DEMENTIA



KNOW all the possible complications of STROKES from the following branches in the objectives, along with the superior & inferior division of the MCA, and the circle of willis in it’s entirety. Know the lobes & all the information they could lose, especially all the lobes involved with vision, voluntary eye movements, etc. Also, aphasia & all the different types and which ones are in which lobes and infarction present how, expression, conductive, word salad, you get the idea. Also, know what to do & what not to do w/ a patient with an acute head injury, in a stepwise fashion, who may be unresponsive. Order does matter and was specific. Know the thyroid and its implications to neurology in detail. This stuff was tested and not really covered well in the objectives, but remember this is only a few peoples opinion. Julie Scott’s notes are pretty well used by most but with a disk you can cut, delete, add & edit, not to mention print for free in the LRC! Good luck ! Kim Northup, 2002

Unit One Cerebrovascular Disease

3 Categories of stroke

- ischemic (usually thrombotic > embolic)

- hemorrhage (usually embolotic at bifurcations of large arteries like MCA)

- lacunar or deep infarcts in the Internal capsule, or Grey matter i.e. potential seizure foci

Risk Factors:

Natural: Preventable:

-age, gender, &race HTN, hyperlipidemia, diabetes, valve abnormalities

smoking, ETOH

Artery distribution:

ACA CL weakness or numbness Legs> arm, MIXED motor& sensory

Loss of decision making capacity (abulia), Urinary Incontinence

MCA CL weakness or numbness Arms & face, CL hemianopsia, CL gaze

Superior branch Palsy (Eyes Look to the Lesion), Aphasia for dominant and neglect if

Nondominant, MIXED motor and sensory

Inferior branch of MCA interrupted visual radiation, Wernicke’s aphasia

PCA CL hemianopsia, pure sensory or pure motor syndrome

PCA bilaterally Cortical blindness, prosoagnosia

PCA branches CL hemibalism if the subthalamic nucleus is involved

Verterbrobasilar Ataxia, vertigo, diplopia, dsyarthria, dysphagia, ipilateral CN, with

Weakness and numbness in extremeties (crossed finding)

AICA Pons, gaze palsy, ipsilateral facial weakness, deafness possibly

PICA branch of vertebral Wallenberg’s syndrome of CL loss of P & T ipsilateral CN findings,

Ipsilateral Horners, ataxia, nystagmus, hoarseness, dysphagia

Lacunar Infarcts of deep Basal ganglia, Pons, cerbellum, and anterior limb of IC

Grey cortical matter CL pure motor or pure sensory hemiparesis Ipsilateral ataxia

Anterior Spinal Artery CL UMN hemiplegia near C5 w/ infarct of spinal cord, CN XII nucleus

1st branch of the vertebral

Carotid dissection usually due to trauma & can present with a Horner’s from SNS fibers

Basal brainstem infarct CN III, VI, VIII , CL hemiparesis

Thrombotic occulsions are almost always atherosclerotic and occur at IC & carotid bifurcation or in the vertebrobasilar system depending upon the normal functioning of the Circle of Willis.

Asymptomatic Carotid Bruit- between 30-70% occulded usually

TIA- stroke w/neurological signs resolving in < 24 hrs ; usually in 30 min, and recurrent TIAs could be due to cardiac or thromobosis or embolism in the cerebral circulation

Lacunar Infarct- occlusion of small penetrating branches from hyalinosis due to HTN, DM, and atherosclerosis

Hemorrhagic infarct- vessel imcompetence leading to destructive or compressive bleeding that can lead to ischemic infarct or edema, presents w/ a severe HA and LOC

Carotid Endarterectomy- surgical removal of thrombus from stenotic carotid arteries w/ stenosis > 70% used for unstable TIA’s w/ major risk of stroke, not done in vertebrobasilar system

Spontaneous Hemmorhage-

- ruptured cerebral artery aneurysm

- AVM’s men > women 2:1, ages 20-40 yo

- Berry aneurysms 50-60 yo ass’d w/ APKD

- Cocaine or Amphetamine use

- Trauma

Clincial findings of hemmorhage

- increased ICP, finding is papilledema

- “worst Headache of my life!!”

- LOC, vomiting, neck stiffness, confusion, stupor, coma

- Increased BP, T

Work up

- CT/MRI

- LP (increased opening pressure, pleocytosis w/ xanthrochromia post hemorrhage)

- ECG short PR interval w/tall inverted T waves

- Arteriography

- Nimodipine (decreases cerebral vasospasm but can cause bradycardia)

- Surgical clipping or wrapping/ En Bloc resection of AVM if accessible

- Bedrest w/ head up, manage pain w/ analgesics NOT Aspirin

- Watch IV fluids to make sure they aren’t increasing ICP

Cerebral Infarcts

- 1-4 hrs no changes possibly detected by MRI

- detectable at 12 hrs w/ softening of the gray matter seen on MRI swelling, dead red neurons + PMN more apparent swelling 48-72 hrs ** may cause herniation WATCH DAY 3 MRI/CT visible most likely

- 2-6 days macrophages are the predominate response w/ maximal edema achieved

- signs of edema are altered conciousness, projectile vomiting, pupillary changes

- 1-3 wks reactive astrocytes, small BV perfusion, beginning of liquifaction w/ macrophages

- months to years resolution via liquifaction, cystic degeneration or cavitation w/ astrocytes around the edge, or glial formation

Work up for Ischemic Stroke

- ECG to look for cardiac source MI or A- fib

- CT or MRI to look for infarct or hemorrhage and R/O lesions or tumors

- LP to r/o SAH

- Carotid Doppler Ultrasound study

- Echocardiogram for embolic source

- EEG to distinguish seizure vs. TIA or Lacunar vs. cortical

- MR Angiography

Classic Carotid Emboli= ipsilateral blindness (amarousis fugax approx 10 mins), CL hemiparesis

Classic Vertebrobasilar= drop attacks, bilateral blindness, confusion, vertigo

Watershed infarcts secondary to hypoperfusion near MCA-ACA border; with reperfusion can be bilateral and/or hemorrhagic

Hemorrhage can be intraparenchymal, subarachnoid, or mixed usually due to vascular damage

VS. Hemorrhagic infarct- a white infarct that has been reperfused, looks like petechial hemorrhages

Pathogenesis of Atherosclerosis

- development of focal area of endothelial damage with increased permeability to lipid insudation, monocyte and platelet adherence

- intimal aggregate of monocytes and macrophages

- chemotactic and growth factors causing smooth muscle (intimal) proliferation

- macrophages and smooth muscle cells engulf lipids forming “foam cells”

- surface plaque can ulcerate, break off and lead to embolus and thrombotic infarction

DDx of Intercranial Hemorhages

Hypertensive hemorhages- intraparenchymal bleeds in regions served by perforator arteries caused by rupture of charcot-bouchard microaneurysm most frequently in basal ganglia, pons, cerebellum, 40% mortality, with resolution there may be some restoration of function

Gross Pathology

- expansion of affected area

- flattening of the gyri

- herniation, midbrain displacement

- ventricular distortion

Berry Aneurysms- bleeding into subarachnoid space occurs at the bifurcation of cerebral arteries or at sites of media weakness, rupture can be ass’d w/ acute increased ICP, common in APKD, fibromuscular dysplasia, AVM, but MOST are sporadic.

SX: sudden severe occipital HA, rapid LOC w/ 50% of patients dying

Other complications: infarction, hydrocephalus, herniation, brainstem hemorrhage, arterial vasospasm

AVM- 1% of intercranial tumors can rupture and bleed into grey matter of brain (setting up a seizure foci) or subarachnoid space, tortuous tangled vessels of various sizes, 90% are w/in the cerebral hemispheres, most common bleeds between the ages of 10-30 Males 2: 1 females

SX: Intercerebral hemorrhage W/ seizures

Supratentorial Hemorrhages- lead to progressive hemiplegia

Posterior Fossa/ Cerebellar Hematomas- lead to intractable vomiting, increased ICP, herniation, & coma

Epidural Hematoma- trauma, middle menigeal artery, lense shaped on CT that does not cross midline

Subdural Hematoma- in young and elderly, tearing of the bridging veins, cresent shape and can cross cranial sutures, shifting midline structures

Unit Two

CT scan- Indicated in a stroke > 48 hrs or early to R/O acute bleed, tumor, trauma, dementia, cerebral hemorrhage

- detection of structural abnormalities

- speedy

- good for evaluating progressive neurological disease or focal deficits due to strxl lesion

- contrast improves detection of tumors & abscesses, AVM, chronic subdural hematomas

MRI- Indicated in early stroke (w/1st day), hematoma, tumor, trauma (parenchymal pathology), dementia, MS or demyelinating diseases, and infections

- better contrast between gray and white matter

- better visualization of the posterior fossa and spinal cord

- better detection of MS plaques or seizure foci

- gadolinium enhancement (contrast) can separate small tumors vs. edema, identify leptomeningio disease, and integrity of the BBB

- Superior to CT for MS, early stroke, tumors at vertex, posterior fossa, and acoustic neuromas

Arteriography

- for visualizing intercranial circulation

- catherization into femoral or brachial a. to a major cervical vessel w/ contrast to visualize cerebral vessels

- risks 1% morbidity/mortality and considerable radiation exposure

MR Angiography (MRA)

- for visualizing carotids, and proximal areas of intercranial circulation where flow is fast

- screening tests for stenosis, plaques, & venous sinus occlusion

- if more than 70% consider carotid endarterectomy

EEG

- non invasive recording of electrical activity of brain w/leads placed on scalp

- for evaluation of epilepsy, abnl rhythmic electrocerebral activity of abrupt onset and termination a negative EEG can not R/O epilepsy not matter how many days it is followed

- for classification of seizures disorder to determine type for proper anticonvulsant therapy

- for assessment and prognosis of seizures & management of status epilepticus

- detection of structural brain lesions (foci)

- diagnosis of neurological diseases, i.e. HSV encephalitis repetitive slow wave complexes & CJK/SSPE w/ presence of periodic complexes

- evaluation of alterations of conscisouness, details of the level of coma

Evoked Potentials

- potentials evoked by non invasive stimulation of specific afferent pathways to monitor functional activity of the pathway

- common used in detection of MS lesions and monitoring functional status, spinocerebellar degeneration, familial spastic paraplegia, Vit E or Vit B12 def’y, assessment following CNS trauma or hypoxia, intraoperative monitoring, evaluation of auditory and visual

- Types:

- Visual w/ checkboard pattern stimulation recording potential from mid occipital region the clinical importance is the P100 response.

- Auditory – monoaural stimulation w/ repetitive clicks elicites BS evoked potential recorded at the vertex of the scalp, senses potential in the first 10 ms, check for latency or increased intervals

- Somatosensory- stimulation of peripheral nerves recorded at spine or scalp

EMG

- assesses regional muscle activity via an electrode placed in muscle in passive and active states

muscle activity at rest (passive state)

- relaxed muscle, normally no spontaneous activity except at NMJ

- fibrillation potentials = + sharp waves, deinnervated muscle seen in myopathic disease such as inflammatory myositis and poliomyositis, not seen on PEX

- fasiculation potential= characteristic of neuropathic disease, especially ALS

- myotonic discharge=myotonic dystrophy, congenital myotonia, and occasionally polio

muscle in active state

- myopathic disease= short, small duration, polyphasic motor units in affected (proximal) muscles

- neuropathic disease= loss of motor units w/ decreased # of activated fiber with and increased rate of firing (in distal muscles)

secondary myopathies common w/ statins & steroidal therapy

Poliomyositis is always Asymmetrical

Dermatomyosistis in kids is usually rheumatological & in adults it is probably a paraneoplastic syndrome

Nerve Conduction Studies

Motor Nerve conduction studies & Sensory nerve conduction studies

- to comfirm the presence & extent of peripheral nerve damage

- Carpal tunnel syndrome Dx’d with EMG/NC studies- sensory and motor velocities slowed at rest w/ possible signs of chronic, partial denervation of the median suppplied muscles of the hand

Lumbar Puncture

- indicated for diagnosis of meningitis or other infective or inflammatory disorder such as SAH, hepatic encephalopathy, Guillan Barre, psuedotumor cerebri, & MS

- assessment of treatment response

- administeration of intrathecal meds or contrast

- to reduce ICP pressure (rarely done)

- contraindications: suspected intracranial mass lesion, local infection, coagulopathy, suspected spinal cord mass lesion, & a infarct w/ hydrocephalus

- procedure= position pt in lateral decubitus position w/ maximal back flexion, site is between L3-L4 or L4-L5 approximated by pt’s iliac crest, sterilized procedure w/ local anesthesia, bevel up, pop through tegmental flavum, enter subarachnoid space, measure opening pressure w/ stop cock. Pt may get post LP HA.

Clinical uses of Biopsies

Brain-

- primary tumors, metastatic tumors, HSV encephalitis, abscesses, CJD

Muscle

- to distinguish neurogenic from myogenic causes: myositis, polio, DMD, BMD, myopathies, & mitochondrial d/o

Nerve

- for metabolic storage diseases (Fabry’s, Tangier’s), infection (leprosy), inflammatory changes (vasculitis) or neoplastic involvement

Artery biopsy

- Temporal artery for Giant cell arteritis

Unit 3 Disorders of Consciousness

Seizures- excessive, oversynchronized discharges of cerebral neurons

Epilepsy- group of disorders characterized by recurrent seizures, episodic loss of consciousness, affecting 0.3% of population at one time in life. Possible etiologies include hereditary, perinatal injury, infection, post infarction stroke, and neoplasms (mass effect).

Benign Febrile Convulsions- 2-4% of children between 3 mo – 5yrs, on the 1st day of a non CNS febrile illness, lasts < 15 mins, non focal 2/3 have one seizure w/ < 10% having more than 3. Seizures occuring early in illness or w/ a family hx of seizures have a 90% risk of a recurrence w/in 2 years of the first seizure.

Generalized Seizures- (i.e. involving both hemispheres) & including absence seizure

- primary generalized are usually in people < 20 and do not have an associated aura

- tonic clonic- loss of consciouness, usually w/ a symptomatic aura or warning

- tonic phase is unconsciousness w/ 10 Hz or faster waves, tonic contraction of limbs 10-30 sec, extension of limbs, arching of body, crying, cyanosis, contraction of muscles of massication (toungue biting)

- clonic phase is alternating contraction /relaxation of muscle for 30-60 sec w/ slow waves & w/ return of ventilatory effort and clearing of cyanosis, frothy saliva, possible muscle flaccidity and incontinence.

- Recovery or post ictal phase, can have confusion and HA, > 30 mins at times, positive Babinski at times, Todd’s paralysis (transient unilateral weakness suggesting focal brain lesion)

Absence Seizures-

- genetic, always begin in childhood and usually end by age 20

- brief LOC w/ loss of postural tone

- subtle motor manifestations= eye blinking or head turning “automatisms”

- no post ictal phase, full orientation

- inducible by hyperventilation

- 3Hz/sec spike wave during seizure usually < 30 sec

Partial Epilepsy (i.e. focal)

Simple Partial

- begins w/ motor, sensory, or autonomic pheonomenon or aura (i.e. pallor, flushing, sweating, pupil dilation, piloerection, vomiting, and incontinence)

- psychiatric symptoms, dysphasia, distorted memory, affect disturbance, cognitive defecits, labored thought processes, & hallucinations w/o LOC

Complex Partial

- temporal lobe or psychomotor episodes of impaired conscionnesss BUT NOT LOC

- arises mostly from medial temporal or medial frontal ; possible HC formation

- symptoms epigastric sensations, fear, déjà vu, olfactory hallucinations, before impaired consciousnesss

- usually < 30 min w/ characteristic coordinated motor activities “automatisim” orobuccolingual movements, pupillary dilation or salivation.

- Secondary generalization may occur if motor area is involved it will be a tonic-clonic

Systemic Diseases that predispose to seizures**know these

- metabolic= hypoglycemia, hyponatremia, hypocalcemia

- HTN, hepatic encephalopathy, uremia, porphyia

- Drug overdose/withdrawal

- Eclampsia/hyperthermia

Syncope

- LOC due to decreased blood flow to brain, light headedness, dimming of vision, usually 7mm non reactive

- Brainstem at the midbrain level= Fixed mid sized pupils =5 mm

- Opoid OD, focal pontine, Organophosphates, Neurosyphillis= Pinpoint 1- 1.5 mm

- Midbrain structural lesion, CN III =Anisoconia, asymmetric pupils

Oculocephalic reflex “doll head” & Oculovestibular reflex (caloric) normal movement’s with intact brainstem fxn

Normal movements COWS ( cold opposite warm same)

Abnormal movements downward deviation w/ cold water= sedative OD

NO OVR = structural lesion at Pons

ABNL OVR = CNIII or midbrain lesion

Motor responses to Pain

Decorticate response- subcortical lesion involves the thalamus, elbow flexed, legs extended

Decerebrate response- midbrain lesion elbow & forearm extension, leg extension

Irreversible Loss of Brainstem Function:

- apneic coma

- PCO2 > 60

- Negative corneal, pupillary, gag, negative OVR/OCR, with + Doll’s

- W/o underlying metabolic or drug induced etiology

Unit 4 Headache, Neck & Back Pain

Tension HA- usually after age 20 female > male, non throbbing bilateral occipital head pain “like a tight band around head”, not ass’d with N/V, prodromes, or auras. Chronic, frequent, sometimes involving tension in scalp or neck muscles, and may overlap with migraine type HA

RX: ASA, NSAIDs, ergot derivatives,

Prophylaxis: relaxation, PT, psychotherapy or TCA, SSRI’s, propranol

Migraine HA- early onset, females> males or + Family Hx, pulsatile unilateral pain often ass’d with N/V, photophobia, and aura (intracranial vasoconstriction) vs HA (extracranial vasodilation), auras can be visual alterations such as hemanopic field defects, scotomas, scinnilations. Diagnostic Test: compression of ipsilateral carotid or superficial temporal artery may decrease severity of HA. Precipitating factors tyramine, nitrites, phenyethylamine, MSG, fasting, caffeine, emotion, menses, OCPs, nitroglycerin, bright lights.

RX: NSAIDS, ergot derivatives

Prophylaxis: propanol, TCA, methylsergide, CCB

Cluster HA- Men> women, > 25 year age @ onset, 10 mins-2hrs duration, Awakens patient at night, burning sensation lateral aspect of nose, pressure behind eye, lacrimation, nasal d/c, & Horner’s are common w/these. Precipitated by Etoh and vasodilating drugs, smoking.

Abortive: Sumatriptan SQ, 100% O2, dihydroergotamine SQ

RX for Chronicity: Prednisone, Indomethacin

Prophylaxis: ergotomine, methysergide, CCB

Trigeminal Neuralgia (Tic douloureux)

- facial pain syndrome of CN V roots possibly due to arterial compression of nerve roots

- V2 V3 unilaterally lighting like jabs of pain, spontaneously cease w/ sensory triggers on face by touch, cold, wind, talking or chewing

- Simillar pain w/ MS or brainstem tumors possible

- RX: Tegretol or Posterior Fossa decompression surgery

Temporal Arteritis

- subacute granulomatous inflammation of temporal or vertebral artery

- HA, severe boring temporal face pain, > women over 50, uni or bilateral

- 50% of untreated pt will develop blindness due to opthalmic artery involvement

- DX w/ biopsy, ESR > 100

- RX: prednisone for 1-2 years

Cervical Spondylosis

- can include neck stiffness, pain in arms, segmental motor/sensory deficit in arms, UMN in legs, cervical disc degeneration and herniation, secondary ossification, and osteophytic outgrowths

- can involve > 1 nerve root & be uni or bilateral

- presents w/ neck pain, limited head movement, occipital HA, radicular pain, sensory change in arms/legs

- C5, C6 commonly involved

- DX: Xray, myelography, CT/MRI

- DDX: myelopathy of MS, motor neuron disease, SCD, cord tumor, syringomyelia, hereditary spastic paraplegia

- RX: cervical collar, surgery

Radicular Syndrome

- localized to one or more nerve roots (segmental paresthesias & numbness & weakness & reflex changes)

- exacerbated by coughing, sneezing, or other increases in ICP

C5 C6 Radiculopathy

- weakness in deltoid, supra/infraspinatus, biceps, brachioradialis

- pain & sensory loss in shoulder, outer arm and forearm

- reflexes : decreased biceps and brachioradialis

- ass’d myelopathy UMN weakness in 1 or both legs, change in tone or reflex, PC or STT sensory defects

L5 S1 Radiculopathy

- weakness dorsiflexion of foot, toes, eversion, plantar flexion,

- Reflexes : decreased ankle jerk

- Spine movement restricted w/ local back tenderness, decreased straight leg raising

Low Back Pain

Trauma

-Musculoskeletal pain w/ lumbar muscle spasms, decreased spinal movement, DX: Xray to r/o vertebral fractures RX: bed rest, local heat, NSAIDs, and muscle relaxants

Prolapsed Lumbar intervertebral disk

- most common L5-S1 and L4-L5

- usually follows normal activity or strain, can be related to trauma

- can cause radicular pain, segmental motor/senosory deficits, & sphincter disturbances

- pain reproducible upon percussion over spine & passive straight leg raising

- Pelvic exam, rectal exam, & Xray to r/o other diagnosis

- RX: analgesics, diazepam, bedrest w/ PT, if not fixed consider CT/surgery, etc

Lumbar Osteoarthropathy

- later in life an increase in activity leads to pain

- mild Sx Tx w/ surgical corsett & severe treat w/ surgery

- can present as root/cord pain, spinal stenosis (intermittant claudication of the cord or cauda equina) requiring surgical compression

Ankylosing Spondylitis

- backache and stiffness, progressive limitation of movement, young men HLA B27

- RX: Pain – ASA, indomethacin, PT, postural excercises

Neoplasms

- suspect if symptoms are persistent and worsen despite bedrest

- MRI to r/o mets, cord compression and cauda equina

Infectious

- TB, pyogenic, cocci, of the vertebra or disc

- LABS: increased WBC’s, increased ESR

- RX: surgical debridment, long term (IV) antimicrobials, drainage

Osteoporosis

- vertebral compression fractures

- RX: NSAIDS, adequate activity, diet, HRT for post menopausal women

Paget’s disease of the spine

- increased alk phosphatase w/ a NL Ca++ and NL PO4

- urine hydroxyproline and urine Ca++ increase w/ active disease

- Xray show increased density and expansion of bone, possible fissure fractures

- RX: high Protein diet, Ca++, Vit C, Vit D, Anabolic hormones, calcitonin, bisphosphates, mithromycin to decrease osteoclast activity.

Congenital Anomalies of Lower Back/Spine

Spinal Dysraphism

- defect in spinal fusion presenting w/ pain, neurologic deficit in 1 or both legs & sphincter disturbances

Spinal Stenosis

- can lead to syndrome of neurogenic claudication later in life

Arachnoiditis

- severe pain in back and legs due to inflammation and fibrosis of the arachnoid area

Referred Pain

- Disease of hip joints, back & thigh pain w/activity w/ limitation of movement, + Patrick’s sign (pain on external rotation), & Xrays show degeneration of hip joint

- Other causes: Triple A, Cardiac ischemia, visceral/retroperitoneal masses, GU disease

Unit 5 Movement disorders

Postural tremor- tremor w/ sustained posture continuing w/ movement w/o increasing in severity

- physiological 8-12 Hz commonly w/outstretched hand

- enhanced physiological – as above but worsened w/ stress, anxiety, thyroidtoxicosis

- Familial Benign essential tremor- AD inheritance involving 1 or both hands, head or voice w/sparing of legs, medically treated w/ beta blocker Propanolol or anticonvul. Primidone

- Drug induced, metabolic d/o (Wilson’s) or cerebellar d/o also produce tremor

Intentional tremor- tremor w/ activity, due to lesion in SCP or as a drug toxicity and can result in fxnl disability, treament is surgical procedure= sterotactic surgery of VLN of the thalamus;CEREBELLAR

Resting tremor- tremor w/o movement, characteristic of Parkinson’s but can be idiopathic or 2ndary to heavy metal poisoning (Pb+/Hg+) or Cu+ (Wilson’s disease); BASAL GANGLIA

Chorea- rapid, irregular, involuntary muscle jerks in various parts of body

Huntington’s disease- due to loss of neurons in caudate nucleus & putamen

Drug induced / SE of Dopa agonists- levadopa, antipsychotics, lithium, phenytoin, OCP’s

Vascular- vasculitis d/o, ischemic/hemorrhagic stroke, subdural hematoma

Static encephalopathy (Cerebral Palsy) secondary to anoxia, hemorrhage, trauma, or kernicterus

Dyskinesia- movement disorder that w/excessive abnl, involuntary movements i.e. tremors, chorea, hemiballismus, myoclonus, dystonia, tic, or athetosis w/ various etiologics including hereditary, vascular, or drug induced.

Isolated Focal dystonias- often treated with Low dose Levadopa, trihexphenidyl (anti cholinergic Artane), diazepam, baclofen or tegretol or surgically w/ stereotactic thalamotomy esp. w/ unilateral limb dystonia

1. Blepharospasm- involuntary closure of eyelids

2. Oromandibular dystonia

3. Torticollis- neck twisting, tx as above w/ botox injections possible CNXI sectioning

4. Writers cramp- drugs not very therapeutic, alternating hand therapy, possibly botox

Important points in evaluation of Movement disorders

Age & Mode of Onset: Infancy think birth trauma, kernicterus, anoxic damage or CP, inherited d/o

Childhood common for tic development, Tourette’s

Abrupt onset think drug induced or ischemic event

Slow onset think degenerative disease

Drug & Medical Hx: anti psychotics(phenothiazine/haldol) commonly cause tardive dyskinesia

Levadopa (dopaminergic agonists) can cause chorea

Lithium, TCA, valproic acid (depakote), bronchodilators can cause tremor

SLE, Thyrotoxicosis can induce chorea

AIDS can cause tremor, chorea, dystonia, hemiballismus

Family Hx: History of demyelinating d/o

Huntington’s disease

Idiopathic Parkinson’s Disease

- loss of pigementation (cells) or presence of intracytoplasmic Lewy bodies in the substantia nigra or other brain stem centers such as the globus pallidus, putamen, spinal cord or sympathetic ganglia

- imbalance between dopamine and Ach allows excess Ach to excite the inhibitory (GABA) release in the globus pallidus inhibiting the thalamus and cortex causing bradykinesia

5 Features of PD

- bradykinesia, rigidity, resting tremor, masklike facies, stooped posture w/shuffling gait

- dx is clinical

Mainstay Treatment of Parkinson’s disease

1. Early PD- no drug Rx

2. Progressive/symptomatic Rx

Levadopa/Carbidopa (Sinemet)- helpful for hypokinesia SE: N/V, hypotension, chorea, restlessness, confusion

Bromocriptine/& Pergolide (dopamine agonists)- ergot derivative, SE: psychiatric , Contraindication in pts w/ psychiatric d/o’s, MI, severe peripheral vascular disease, and active PUD

Amantadine- antiviral that increases the release of dopamine SE: restlessness, confusion, skin rash, edema, arhythmias

Anticholinergic (muscarinic agonists) – to alleviate tremor & rigidity

Ex’s hexyphenidyl, benztropine, procyclidine, phenadrine

SE’s dry mouth, urinary retention, constipation, defective pupillary accomodation & confusion

Parkinson Plus Syndromes

1. Shy-Drager Syndrome- Parkinson’s with ANS instability & other neurological involvement

2. Cortical Basal Ganglionic Degeneration- Parkinson’s w/ sensory loss, apraxia, focal reflex myoclonus w/asymmetric symptoms & no ANS instability or cognitive loss. Some Pt’s respond to sinemet (levidopa/carbidopa)

Tardive Dykinesia: Pt w/ abnormal choreoathetoid movements usually of face or mouth in adults or limbs in kids that can develop w/ long term Rx w/ antipsychotics (anti-dopaminergics)

Neuroleptic Malignant Syndrome: Patient develops rigidity, fever, altered mental status, and autonomic dysfunction over 1-3 days of antipsychotic treatments ESP’LLY WITH HALOPERIDOL, RX w/ dantrolene or manage symptoms with antipyretics, anticholinergics, antipsychotics lithium. DDx is infectious cause.

Unit 6 Motor and Sensory Deficits

Jaw - mandibular branch V3 3and pons

Biceps - Musculocutaneous and C5, C6

Brachioradialis – radial and C5, C6

Triceps- Radial and C7, C8

Finger- Median and C8, T1

Knee- Femoral and L3, L4

Ankle- Tibial and S1, S2

Pyramidal System from Motor cortex to IC- medullary pyramid decussation to descend in CL CST : lesions here will cause difficulty in fine motor movements. (UMN)

Extrapyramidal System- UMN input from Basal Ganglia or cerebellum : lesions here will have uncoordinated rate, rhythmic, and irregular amplitude movements

LMN motor fibers are cranial nerves or peripheral nerves

Appearance

Fasiculations are to LMN injuries (or anterior horn disease) as flexor and extensor spasms are to UMN.

Psuedohypertrophy is usually due to myopathies w/ replacement of muscle by adipose or other tissue.

Tone

Two types of hypertonia- Spasticity (clasp knife-velocity dependent w/ arm F>E and legs E>F most likely due to UMN damage) & rigidity (lead pipe- seen in passive movement affecting all muscles equally most likely due to BG/cerebellar lesion with cogwheeling superimposed )

Paratonia- seen in pts with frontal lobe or diffuse cerebellar disease where patient is unable to relax and will move limbs as examiner does.

Power (or weakness 0 –no contraction 1 flicker 2 active w/ gravity eliminated 3 active but no resistance 4 active to gravity and resistance & 5 nl)

UMN weakness pattern affects Extensors and Abductors in the ARMS and flexors in the legs.

LMN weakness pattern of supplied muscles at SC, root or peripheral level with distal > proximal

Proximal more than distal suggests a myopathy or NMJ

Variability in pattern of severity and distribution over time suggest Myasthenia gravis/Lambert Eaton syndrome.

Reflexes

Areflexia- root lesion, or peripheral neuropathy or early UMN disease, deep coma

Hypereflexia- (clonus)- rhythmic contractions in response to a stretch

Lateralized asymetrical of reflex responses (UMN disease)

Focal reflex deficits relating to root, plexus, or peripheral nerve lesions

Loss of distal DTR common in polyneuropathies & can be normal finding in elderly.

Gaits

Apraxic (dominant frontal lobe d/o- hydrocephalus, dementing processes, w/o weakness or coordination problems) usually can’t stand unsupported, feet glued to ground, unsteady short stepped gait

Hemiparetic w/ circumduction- unilateral lesion of CST pt tilts at waist and swings affected leg around

Scissoring- w/severe bilateral spasticity, legs brought stiffly forward & adducted w/ compensatory trunk movements (UMN sign)

Parkinsonian gait- small steps taken at a increased rate (festination), problems with starting and stopping, forward stooping, reduced arm swing

Steppage gait in persons w/ impaired sensation.

UMN SYNDROMES (damage to cortical areas where UMN orginate pyramidal & extrapyramidal CST)

- hypertonia “spasticity”

- arms flexed (w/ weakness of extensors and abductor) legs extended (w/ weaker flexors)

- hyperreflexia “sustained or nl clonus ”

- changes in gait

- + Babinski

Radiculopathy- lesions affecting specific nerve roots yielding characteristics changes in muscle innervated, location of sensory and reflex changes, dermatone distribution

Neuropathy- pathology of a nerve that causes weakness in innervated muscles (distal >proximal) w/ a decreased reflex or sensory change, fasiculations and muscle wasting can occur later in disease

Myelopathy- lesion affecting spinal cord

Myopathy- d/o affecting the muscles, usually presenting with weakness in proximal area vs. distal, w/ NO sensory loss, no sphincter disturbances, nl abdominal and plantar reflexes, with muscle wasting & decreased/absent reflexes only late in disease

ALS

- Pure motor syndrome mixed UMN & LMN signs w/ NO sensory deficit usually beginning in the limbs

- (40%) c/o weakness, cramping, twitching, stiffness, etc in upper extremites and (40%) c/o of the same type of stuff in lower extremities

- (20%) present with bulbar signs; which has a worse prognosis

- Progressive, fatal w/in 3-5 yrs, mainly due to respiratory problems, infections

- Clinical Dx., CSF is normal, no sensory change or mental dysfunction

- Rx of Symptoms

- Drooling- treat w/ anticholinergics

- Mobility – walkers, braces

- Contractures- prophylaxis PT

Myasthenia Gravis

- occurs at ANY age; Females > males & often ass’d w/ Thymomas, thyroidtoxicosis, or SLE

- characterized by fluctuating weakness of voluntary muscles w/ a predilection for extraocular muscles (bilateral opthalmoplegia) and other CN muscles (massetor, facial, laryngeal, pharyngeal) but can get limbs & diaphragm as well

- Pt present w/ ptosis, diplopia, difficulty swallowing or chewing, nasal voice, weak limbs and respiratory difficulties w/o sensory changes & w/o reflex changes

- autoimmune etiology- Ab’s against the Ach receptor at the postsynaptic NMJ causing a relapsing, remitting course

- can have insidious onset or become symptomatic in times of stress (infection, menses, pregnancy)

- drug induced onset w/quinine, quinidine, procainamide, propranolol, Lithium, phenytoin, PCN & aminoglycosides

- Dx is clinical and the edrophonium (anticholinesterase drug- Tensilon) test is useful to check for improvement right away. If improved, prescribe a longer acting pyridostigmine (Mestinon)

- Other Rx’s & tests

- Other anticholinesterase drugs(Mestinon), corticosteroids, azathioprine, plasmapharesis, IVIG

- CXR for thymomas, or thymic hyperplasia w/ thymectomy if indicated

- EMG

Symmetric Polyneuropathies:

Guillen – Barre Syndrome-

- weakness usually begins in legs, may have sensory and autonomic complaints

- often follows infective illnesses, innoculations, or surgical procedures. May be associated with camplobacter jejuni

- speed and progression variable but can be fatal

- Dx: acute onset w/progressive weakness of > one limb up to 4wks, symmetrical, distal areflexia w/ proximal hyporeflexia, mild sensory changes, CN VII involvement, autonomic dysfunction, with recovery beginning 4wks after onset.

- Tests: CSF w/ increased protein BUT NO Cellls; EMG shows slowing

- Rx’s: plasmaphoresis, IVIG (400mg/kg/d X 5d)

- Prognosis: Sx’s decrease after 4 weeks, 75% recover, 25% have neurodeficits w/ 5% dying acutely

Critical Illness Polyneuropathy-

- pt w/ sepsis or multi-system failure

- wasting of extremities w/ absent DTR’s and sensory changes

Diptheria Polyneuritis-

- neurotoxin produced by C. diptheria 2-3 wks following a palate or muscle infection

- within 4-5 weeks pupillary response impaired

- 1-3 months generalized sensory polyneuropathy; weakness proximally> distal

- Dx. CSF w/ increased protein, pleocytosis & EMG showing decreased conduction

- Rx Antitoxin + 2 weeks of PCN

Paralytic Shellfish Poisoning-

- poison blocks Na+ channels causing a rapidly progressing sensory & motor ascending paralysis

Porphyria-

- weakness usually proximal > distal; beginning in upper extremities and later involving leg and trunk

- decreased or absent DTR’s, sensory changes

- also causes abdominal pain, psychosis, fever/chills, HTN, hyponatremia, peripheral leukocytosis

Arsenic/ Thallium poisoning-

- sensorimotor polyneuropathy w/ GI disturbance

- distal > proximal & legs > arms

Organophosphate poisoning-

- cramping pain to distal numbness progressing to leg weakness & decreased DTR

- sensory changes 1st in legs then arms

Sensory Disorders

Complete loss of touch= anethesia of pain= analgesia

Partial loss of touch= hyesthesis of pain= hypalgesia

Increased sensitivity= hyperesthesia of pain= hyperalgesia/hyperpathia

Light touch= ipsilateral posterior columns (large fibers) to medial lemiscus of brain and on the contralateral anterior spinothalamic tract

Pinprick & Temperature= ipsilateral lateral spinothalamic tracts for 2-3 segments after entry and then cross over infront of central canal

Deep Pressure= evaluated on tendons (achilles)

Vibration= 128 Hz tuning fork on bony prominence, often decreased in elderly below the knees

Joint Position= passive movement of DIP joints thought to be carried in fibers also in the posterior columns

Complex sensory tests

Romberg’s Test= + w/ unsteadiness to evaluate impairment of joint sense in legs ( tabes dorsalis )

Two point discrimination= depends on intergration between the CNS & PNS and sensory compenent of the area stimulated. Threshold is 4mm on fingers to several cm on back. If PNS is intact w/decreased two point discrimination d/o in sensory cortex (CL parietal is suggested).

Graphesthesis, Stereognosis, & barognosis= able to identify a number traced in palm, shapes presented or pressures applied in area w/ normal sensation implies problem in CL parietal lobe.

Bilateral sensory discrimination (EDSS) = neglect/or decreased reaction to 1 side of stimulus suggest CL cerebral lesion

Sensory changes often develop well before onset of sensory signs and should not be considered psychogenic.

Peripheral nerve lesions:

Mononeuropathy= not usually noticed b/c of overlap but can be caused by compression lesions that preferentially involve the large touch fibers.

Polyneuropathy= symmetric and distally> proximally (glove and stocking sensory loss) feet before hands usually. EX’s Tangier disease (metabolic AR d/o w/ little or no HDL) tends to involve small pain & temperature fibers. Can also involve motor and reflex changes.

Distal axonpathies > myelinopathies ; including acute idiopathic GBS, CIDP, infective (AIDS, leprosy, diptheria, sarcoid), vasculitic ( PAN, RA, SLE), paraneoplastic syndromes, drug induced ( alcohol, INH, Dilantin, dapsone, vincristine, etc.), toxins, heavy metals (Pb), metabolic and nutritional neuropathies (DM, hypothyroid, uremia. B 12), entrapment, & hereditary neuropathies often w/ motor involvment (metachromatic leukodystrophy, Krabbe’s disease, Freidreich’s ataxia, Fabry’s, Refsum’s, etc)

Radicular lesion/Root involvement= impairment of a segemental cutaneous pattern

Root + pain suggests compression lesion

Loss of reflexes at specific root levels C56 bi/brachio C78 tri L34 knee and S1 ankle

W/ weaknes and muscle atrophy= suggest anterior root involvement

Central Cord lesion= syringomyelia, cord tumors, loss of P & T 1st, bilateral, segmental, and possibly w/ LMN weakness & pyramidal or PC deficit below level of lesion.

Anterolateral cord lesions= CL impairment of P & T below level of lesion, with sacral segments involved first with extramedullary lesions and can be spared in intrinsic or intramedullary lesions.

Anterior Cord lesions: CL impairment of P & T below level of lesion w/ LMN weakness of paralysis of muscles supplied by the segment due to damage of the anterior horns

Posterior Column Lesion= tight or bandlike sensation at level of the lesion w/ paresthesias (electrical shocks) shooting down extremities on neck flexion (Lhermitte’s sign). Loss of vibration and position sense w/ preservation of sensory.

Cord Hemisection or Brown-Sequard Syndrome= ipsilateral pyramidal deficit (UMN signs) w/ disturbed vibration and positional sense w/ CL loss of P & T 2-3 segments below the lesion

Crossed Sensory Deficit in Medullary lesions= P & T lost in CL limbs w/ Ipsilateral facial pain & T lost Wallenberg type

Brain Stem lesions= usually sensory combined w/ motor, cerebellar signs, & CN palsies

Pons= P&T lost in CL limbs and trunk

Medulla= P & T lost in CL limbs and trunk w/ ipsilateral P & T loss of facial sensation (spinal

Trigeminal nucleus) (Wallenberg syndrome)

Medial Lemiscus= CL loss of touch and propioception or w/ upper brainstem involvement

Complete CL loss of sensation

Thalamic lesions= CL impairment of all forms of sensation except in some cases where there is a painful sensation with any stimulation (Dejerine-Rousy syndrome) This can result from lesions of white matter of the parietal lobe or SC. (also know as thalamic pain syndrome).

Sensory Cortex Lesions: CL impairment of discrimatory sensory function usually worse distally more than proximally

Psychogenic disturbances could be conversion disorder & are usually cutaneous.

Non organic tends to surround an area defined by surface landmarks, sharp margins & commonly stops at midline

Organic does not usually extend to midline b/c of overlapping innervation and has surrounding areas of altered sensation.

Examples of types of Peripheral Neuropathies

Metabolic: DM, hypothyroidism , acromegaly, uremia, liver disease, vit B 12 def’y, hyponatremia, hypoglycemia

Infectious: AIDS, TB Leprosy #1, diptheria, sarcoidosis, sepsis, multiorgan failure

Hereditary: HMSPN, Friedreich’s ataxia, Familial Amyloidosis, Porphryia, Krabbe’s, Tangier’s

PN’s associated with PAIN: DM, Etoh, porphyria, Fabry’s, amyloidosis, HSN, paraneoplastic senosry neuropathy.

Entrapment Neuropathies:

Carpal Tunnel Syndrome: compression of median nerve @ writst

- often during pregnancy, or seen w/ trauma, degenerative arthritis, myexedema, acromegaly, amyloidosis

- pain & paraesthesia of median n. distribution of hand (thumb, index, middle, & lateral ½ of ring) can radiate up to neck and can keep pts awake at night, weakness and thenar atrophy may occur later

- Dx. Tinnel’s sign (pressing over median nerve) & Phalan’s sign (wrist flexion) both positive

- EMG

- Rx: corticosteroids, splint, surgery

Ulnar nerve palsy: compression @ elbow

- may result from ext.pressure, entrapment @ cubital tunnel or from cubitus valgus deformity

- nocturnal pain in little finger & ulnar border of hand +/- pain at elbow, possible weakness/sensory Sx’s

- Rx: surgical decompression

Peroneal Palsy: secondary to trauma or pressure @ head of fibula

- weakness or paralysis of foot and toe extension/foot eversion, impaired sensation over dorsum of foot & lower ant. Aspect of leg, ankle DTR’s are present

- Rx: supportive

Femoral Nerve Palsy: L3,L4 ass’n w/ DM, vascular ds bleeding diathesis, or retroperitoneal neoplasms

- weak quadriceps, decreased patellar reflex, sensory changes of ant/med aspects of leg

- Rx: treat underlying disease

Herpes Zoster-

- viral d/o that causes an inflammatory rxn in dorsal root ganglion or CN nuclei, in the affected root, or in nerve itself, can also be in CSF

- intial c/o burning or shooting pain w/in dermatome with rash 2-5 days later, rash resolves with scars and possibly post herpectic neuralgia

- common sites: thoracic dermatome, V1 of CNV which may lead to corneal scarring and anesthesia

- Ramsey Hunt syndrome CNVII palsy w/ herpectic eruptions in ear, palate, pharynx and nose

- Rx: Acyclovir or steroids for Sx’s and tegretol 1200 mg/d, phenytoin 300mg/day, and amitryptiline 10-100 mg q hs.

Syringomyelia-

Communicating ( hydrodynamic d/o of CSF pathways) opening between central cord and cavity

Non Communication- cystic dilation of cord not communicating with CSF pathways

- severity is site dependent, mainly w/ impaired P & T at that level with intact light touch

- sensory loss commonly seen by presence of painless skin ulcers, neuropathic joints, edema, scars, etc

- muscle wasting @ level of lesion with Extrapyramidal signs and sphincter disturbance possible

- pyramidal lesion w/ gliosis or compression of CST

- scoliosis is common

- decreased DTR below lesion and Increase above lesion

Friedrich’s Ataxia- idiopathic degenerating disease of cerebellar ataxia

- AR chrom 19 begin’s in childhood

- Demyelination of Spinal cerebellar tract, posterior column, dorsal roots and neurons in clark’s nucleus

- Large axons of periferal nerves as well as cell bodies of sensory nerves, and dorsal root ganglion

- Sx’s: Gait ataxia, progression to involve all limbs, dsyarthias, w/2ndary features impaired flexor /extensory response, pes cavus, scoliosis, and cardiomyopathy.

Unit 7 Multiple Sclerosis

CNS demyelination Signs in MS:

- Demyelination slows AP’s or blocks them and effects heavily myelinated tracts i.e. optic nerves, posterior columns, corpus callosum, brainstem pathways, and spinal cord.

- Can present w/focal weakness, numbness, tingling or unsteadiness in a limb, sudden loss or blurring of vision in 1eye (optic neuritis), diplopia, disequilibrium, or bladder fxn disturbance. Some patients present with mainly UMN sx’s as acute to gradual spastic paraparesis w/ sensory deficit.

- Mechanism considered to be autoimmune, with hypersensitive T cell (or IgG’s) attacking myelin & CNS myelin producing cells oligodendrocytes, HLA DR2 genetic relation, women > men, ages 20-40, and increased exacerbations with heat which sensitizes the Na+ channels that are responsible for AP conduction

- Impaired/blocked conduction can result in gliosis

6 common clinical presentations of MS

1. optic neuritis- tested w/ swinging light test shows Marcus Gunn pupil or pupil w/ afferent defect which will constrict less markedly w/ direct illumination and also have decrease in color vision a sign of retinal involvement

2. INO- demyelination of the MLF clinically seen as MR palsy or diplopia sign of Brainstem disease and if not MS DDx includes brainstem encephalitis, tumor, syringomyelia, drug intoxication, Wernicke’s

3. Disequilibruim- posterior column (+ Romberg’s test)

4. Urinary incontinence or frequency, ANS dysfunction that causes spasms or retention

5. Focal weakness (bilateral paraparesis of both legs)

6. Paraesthesias- numbness or tingling or dyesthesias- painful sensation from a non painful stimulus allodynia

7. Spasticity- increased tone, clasp knife effect w/ velocity dep’t passive movement, UMN lesion

Clinical criteria for Dx of MS

1. 2 signs or symptoms separated in time and space

2. clinical picture w/ history and elimination of other possible cause for symptoms

3. MRI (T2 w/contrast) -w/ evidence of plaques (areas of demyelination in the white matter;commonly periventricular)

Paraclinical supporting tests

1. LP w/ CSF showing increased IgG; oligoclonal bands (+ in 90% of pts)

2. VEP, BAER, SSEP evoked potential showing slowing

Other conditions or DDx for MS

1. isolated Optic neuritis (70% will go on to develop MS later)

2. transverse myelitis (Brown Sequard Syndrome)

3. vasculitis (SLE, Sjorgens, etc)

4. AIDS

5. Lyme disease (bilateral Bells or CN VII palsy)

6. Inherited spinocerebellar degenerations (Freidrich’s Ataxia)

7. Adrenoleukodystrophy/Metachromatic leukodystrophy

8. CNS mass lesion (more likely if focal defecits clinically)

Prophalaxis drugs (ABC’s of MS)

1. Avonex (beta interferon 1a)

2. Betaseron (beta interferon 1b)

3. Copaxone (glatiramir acetate) alters or interferes w/ T cell response to myelin w/ SE of flu like syndrome, increased spasticity & side reactions w/ betaseron

Rx for acute exacerbations

IV methylprednisolone (Solu-Medrol) 500-1000 mg/d, IVIG, plasmaphoresis

Management of MS

ACTH- 80 U IM x 1wk, tapered for 2-3 wks

Steroids: Methylprednisolone 15-20 mg/kg/d IV x 3days, taper 2 wk Prednisone 60-80 mg/day PO x 1 wk, taper 2 wks Dexamethasone 16 mg/d x 1wk

Immunosuppressants: Cyclophosphamide, azathioprine

IVIG, plasmapharesis

PT/OT & speech therapy

Spasticity- Baclofen (GABA agonist), Tizanidene alpha agonist(zanaflex), Dantrolene, Diazepam

Neuropathic Pain- TCA amitriptyline (elavil), or anti-convulsants gabapentin (neurontin), dilantin, tegretol

Fatigue- pemoline (cylert), amantadine (Dopa agonist), fluoxetine

Unit VIII DEMENTIA

Dementia- progressive deterioration in mental function that interferes w/ ADL’s

Need 2 or more of the following:

-Defecits of memory, language, visualspatial skills, calculation, apraxia, agnosia, abstract reasoning, behavioral component

Persistance of deficits is the differentiating feature from delirium.

Evaluation duration, progression, risk factors, ass’d neurological findings, Hx of psychiatric disease, head injury, or FHx of neuro/psychiatric disease

DX work up: CT/MRI, SPECT/PET, EEG, LP, metabolic panel w/ CBC

Alzheimer’s- most commonly diagnosed form of dementia

- risk factors= age, FHx, head trauma w/ LOC

- present w/ loss of ST memory, language, orientation, & judgement

- progresses over 2-20 year period ultimately involving all functioning

- pathological dx senile (amyloid) plaques, NF (tau microtubule protein) tangles, neuronal degeneration

- association cortex in parietal, temporal and frontal lobes particularly affected

- loss of cholinergic activity in forebrain: nucleus basalis of Meynert

- dx is one of exclusion, with diffuse cerebral atrophy possibly seen on CT/MRI

- PET scan may show regional hypoperfusion in parietaltemporal region, EEG w/ slowing

- Apo E4 Genotype testing & CSF levels of high Tau and lowAmyloid have poorer prognosis

- RX: acetylcholinesterase inhibitors (Cognex & Aricept)

- Symptomatic treatments: LD benzo’s, neuroleptics, AD’s

- Genes involved in SDAT chrom 21 amyloid, chrom 14 is presenilin, chrom 1 is presenilin 2, 19 is Apo E w/ E4 homozygotes at highest risk , Apo E2 may have protective benefit in caucasians

Multi Infaract Dementia

- elements of both cortical and subcortical dementia

- stepwise progression, psuedobulbar effect w/ impossible localization of a single focal lesion

- DX: CT can miss small infarcts & MRI is more sensitive

- Non invasive or invasive vascular studies are useful

- RX: antiplatelets, anticoag, decrease risk factors

Infectious Dementia

- resulting from meningitis or encephalitis

- general paresis of the insane “treponemen pallidum” neurosphyllis delayed onset up to 30 yr, RX w/ PCN

- chronic meningitis agents: syphillis, crytococcus, TB, borellia, other fungal/ parasites possibly presenting w/ hydrocephalus

- AIDS dementia complex – HIV 1 presents w/ psychomotor retardation progressing to global dementia consider concurrent infections; cryto, PML

- Herpes Zoster Encephalitis- diffuse slowing on EEG

- CJD disease w/ characteristic EEG pattern of periodic complexes

- DX : RPR, VDRL-ABS, lyme serology, MRI/CT, CSF culture

- RX: acyclovir for herpes, Ab’s

Normal pressure Hydrocephalus (NPH)

- Communicating & non communication Hydrocephalus are both able to produce dementia

- Nl CSF pressure 100-200 mm H20

- NPH can be caused by SAH, CSF infections or idiopathic

- Present w/ ataxia, urinary incontinence, and dementia

- DX: CT/MRI showing dilation of ventricles, or radionucleoside cisternography

- RX: ventricular shunting improves 50% of cases

Dementia in Extra Pyramidal Syndromes (UMN Globus pallidus & cerebellum)

Parkinson’s -- can present w/ tremor, cog-wheel rigidity, bradykinesia, shuffling gait, masked facies

RX: sinement, amantadine, bromocriptine, selegelline (MAO B inhibitor)

Diffuse Lewy Body disease

Huntingtons- choreoathetosis w/ positive FHx

RX: anti dopaminergic haloperidol & risperidone (antipsychotics)

Wilsons- deposition of Cu++ seen on CT/MRI, genetic testing, serum copper levels

Progressive Supranuclear palsy- paralysis of vertical gaze and axial rigidity

Corticalbasilar degeneration- apraxia & alien limb phenomenon

Random questions

Which extrapyramidal syndrome presents with dementia, paralysis of vertical gaze and axial rigidity?

Progressive supranuclear palsy (PSP)

Which extrapyramidal syndrome that has dementia, choreoathetosis, can be sometimes diagnosed with MRI, and whose motor symptoms often respond to anti-dopaminergic agents such as Haloperidol and risperidone?

Huntingtons disease, AD genetic disease, chromosome 4, atrophy of caudate nucleus

Which disease of dementia has the histopathological change of neuronal granulovacuolar degeneration, especially in the nucleus basalis of Meynert & favors the association cortex in the parietal, temporal, and frontal lobes and can be diagnosed sometimes on a volumetric analysis of the hippocampal/temporal regions?

Alzheimer’s Disease, also with senile plaques and NF tangles but too obvious to include in the question.

Associated with chromosome 1 (presenilin 2), 14 (presenilen 1), chromosome 19 (Apo E) and chromosome 21 (APP-amyloid precursor protein).

Which disease can be symptomatically treated with Cognex and Aricept, along with low dose benzodiazepines, AD’S trazodone, or neuroleptics for behavioral changes or paranoia?

Alzheimers

Which allele is associated with higher LDL and familial and sporadic AD?

Apo E4, and combined with head injury it may increase the risk ten fold

Which protein that is associated with microtubules is elevated in AD w/ a 90% specificity?

Tau

Is beta amyloid, the protein ass’d with neuritic plaques increased/decreased or nl in the CSF of pt with AD?

They are decreased in pt’s with AD and if they are elevated in a patients CSF it can rule out AD.

High Tau > 420 pg/ml and low Ab lateral ventricles > third ventricle> aqueduct of Sylvius

Medulloblastoma

- occur in the cerbellum w/in the 1st two decades of life

- dissseminate through CSF, neuroectodermal in origin

- highly malignant but radiosensitive

Meningioma

- most commonly appear in frontal hemispheres, near falx, the lesser wing of sphenoid, and near olfactory groove

- usually solitary but can be multiple if ass’d with NF Type 2

- whorled pattern on histology, psammoma bodies

Common Mets to Brain

Lung, Breast, Colon, Melanoma, & Renal

Psuedotumor Cerebri- obese females w/ increased ICP, taking OC’s, that present with throbbing headache, visual changes (peripheral blurring/decreased acuity), and have papilledema on exam.

- benign intracranial HTN w/ diffusely increased ICP

- often present w/diplopia with CNVI palsy

- females > males

- CT/MRI shows small ventricles

RX: decrease ICP w/ furosemide, CA inhibitors or surgical optic nerve sheath fenestration

For refractory cases prednisone can be used.

Major danger of not treating an elderly patient with steroids that presents w/ a throbbing, acute HA, jaw claudication, with a HX of Polymyalgia rheumatica, and a current ESR of 100 is loss of vision due to thrombosis of the opthalamic artery due to Temporal Arteritis.

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