings:

Distal assymetric weakness

Hyporeflexia

Numbness w/ "pins and needles"

Denervation Changes: Atrophy, Muscle tone dec., Fasciculations, Sensory Loss (in pattern of nerve involved)

Ex: Carpal Tunnel Syndrome (Median N. compression: +Phalen's, +Tinel's. Tmt: wrist splint)

RADICULOPATHY

Sharp, stabbing, hot, electric shooting down the limb pain!

Neuro findings:

Assymetric muscle weakness

Atrophy

Fasciculations

Nl to Dec tone

Sensory loss in Dermatomal pattern

Ex: L5 (herniated disk), S1, C5 & C6

SPINAL CORD DISEASE

Neuro findings:

Sensory level

Distal weakness of both extremities equally (face is spared)

Retention/or Incontinence

UML signs

Ex: Acute Spinal Cord Compression (emergency! Paralysis of both LE, distended bladder)

Brown Sequard Syn (Hemisection of cord: loss of pain & Temp on Contralater side & Weakness and loss of position and vibration on Ipsilateral side)

Central Cord Syn (Cape-like distribution of dec pain & temp w/ weak of UE; Syringomyelia (dilatation of central canal))

BRAINSTEM DISEASE

Diplopia

Dysarthria

Dysphagia

Dizziness

Deafness

Decreased strength/sensation over face

Dysequilibrium

Neuro: Contralateral Hemiparesis (corticospinal tract) &/or Hemisensory loss (medial lemniscus)

CEREBELLAR DISEASE

Clumsiness

Dysmetria on finger to nose and heel to shin

Past-pointing

Disdiadochokinesis

Wide-based, ataxic gait, inability to tandem

Cerebellar Hemisphere: symptoms on SAME side as lesion

Vermis: bilateral LE affected

CORTICAL DISEASE

Higher Cortical Fxn: orientation, language, judgment, planning, memory, agnosia, apraxia, visual-spatial skills, motor & sensation

Dominant Hemisphere (i.e. Left): Aphasia & visual field defect opposite to lesion

Non-Dominant (i.e. Right): Visual-spatial deficits, Neglect (agnosia), Graphesthesia, stereognosis & Visual field defect

Middle Cerebral A.: lower face > arm > leg

Ant. Cerebral A.: leg weakness w/ sensory loss

SUBCORTICAL DISEASE

PURE CONTRALATERAL WEAKNESS/NUBMNESS face=arm=leg

No aphasia, parietal lobe findings or visual field deficits

SEIZURE VS. EPILEPSY

Paroxysmal abnl synchronous electrical discharge in brain (10% population)

vs.

Recurrent, unprovoked seizures (1% population)

FEBRILE SEIZURE

Associated w/ FEVER

Age: 3 mths to 5 yrs (peak at 18 mths)

Simple: single, generalized & brief

Complex: multiple, focal, prolonged (MRI)

Outcome: 1/3rd recurrence, 10% have ≥4 FS

NO medication tmt

IDIOPATHIC CHILDHOOD EPILEPSY SYN:

CHILDHOOD ABSENCE EPILEPSY

Age: 3-12 yrs

Absence Type: dazed, staring & blinking

Induced by Hyperventilation

Outgrow by puberty

IDIOPATHIC CHILDHOOD EPILEPSY SYN:

JUVENILE MYOCLONIC EPILEPSY

Age: 12-18 yrs

Type: myoclonic, generalized tonic-clonic

EEG: polyspike-wave discharges

Photic Stimulation Induced

Lifelong tmt w/ AEDs

IDIOPATHIC CHILDHOOD EPILEPSY SYN:

PARTIAL

1. Benign Childhood Epilepsy w/ Centrotemporal Spikes (Rolandic): 2-13 yrs, Nocturnal seizures, Males, Centrotemporal Spikes w/ sleep activation, Resolves by adolescence

2. Benign Occipital Epilepsy: 15 mths to 17 yrs. Visual symptoms prominent. Nausea, vomiting, hemiclonus, automatisms. Spikes in occipital region w/ eye closure. Association w/ Migranes.

TICS

Stereotyped mvmt

Sudden, brief, puposeless

Motor, vocal or sensory

Involuntary

Increase w/ stress, fatigue, anxiety, stimulants

Decrease w/ absorbing activities/sleep

Wax & Wane

TOURETTE SYNDROME

Motor & Vocal tics for more than 1 yr

Onset: 2-15 yrs

Males

Comorbidities: OCD & ADHD

TREMOR

Rhythmic oscillatory involuntary mvt

Essential: benign, onset infancy to adulthood (8yrs), Male, Fam Hx, Worsened by stress, anxiety, physical activity & caffeine

Shuddering Attacks: form of ET, seen in infancy to early childhood, resolve w/ maturation

CHOREA

Rapid, brief irregular contractions that appear to flow from one muscle to the next

Causes: Inherited, Degenerative, Metabolic, Idiopathic, Drug-induced, Endocrine, Immune, Post-traumatic, Toxins & Vit. deficiency

Sydenham's Chorea (SC): most common cause of acquired chorea in children. Age 5-15. Due to Rheumatic Fever (GAS), resolves spontaneously in 3-6mths

DYSTONIA

Disturbance of posture caused by simultaneous contractiosn of agonist & antagonis (PAINFUL)

Acute: drug induced

Chronic: genetic, metabolic, or hypoxic ischemic injury

DYT1 Gen. Dystonia: Early onset, AD inheritance, reduced penetrance, defect in gene encoding Torsin A protein

MIGRANE HEADACHES

Classic (with Aura: Visual symptoms, unilateral numbness/weakness, speech difficulty/aphasia) At least 2 attacks w (3/4): 1+ aura symp (cerebral/cortical/brainstem dysfxn); 1 aura symt dev. gradually or 2 dev in succession; No aura symp >60 mins; Headache follows aura <60 mins; No organic disease explains symp

Common(W/out Aura) 5+ attacks w/ fllg: Headache 4-72 hrs untreated; 2 of fllg- unilateral, moderate to sever intensity, pulsating quality, aggravated by routine physical activity; 1 of fllg- nausea, vomiting, photophobia & phonophobia; No organic disease explaning symp

CHILDHOOD PERIODIC SYNDROMES

1. Cyclical vomiting: recurrent episodic attacks of vomitting and intense nauseas; 1 hr to 5 days duration

2. Benign Paroxysmal Vertigo of Childhood: Recurrent brief episodic attacks of vertigo lasting mins to hrs. Associated w/ nystagmus or vomiting

MIGRAINE ASSOCIATED W/ NEUROLOGICAL DEFECTS

1. Hemiplegic Migraine: evolution of unilateral face/arm>leg weakness w/ headache lasting < 24 hr. Familial is AD inheritance, attacks during childhood, pcpted by head trauma, sympt last days.

2. Ophthalmoplegic Migraine: in childhood, dysfxn of eye mvt, Horner syn may precede headache

3. Basilar Arthery Migraine: in adolescents (girls), onset w/ or w/out aura, then dizziness, vertigo, syncope, gait ataxia, vision loss &/or dysarthria

4. Acute Confusional Migraine: pds or confusion & disorientation w/or w/out agitation, vomiting; relieved by sleep. Headache may not be prominent feature

AMYOTROPHIC LATERAL SCLEROSIS (MOTOR NEURON DISEASE)

Lasts mths to years; Distal Asymmetric weakness, UMN+, LMN+, Sensory-, Genetics AD (10%)

Demographics: Males, peak onset 6th decade (<20 to >90). No racial predilection, 95% sporadic

UMN Symptoms: Loss of dexterity, slowed mvt, loss of muscle strength, stiffness, psudobulbar affect

LMN Symptoms: Loss of mus. strength, atrophy, fasciculations & muscle cramps

Labs: CK levels nl to inc; everything else nl

Prognosis: 50% dead  3 yrs, 20% live 5 yrs and 10% live 10 yrs

PRIMARY LATERAL SCLEROSIS (MND)

Lasts years, Distal Asymmetric weakness, UMN+, LMN-, Sensory-

UMN Syndrome, pts w/ slowly progressive spastic paralysis/bulbar palsy

PROGRESSIVE MUSCULAR ATROPHY (MND)

Lasts years, Distal Asymmetric weakness, UMN-, LMN+, Sensory -

Lower Motor Neuron Syndrome, better prognosis than ALS

SPINAL MUSCULAR ATROPHY (MND)

Lasts decades, Symmetric proximal & distal weakness, UMN-, LMN+, Sensory-, Genetics AR (2/3), AD or X-linked

Juvenile onset (types II & III), Adult onset (type IV). Quadricept weakness is predominant feature. Types I-III associated w/ microdeletions in 5q11.2-q13.3. Slowly progressive.

MANAGEMENT OF PTS W/ MND

1. Dysphagia: wt. loss, difficulty swallowing meds, chokind during meals, prolonged meal times, aspiration pneumonia

2. Sialorrhea: inability to clear oropharyngeal secretions

3. Psudobulbar Affect

4. Spasticity

5. Weakness

6. Respiratory Insufficienty

Palliative Care & Education

NEUROMUSCULAR JXN DISEASES

1. Pre-synaptic: not enough ACh released

2. Post-synaptic: not enough ACh binds to receptor

NEUROMUSCULAR JXN DISEASES:

MYASTHENIA GRAVIS

Occurs at any age w/ peaks in 3rd & 6th decade

Women: 10-40         Men: over 40

Clinical Features: Weakness & fatigability, diplopia, ptosis, dysarthria, dysphagia, proximal extremity & neck weakness, dyspnea, sympt worsened by rise in body Temp

Grading: 0(remission) to 4 (severe)

Dx: Hx, neuro exam, Tensilon Test, ACh (most specific) & MuSK Abs, Thyroid fxn studies

Tmt: Inc Ach (Mestinon). Dec Abs (prednisone, iv Ig, thymectomy, plasmapheresis)

NEUROMUSCULAR JXN DISEASES:

LAMBERT-EATON SYNDROME

Caused by Auto-Abs against pre-synaptic Ca Channels which block Ca-facilitated release of ACh.

50-70% associated w. SMALL CELL LUNG CANCER

Clinical: weakness in limb-girdle distribution, Exercise improve strenght, mild ptosis, diplopia, dysphagia &/or dysarthria. Autonomic symptoms: dry mouth, orthostasis & impotence

Dx: Voltage-gated Ca Channel Ab

Tmt: tumor therapy, mestinon, prednisone, plasmapheresis, iv Ig

NEUROMUSCULAR JXN DISEASES:

BOTULISM

Caused by exotoxin of Clostridium Botulinum (ingestion of improperly canned food/ or infancy 3-18 wks)

Toxin interferes w/ release of ACh

Adult botulism: Nausea, vomiting, blurred vision, diplopia, paralysis of cranial & respiratory muscle, nl reflex

Tmt: trivalent antiserum, supportive care

Tick Paralysis: tick neurotoxin blocks neuromuscular transmission; resembles botulism or GBS. Paralysis subsides w/ removal of tick!

PARKINSONISM

Bradikinesia

Rigidity (cogwheel)

Instability (postural)

Tremor (at rest/postural)

ATYPICAL PARKINSONISM (PLUS)

1. Multiple system atrophy: parkinsonism w/out tremor and minimal response to DA. Autonomic or Cerebellar dysfxn

2. Progressive Supranuclear Palsy: parkinsonism, but axial rigidity (upright posture) & Downward gaze palsy & frequent falls early

PARKINSON'S DISEASE

1. Resting Tremor: asymmetrically, Pill rolling

2. Bradykinesia: slowing/poverty of voluntary mvt, mak-like facies/ dec arm swing, shuffling gait

3. Micrographia & microphonia

4. Rigidity: inc resistance to passive mvt (cogwheel)

5. Postural instability

Spares NO ONE, Inc w/ age.

Path: loss of DA in dopaminergic nigrostriatal system. Lewy body eosinophilic inclusions

Tmt: inc DA (L-dopa, COMT inhibitors, DBS) pts can have on/off pds

CHOREA

BALLISM

"To throw", violent, irregular flinging mvt of limbs, due to contractions of proximal muscles

ATHETOSIS

MYOCLONUS

HUNTINGTON'S DISEASE

AD inherited due to CAG repeats (>40) on chromosome 4

Path: atrophy/absence of corpus striatum

More common in Europena ancestry, 30-50 yrs

Clinical: abnl extra-ocular mvt w/ delayed, slow saccades, clumsiness, chorea, and dystonia/parkinsonism

Affects: motor (chorea, dystoni, gait disorder), psychiatric (affective, & ocd) and Cognitive (executive dysfxn, dementia, personality changes)

DYSTONIA

Sustained muscle contraction (twisting/repetitive mvt or abnl posture)

Early onset torsion dystonia: idiopathic, inherited (DYT1-13)

Secondary: lesions of basal ganglia, toxins/drugs

Focal torsional dystonia: most common, blepharospasma, writer's cramp, spasmodic torticollis

Hemidystonia: unilateral involvment of limbs, usually secondary to stroke, AV malformation of MS

Tmt: Botox, anti-cholinergics, DBS

ESSENTIAL TREMOR

Tends to be familial

Bimodal onset, peaks 40-60 yrs

Interferes w/ simple taks of hands

Fine and more rapid tremor of one/both hands, head, voice (rarely legs)

Inhibited w/ EtOH

Tmt: primidone or propranolol

WILSON'S DISEASE

AR inheritance of impaired Cu metabolism (hepatolenticular degeneration)

Clinical: Kayser-Fleischer rings (yellow-green-brown corneal deposits) Neuro- tremor, chorea, rigidity, dysarthira, flexed porsture, ataxia, dementia

Dx: Low/absent ceruloplasmin & Kayser-Fleischner rings

Tmt: Penicillamine, tetrathiomolybdate, LIVER transplant

RESTLESS LEGS SYNDROME

Associated w/ Fe deficiency anemia, prevalence inc. w/ age. more common in Females.

Clinical: unpleasant sensation in the legs, relieved by moving the legs or walking. Occurs most at rest and night

Tmt: treat Fe deficiency, dopaminergis, BZ, opiods

MUSCULAR DYSTROPHIES

Disease w/ abnl Dystrophin protein

Dystrophin: acts as functional link btw cytoskeletal proeins and extracellular matrix. It is essential for maintaining membrane integrity during muscle contraction

DUCHENNE MUSCULAR DYSTROPHY

X-linked recessive dystrophy. Most common MD. Female carriers are asymptomatic. Mutation results in non-functional protein (Out-of-frame deletion of Dystrophin gene)

Dx: CK elevation, PCR, Western blot, DNA testing, Muscle biopsy w/ dystrophin stainin

Tmt: prednisone, sgy for scoliosis

BECKER'S MUSCULAR DYSTROPHY

X-linked recessive dystrophy due to in-frame mutation in Dystrophin gene. Milder phenotype.

Age onset >7yrs. Failure to walk by 16-80 yrs.

Cardiomyopathy may dev. prior to weakness

MYOTONIC DYSTROPHY

Myotonia: repetitive firing of the muscle fiber due to membrane hyperexcitability & delayed relaxation

Myotonic dystrophy Type 1 (DM1) most common!

DM1: serine-threonine protein kinase on chromosome 19 (CTG repeats). Anticipation occurs

Onset: childhood or adult (2nd-4th decade)

Facial weaknesss w/out ophtalmoplegia, Distal hand, leg weakness & neck flexion weakness. Other features: MR, Personality disroder, frontal balding, hatchet face, cataracts, testicular atrophy, hypoventialation & cardiac arrhythmia.Infants succumb to respiratory failure

Dx: CTG repeat & CK nl to 3x nl

Managemement: annual eval by cardiology & ophthalmology!

LIMB GIRDLE MUSCULAR DYSTROPHY

Inheritance AR, AD or X-linked

Onset ranges 1st - 5th decade

10% associated w/ Cardiomyopathy

CK levels elevated but less than 10x nl

INFLAMMATORY MYOPATHY

Polymyositis: cell mediated autoimmune, >20yrs, Limb-girdle weakness. Associated w/: ILD, Cardiomyopathy, arrhythmias, esophageal paresis & myalgias. CK elevated 5-10x nl

EDX: low amplitude & short duration polyphasic motor units. Muscle biopsy: necrosis, phagocytosis (CD8+ cytotoxic T-cells, mΦ)

Dermatomyositis: Humural mediated autoimmune response; affects children & adulst. Associated w/ Malignancy. Limb-girdle weakness w/ erythema, Heliotrope rash, Gottron's papules. CK elevated. EDX: + sharp waves & fibrillations. Muscle biopsy: perifascicular atrophy & perivascular inflammation (cd4+ t-cell & b-cells)

Inclusion Body Myopathy: most common in adults, asymmetric distal arm & proximal leg weaknes. Males >50, mildly elevated CK. Bioplsy shows Rimmed vacuoles. No tmt.

STATIN MYOPATHY

Associated w/ lipid lowering agents: Lovastatin & Simvastatin (HMG CoA inhibitors & fibrates). Onset 2-3 mths after meds.

Myalgia, weakness and myoglobinuria

Tmt: stop medications

ENDOCRINE MYOPATHY

Hypothyroid Myopathy: muscle pain, cramps, stiffness, fatigue & paresthesias

Neuro exam: proximal weakness, muscle hypertrophy, myoedema

CK significantly elevated (10-100x)

CRITICAL ILLNESS MYOPATHY

Seen in ICU pts w/:

Tmt w/ corticosteroid (CK inc w/in 9 days) & neuromuscular blocking agents

Status asthmaticus

Multi-organ failure/sepsis

Clinical: ventilatory dependence, generalized/proximal flaccid paralysis & muscle atropy

PSYCHOGENIC NON-EPILEPTIC SEIZURES (PNES)

Paroxysmal ghanges in behavior that resemle epileptic seizure, but psychologically induced.

Clinical: gradual onset, asymmetrical thrasing mvts oflimbs, pelvid thrusting, taling or screaming, long duration, sudden return to consicouusness, poor response to AEDs. *Eye closure*

Tmt: psychotherapy, stress reduction, reassurance and discontinue AEDs

SAFETY DRUING A SEIZURE

• Do NOT hold person down or try to stop mvt
• Clear area for anything hard or sharp
• Loosen ties or anything around neck
• Turn pt gently onto one side (to clear airway)
• Do NOT try to force mouth open or put anything in pts mouth (CANNOT swallow tongue!!)

DEMENTIA

Acquired syndrome of intellectual impairmentt

Interferes w/ social & vocational fxn

Impairment in at least 3 fllg:

Language

Visual-spatial

Cognition

Memory

Personality

NOT a nl part of aging

PURE MEMORY LOSS

Herpes encephalitis sequelae (bilateral m. temp lobe lesion)

Korsakoff's amnesia

Vertebral-basilar infarct

Transient global amnesia

Limbic encephalitis (damage to hippocampus/limbic pathway)

CORTICAL DEMENTIA

1. Alzheimer's disease: Most common dementia. Memory impairment plus- aphasia (fluent, anomic, good repetition), apraxia, agnosia and disturbance in executive functioning. Gradual onset. Visual-spatial deficit (gets lost), Memory deficit (recent & remote). Histo: Neurofibrillary tangles & senile plaques, neuronal loss in nucleus basalis of Meynert (dec. ACh). ApoE allele inc risk for AD

2. Frontotemporal Dementia (Pick's): early extravagant personality changes & stereotyped language output (repetitive joke telling)

SUBCORTICAL DEMENTIAS

Psychomotor Retardation & Forgetfulness

Abnormal speech & cognition

Forgetfulness can improve if provided w/ a clue (prompted)

Cognitive dilapidation, Impaired insight, poor strategey formulation.

Involves: thalamus, basal ganglia, & rostral brainstem

DEMENTIA W/ LEWY BODIES

LB occur in the Cortex

Clinical criteria:Fluctuating cognition, attention & alertness, visual hallucinations & extrapyramidal signs

NORMAL PRESSURE HYDROCEPHALUS

Clinical triad: Ataxia, Incontinence & Dementia

 Shunt, high morbitiy

MAJOR RISK FACTORS OF CEREBROVASCULAR ATHEROSCLEROSIS

Hypertension (5-10x)

Smoking (2x)

Diabetes (2x)

Hyperlipidemia (1.5x)

Obesity (1.5x)

TRANSIENT ISCHEMIC ATTACK

Reversible "warning strokes" (5-10 mins)

Carotid territory TIA: unilateral weakness & sensory symp. Aphasia, dysarthria & amaurosis fugax (transient monocular blindness).

Vertebrobasilar territory TIA: bilateral weakness & sensory symp, Diplopia, Dizziness, Dysarthria, Dysequilibrium (ataxia) & Dysphagia

MAJOR MECHANISMS OF ISCHEMIC STROKE

1. Large artery atherosclerosis (60%): plaques cause stroke by thrombotic occlusion (flow compromise) or artery to artery embolism

2. Penetrating artery disease (20%): cause lacunar strokes

3. Cardiogenic embolism (15%)

4. Unusual causes (5%): young pts, drug induced

STROKE IN CORTEX OF RIGHT HEMISPHERE

Neglect of Left side of body

Stereognosis contralaterally

Graphesthesia contralaterally

Left homonymous hemianopsia

Gaze preference to the Right

MIDDLE CEREBRAL ARTERY STROKE

Hemiparesis (face, arm & hand worse than leg)

Hemianopsia and either aphasia (dominant hemisphere) or neglect (non-dominant hemisphere)

Usually due to atherosclerosis or cardiogenic embolism

INTRACRANIAL HEMORRHAGE

1. Subarachnoid: due to Aneurysm or AV malformation. In younger pts. "worst headache of my life" and/or stiff neck, stupor w/out focal neurlogic findings

2. Intraparenchymal: hypertensive; cerebral amyloid angiopathy in cortex of very elderly. Acute headache w/ obtundation and focal neurological signs

STROKE CHECK

WALK (balance off)

TALK (slurred speech or droopy face)

REACH (one side weak or numb)

SEE (vision partly or all lost)

FEEL (headache severe)

MULTIPLE SCLEROSIS

Demyelinating disease of CNS (WHITE MATTER).

Lesions separated in both TIME & SPACE

MS attacks better prognosis if Relapsing-Remitting and Primary Progressive

Initial Symptoms:

Sensory: Ascending numbnesss in feet & hands, hemiparethesisa, Dysesthesia & genera heat intolerance

Double Vision (internuclear ophthalmoplegia)

Optic Neuritis (painful vision)

Muscle weakness & Gait disorder

Do NOT forget: Urinary Incontinence, Cognitive & Emotional abnl, Fatigue & Sexual dysfxn

MRI ABNORMALITIES OF MS

Multiple White Matter Lesions

Lesions >3mm

Corpus Callosum invlmt

Periventricular (Dawson's fingers)

Ovoid Shape

Gadolinium enhancement, sometimes ring enhancing (Active Lesion)

Short, <3, spinal cord segments

CSF STUDIES IN MS

Immunoglobulin [] elevated in >90% MS pts

Elevated IgG index

Oligoclonal Immunoglobulin Bands via electrophoresis

Elevated Protein levels

MS

Affects: Women, Germanic descent, 20-30 yrs old

Causes:

Autoimmune: inflammation & demyelination in CNS (memory T-cells against myelin)

Viral: EBV + serology

Environmental: Vit D & Sunlight Exposur protective; Smoking - 60% risk

Genetics: HLA-DRB1 gene on chromosome 6p21

LARGE FIBER NEUROPATHY

Reduced sensation to Vibration & Position sense

Reduced Reflexes

SMALL FIBER NEUROPATHY

Reduced sensation to Touch or Pinprick

Skin Color Changes

Reduced Hair Growth & Sweating

MOTOR NEUROPATHY

Reduced strength

Atrophy of muscles

Fasciculations

Reduced Reflexes

AUTONOMIC NEUROPATHY

Orthstatic/Postural Tachycardia

Orthostatic Hypotension

Tonic pupis- absent light reflex

Delayed Gastric Emptying Times

ENTRAPMENT NEUROPATHIES

1. Bell's Palsy: Facial Nerve (VII) palsy; may be due to reactivation of HSV-1; sudden onset weakness of upper & lower half of face, loss of taste and hyperacusisipsilaterally. Tmt: Steroids & antiviral meds

2. Carpal Tunnel Syndrome: Median Nerve entrapment; parethesia of hand worse at night, weakness & wasting of abductor pollicisbrevis m.; +Tinel's & Phalen's sign

3. Cubital Tunnel Syndrome: Focal entrapment o Ulnar nerve. Dec. sensation in pinky. Does NOT go above wrist. Weakness & wasting of First dorsal interossei. Partial Claw hand

MYELONOPATHIES

Primary attack: Schwann Cell & Myelin sheath

Produces: Random/Semental Deymyelination

Includes: AIDP(GBS), CIDP, CMT

Clinical: Proximal & Distal weakness, Areflexia

Labs: Nerve conduction velocities slow; CSF protein elevated (albumin-cytologic dissociation)

GUILLAIN-BARRE SYNDROME (AIDP)

Triggers: Viral (EBV, CMV, HIV), Immunizations

Clinical: P/D weakness, areflexia, facial weakness, pain, paresthesias, autonomic signs, resp failurs

80-90% recover w/ little disability

Tmt: ivIg or plasmapheresis if- non-ambulatory, impaired resp fxn & rapid progressive weakness

CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY (CIDP)

Clinical: symptoms for at least 8wks; p/d symmetric weakness, areflexia

Path: immune mediated; affects motor>sensory fibers; small & large fibers (pansensory loss); monoclonal/M proteins

EDS: demyelination indicated by slowed conduction velocities & prolonged distal latencies

DISTAL AXONOPATHIES

Clinical: weakness/atrophyonly Distal, tingling/burning stocking-glove pattern, loss of ankle reflexes

Path: degenaration of distal axon; initially affects Longest axons, may be metabolic dysfxn

Labs: cmpd ms. AP are low amplitude; Fibrillations on EMG

AXONAL NEUROPATHY: VIT B12 DEFICIENCY

Clinical: Length dependent

Early distal paresthesias: loss of vibration & position sense, areflexia (esp ankle jerk), UMN signs due to long tract invlmt

Lab: low B12

Tmt: intramuscular cyanacobalamin

DIABETIC NEUROPATHY

Risks: poor glycemic control, hypertriglyceridemia, smoking & obesity

Most common: distal symmetric large & small fiber sensory neuropathy

Lumbosacral plexopathy (L2-4): severe pain in hip & thigh (poor diabetic control & wt. loss)

HEREDITAY SENSORIMOTOR NEUROPATHIES

Presents: 1st decade, usually AD

Pescavus & Hammer toe Deformities

Distal muscle atropy/weakness

Enlargement of peripheral nerves in HSMN1 & HSMN3

SYMMETRIC PROXIMAL + DISTAL WEAKNESS W/ SENSORY LOSS & AREFLEXIA

SUSPECT: GBS & CIDP

ONLY DISTAL WEAKNESS ASYMMETRIC W/ SENSORY LOSS

SUSPECT: VASCULITIS (NEED NERVE/MUSCLE BIOPSY & TMT W/ IMMUNOSUPPRESSIVES)

ONLY DISTAL WEAKNESS ASYMMETRIC W/OUT SENSORY LOSS

SUSPECT MND

ONLY DISTAL WEAKNESS SYMMETRIC W/OUT SENSORY LOSS

SUSPECT: HEREDITARY (W/ PESCAVUS FOOT DEFORMITIES)

ONLY DISTAL WEAKNESS ONLY SENSORY LOSS

SYMPTOMATIC Rx

ONLY DISTAL WEAKNESS SENSORY + UMN

SUSPECT B12 DEFICIENCY

DECEREBRATE

Extension of the UE

Lesion in Upper Brainstem

DECORTICATE

Flexion of UE

Lesion of Hemisphere or Capsule (Cortex)

OCULOVESTIBULAR REFLEX (CALORICS)

Normal, Alert pt:

Cold Water: Fast nystagmus OPPOSITE Side

Warm Wate: Fast nystagmus SAME Side

Coma pt:

Cold Water: NO nystagmus; Eye gaze to SAME side

Warm Water: NO nystagmus; Eye gaze to OPPOSITE side

OCULOCEPHALIC REFLEX (DOLL'S EYES)

AVOID CERVICAL TRAUMA!

Brainstem Intact: Eye gaze conjugately OPPOSITE direction of head mvt (Doll's intact)

Doll's Absent: Eye's do NOT move (brainstem damage or coma too deep)

Brainstem Damage: Eyes move NON-conjugately

FRONTAL EYE FIELDS (CORTICAL AREA 8)

Irritatvie Lesion (Seizure): Pt looks AWAY from source of irritation

Ablative Lesion (Infarct, Hemorrhage): Pt looks TOWARD lesion

Pontine Gaze Center Lesion: Pt looks AWAY from lesion

METABOLIC COMA (SUPRA/INFRA TENTORIAL)

Most common (70%); affects cortex & brainstem

Pupillary rxns preserved until late

Confusion, lethargy & delirium precede coma

Motor signs symmetric

Asterixis: flapping mvt due to liver/kidney disease

INFRATENTORIAL LESION (BRAINSTEM)

Primary: due to hemorrhages, infarction, tumors & abscesses in the brainstem

Secondary: due to Supratentorial lesions that lead to Brainstem Herniation-

Central: edema or inc. pressure causes hemispheres to push Down on Braintem (leads to tearing, duret hemorrhage)

Uncal: focal lesion of one hemisphere causes Pressure on Midbrain on that side. Uncus pushes on Brainstem. IPSILATERAL DILATED PUPIL!

Hallmarks: Loss of pupillary reflex, Oculomotor reflexes absent & Coma onset is Sudden

SUPRATENTORIAL LESIONS

Onset involves Focal cortical symptoms: aphasia, contralateral hemiparesis & sensory changes

Bilateral cortical lesion >> Coma

Dysfxn moves: Rostral to Caudal

Brainstem fxn Spared (unless herniation develops)

VEGETATIVE STATE

No Cortical Fxn

Intact Brainstem: can swallow & breathe, nl diurnal rhythms (reflex)

ACUTE VIRAL MENINGITIS

85% viral meningitis due to : ENTEROVIRUS (fecal-oral route; acid/bile resistant) Enteroviral M.: Infants & Children

HSV-1: ENCEPHALITIS rather than meningitis

Clinical: subacute w/ gradual progression, may have severe headache; "less sick"; post-neonatal group viral meningitis is self-limiting

Labs: CSF pressure mildly elevated, protein mildly elevated, glucose normal, Early Neutrophils predominant

Tmt: supportive; Acyclovir if HSV2

ACUTE BACTERIAL MENINGITIS

Big Three: S. pneumoniae (very  young & old). N. meningititds (child-teen). H.influenzae (very young)

Others: S.aureus (primary/remote focus endocarditis/osteolmyelitis). Enteric Gram N. (trauma/neonates). GBS (neonates) Listeria (immunocompromised). Brucella (unpasteurized dairy products)

Pathogens: colonized nasopharyngeal mucosa, have antiphagocytic capsule (virulence factor)

Sx: fever, headache, meningismus (stiff neck), and mental dysfxn

Labs: CSF pressure & protein high, glucose Low, PMN predominance

Tmt: 3rd gen cephalosporin (ceftriaxone/cefotaxime), Vancomycin & Ampicillin (for Listeria) + Corticosteroid (prior or at time of antibiotics to decrease neurological sequelae)

CHRONIC MENINGITIS

Due to: TB, Lyme, Coccidiodes, Cryptococcus (AIDS pt), Syphilis, Listeria (queso fresco) & Cysticercosis (pork tapeworm; Mexico)

Dissemination through: blood, primary focus (immunocompromised)

High risk for Hydrocephalus

Labs: CSF pressure Extremely High, protein elevated, glucose low, Lymphocytes

BRAIN & EPIDURAL ABSCESS

Brain: oropharyngeal anaerobes, streptococci, pneumococci & Haemophilus. Hematogenous dissemination (distant foci-middle cerebral a. distribution) or Contiguous (from osteomyelities/embolus-frontal/temporal lobes). Lumbar puncture Contraindicated. Tmt- antibiotics & sgy drainage

Spinal Epidural: S. aureus. Back pain. Tmt- sgy drainage immediately

VIRAL ENCEPHALITIS

Sx: fever, myalgia, arthralgia, weakness, nausea & vomiting (more sever in young child, older adults and imcmpd)

Etiology: HSV-1 (temporal lobes; tmt-Acyclovir), VZV, childhood viral infxn, Arbovirused (july-sept)

CSF: mild pleocytosis, mildly elevated protein & normal glucose (similar to virus)

CHRONIC VISUAL LOSS IN GERIATRIC POPULATION

CATARACT

MACULAR DEGENRATION

DIABETIC RETINOPATHY

GLAUCOMA

AGE RELATED DEFICITS

Decrease in:

Visual Acuity

Contrast Sensitivy

Visual Spatial Attention

GLAUCOMA

Significant cause of blindness: African Americans, Inc. w/ Age.

Asymptomatic

Loss of Peripheral Vision

High IOP (>21 mmHg) > Optic N. damage

IOP: dependent on ease of flow thru trabeculum & Schlemm's canal

TYPES OF GLAUCOMA

1. Open-Angle: chamber angle remains open

2. Acute-Angle-Closure: emergency! due to abrupt rise in IOP: pain, nausea, coored halos or rainbows around light

3. Chronic-Angle-Closure: low grade symptoms of headache & blurred vision; gradual inc of IOP

4. Congenital/Infantile

5. Juvenile

GLAUCOMATOUS CUPPING

Increase in size of Optic Cup relative to Optic Disc

Due to loss of nerve fibers bundled in the optic nerve

C/D ≥ 1/2 raises suspicion of Glaucoma

Asymmetric C/D ratios suspicion of Glaucoma

Disc hemorrhage

MACULAR DEGENERATION

Leading cause of Irreversible Central visual loss (20/200 or worse) in old adults (>50)

Macular Changes due to Age:

Drusen: hyaline nodules deposited in Bruch's membrane

Degenerative changes in Retinal Pigment Epithelium: hyper/hypopigmented

Choroidal Neovascularization: "wet" MD (dected w/ Fluorescein Angiography)

Metamorphosia: central visual distortion

Scotoma: blind spot

ANTIOXIDANT TMT FOR MD

Vitamin C

Vitamin E

Beta-Carotene (not in smokers b/c Inc. risk for lung cancer)

Minerals: zinc/cupric oxide

CATARACT

Most COMMON caue of Decreased Vision in people >70

Opacity or Discoloration of the lens

1st symptom: Image Blur as lens loses ability to resolve separate objects

Nuclear Sclerosis: pts dev. myopia (due to inc refractive power of denser nucleus), "second sight", "starbursts" & difficulty driving at night

Posterior Subscapular Cataracts: rapid dec. in vision, affects Near vision, associated w/ metabolic causes (i.e. DM, corticosteroids)

Denser Catarct: Poorer Red Reflex

ACUTE VISUAL LOSS: HYPHEMA

Blood in the Anterior Chamber

Reduces Vision (can reduce to light perception only If Complete)

Due to Direct Trauma Or abnormal iris vessels (spontaneous)

ACUTE VISUAL LOSS: VITREOUS HEMORRHAGE

Bleeding into Viterous reduces vision

Can be due to: trauma, Retinal tears, Subarachnoid Hemorrhage

Need to dilate pupil

Red Reflex Absent

ACUTE VISUAL LOSS: RETINAL DETACHMENT

Pt complains of: flashing lights > floaters > shade over vision in one eye

Relative Afferent Pupillary Defect in uninvolved eye

Emergency opthalmologic consultation

Need dilated

ACUTE VISUAL LOSS: RETINAL VASCULAR OCCLUSION

Sudden visual loss transient or permanent

Amaurosis Fugax: transient monocular visual loss due to arterial insufficiency

Embolic Source: look at valves & chambers of ♥

CENTRAL RETINAL ARTERY OCCLUSION (CRAO)

Prolonged interruped retinal arterial blood flow > damage to ganglion cells> optic atropy

Sudden, painless Visual Loss

Cherry-Red Spot: pallor of perifoveal retian in contrast to fovea

Pale disc in a blind eye

Ophthalmic Emergency

Press/Release on eye (rise/fall in IOP) to dislodge small embolus

Evaluate for Giant Cell Arteritis if no visible emobulus

BRANCH RETINAL ARTERY OCCLUSION (BRAO)

Vision ONLY Partially lost

Pt will know moment of vision loss (exact outline of missing area of vision)

Due to Embolus

Ocular Massage: to dislodge embolus

CENTRAL RETINAL VEIN OCCLUSION (CRVO)

Disc swelling, venous engorgement, cotton wool spots & diffuse retinal hemorrhages: "BLOOD & THUNDER"

Not true ophthalmic emergency

Older pts due to HTN & arteriosclerotic vascular disease (rare cases of blood viscocity SCD, Leukemia)

Pt need general medical evaluation and followup w/ ophthalmologist to prevetn neovascular glaucoma

DIABETES MELLITUS

Diabetic Retinopathy

Risks: poor glycemic control, hypertension, proteinuria, or anemia

1st stage: Nonproliferative Diabetic Retinopathy (NPDR): microaneuryms, dot-blot-hemorrhages, hard exudates & macular edema

Clinically Significant Macular Edema (CSME): most common cause of mod. visual loss

Proliferative Diabetic Retinopathy (PDR): profound visual loss; due to continued retinal ischemia & neovascularization (rubeosis iris)

HYPERTENSION

Arteriolar Sclerosis

Elevated Blood Pressure

Opthalmoscopic Indicators: attenuation of retinal arterioles, focal narrowing & A/V crossing changes

HYPERTENSIVE RETINOPATHY

GRADE 0:

GRADE 1:

GRADE 2:

GRADE 3:

GRADE 4:

NO changes

Barely detectable arterial narrowing

Obvious arterial narrowing w/ focal irregularities

Grade 2 PLUS retinal hemorrhages &/or Exudates

Grade 3 PLUS Disc Swelling

ELEVATED BLOOD PRESSURE

Constriction of the Arterioles

Fibrinoid Necrosis of vessel wall, exudates, cotton-wool spots, flame-shape hemorrhages & whitish edema of retina

Most severe form of Hypertensive Retinopathy: PAPILLEDEMA

ARTERIOLAR SCLEROSIS

Retinal Arteriolar Sclerosis: an increase in the light reflex of the arteriole & changes in AV crossing

Ophthalmoscopy:

1st: central light reflex increases in width

2nd: light reflex occupies most width of vessels: Copper-Wire Arterioles

3rd: light reflex totally obscured: silver-wire arterioles

AV nicking: dilation of distal portion of vein & tapering of vein on either side of the artery (can lead to branch retinal vein occluion > hemorrhage)

RED EYE

Hyperemia or Injection of the superficially visible vessels fo the conjunctiva, episclera or sclera

Due to simple d/o: subconjunctival hemorrhage or infectious conjunctivitis

Due to serious d/o: intraocular inflammation, corneal inflammation or acute glaucoma

DISORDERS ASSOCIATED W/ RED EYE

ACUTE ANGLE-CLOSURE GLAUCOMA: occlusion of anterior chamber angle. Iris appears displaced anteriorly, shallow A/C, elevated IOP

IRITIS/IRIDOCYCLITIS: inflammation of iris or iris & ciliary body: Ciliray Flush

HERPES SIMPLEX HERATITIS: by HSV, inflammation of the cornea, can lead to corneal ulceration

CONJUNCTIVITIS: hyperemia of conjunctival blood vessels (bacterial, viral, allergic or irritative). If purulent think Gonococcal conjunctivitis

EPISCLERITIS: inflammation of episclera, discharge is common, possibly allergic (not serious)

SOFT CONTACT LENS-ASSOCIATED: inflammation superficial & easily resolved or vision-threatening injection

SCLERITIS: inflammation of slcera, often protracted, PAIN, assoc. w/ systemic disease (collagen-vascular d/o)

DISORDERS ASSOCIATED W/ RED EYE

ADENXAL DISEASE: affects eyelids, lacrimal apparatus & orbit (dacryocystitis, stye & blepharitis or from 2ary lesion i.e. tumor)

SUBCONJUNCTIVAL HEMORRHAGE: accumulation of blood bwt conjuntiva & sclera. Bright Red color, nl vision

PTERYGIUM: abnl growth of triangular fold of tissue over the corneal (from nasal side; not serious). Sgy excision if encroachin on visual axis

KERATOCONJUNCTIVITIS SICCA: from lacrimal deficiency (dry eyes) not serious

ABRASIONS & FOREIGN BODIES: Painful! fluorscein staining

CORNEAL ULCERATIONS: loss of integrity of corneal epithelium w/ infxn/inflammation lead to ulcer. Cornea appears Hazy or White, pain & photophobia

SECONDARY TO ABNL LID FXN: Bell's Palsy, thyroid ophthalmopathy. Lagophthalmos: poor lid closure

SYMPTOMS OF RED EYE

Blurred Vision                           Shallow Anterior Chamber Depth

Severe Pain                             Elevated IOP

Photophobia                            Proptosis

Colored Halos                          Discharge

Exudation                               Periauricular Lymph Node Enlargement

Itching                                   Reduced Visual Acuity               

Ciliary Flush                            Conjunctival Hyperemia

Conrneal Opacification              Corneal Epithelial Disruption

Pupillary Abnormalities

SUBCONJUNCTIVAL HEMORRHAGE

RISKS OF TOPICAL CORTICOSTEROIDS

Keratitis

Cataract

Elevated IOP

Combination w/ antibiotics carries same risks