Neuro Block

Alzheimers Disease

FTD

Lewy Body disease

PD

PSP progressive supranuclear palsy

ALS

Huntingtons

CJD

FTD , PD

LBD, PD

ALS

4, CAG repeat (polyglutamine)

5HT

Ach

DA

DA, Ach

Alzheimers Disease

LBD, PD

FDT

HD

midle meningeal artery

subdural hematoma

subdural Hematoma

diffuse axonal injury

subfalcine

transtentorial (uncal) herniation

Rarely, uncal herniation can compress the opposite cerebral peduncle against the tentorial edge, resulting in a hemiparesis that is ipsilateral to the mass lesion and herniated uncus

central herniation

tonsillar herniation

tonsillar

vasogenic result of breakdown of BBB (excitotoxicity glutamate and increase in intracellular Ca++), cytotoxic edema results from astrocytes inability to clear K+ from extracellular space (in case of excitotoxicity), and they swell, "clamp=off" capilaries, and cut down blood flow to brain.....

1- ABCs, 2- elevated head decreased venous congestion, 3- IV osmotic agents like mannitol (1g/Kg body weight), 4- ventral catheters drain and prevent hydrocephalus, 5- barb induced coma reduces metabolic demand/ free radicals

eye opening 1-4, best motor response 1-6, verbal 1-5

mild 13-15, moderate 9-12, severe 3-8

headache, dizziness, poor attention, inability to concentrate, memory problems, fatigue, irritability, depressed mood, intolerance of bright light or loud noise, and sleep disturbance.

Grade 1 – Confusion without amnesia or LOC, Grade 2 – Confusion and amnesia, Grade 3- LOC

second impact syndrome- after 1st concussion- bad news

CSF rhinorrhea, B/L periorbital haematomas, subconjunctival hemorrhage, bleeding from external aufitory meatus, CSF otorrhea, battle's sign, VII palsy

pupillary, corneal, gag, caloric,

II to III in the midbrain

V to VII in the PONS

IX to X in the medulla

VIII, VI, III Pons to midbrain

nl <15, moderate 15-20, severe >20

increased ICP, low MAP

B12 deficiency - subacute combined degeneration

JC virus- PML, HIV- myelin palor in AIDs Dementia Copmlex

measles, rubella, chicken pox infection

1.      SLE, Sjogren’s, Mixed Connective Tissue Disease, CNS Vasculitis

Genetic - Link to DR2, IL-7 and IL-2 receptor alpha chains

30+ days from CIS for RRMS

paresthesias (numbness and tingling), monocular loss of vision (optic or retrobulbar neuritis), gait problems, weakness, diplopia (double vision), Lhermitte's (paresthesias down spine with neck flexion), urinary urgency/frequency, constipation

early features + fatigue, sexual dysfunction, depression, cognitive dysfunction, pain, dysphagia; rarely, seizures and hearing loss

Protein usually Nl, may see mild elevation in protein (<110 mg/dL)

WBC usually Nl, may see mild elevation, lymphocytes (<40/mm3)

always normal

1.      Fatigue: Symmetrel (Amantadine) 100 mg b.i.d. - t.i.d.; Modafanil (Provigil) 100 mg b.i.d.; Prozac (Fluoxetine) 20 - 40 mg qam; Ritalin (Methylphenidate) 5 - 20 mg qd in divided doses; Dexedrine (Dextroamphetamine) 5 - 30 mg qd in divided doses

1.      Spasticity: Physical therapy; Baclofen (Lioresal) 10-30 mg t.i.d.; Zanaflex (Tizanidine) 4-8 mg t.i.d.; Neurontin (Gabapentin) 300-1300 mg t.i.d.; Dantrium (Dantrolene) 25 mg qd - t.i.d.; Botulinum toxin for focal tone/spasticity; intrathecal Baclofen pump in severe cases

1.      High-dose corticosteroids Solumedrol 1 gr IV qd x 5 d; +/- Prednisone taper

"Immunomodulation with ABC-R therapy - a. Betaseron (Interferon beta-1b) 8 M IU, SQ, qod approved 1993 b. Avonex (Interferon beta-1a) 30 mcg, IM, q week approved 1996 c. Copaxone (Glatiramer acetate) 20 mg SQ, qd approved 1997 d. Rebif (Interferon beta-1a) 44 mcg SQ 3x weekly, approved 2002

tysabri

broca's area

weirnicke's area

conduction aphasia- arcuate fasciculus

global aphasia- perisylvian area

motor prosody- inflection and emotion in speech

disinhibition- Disinhibition is a disorder of comportment, whereby a person can no longer adequately integrate limbic drives into an appropriate behavioral repertoire. Irritability, loss of empathy, impulsivity, hypersexuality, hyperphagia, and even violence can be sequelae.

loss of executive function- Executive function is an important domain that involves the capacity to plan, carry out, and monitor a sequential goal-directed action. One disabling deficit is perseveration, the failure to alter one’s actions in response to changing environmental stimuli.

apathy- Apathy is the loss of motivation, and more severe forms are known as abulia and akinetic mutism.

sensory aprosody, which means diminished ability to comprehend the emotional inflection of speech, and is due to a lesion in the right hemisphere analogue of Wernicke’s area.

new learning- HM = bilateral hippocampal resection= amnesia

The parietal lobes are prominently involved with tactile sensation, but also with visuospatial function, attention to the contralateral side of space, reading, writing, and calculations

left occipitotemporal lobe

right occipitotemporal lobe

bilateral occiptoparietal lesion

usually inheriteded, tremor with posture and action, alcohol helps,

primidone (antiepileptic), propranalol, topamax (antiepileptic)

thalamus, DBS, or thalomotamy

age of onset <16, >1 year duration, supressible, motor and vocal tics

ADHD, OCD, poor impulse control

athetosis

myoclonus

PD

7-10 years

progressive, A of Onset >50, impaired eye movements, cant look down. Falls in 1st year

multiple systems atrophy

Ischemic Stroke (often shortened to stroke, yes, just to confuse you) is an ischemic injury to the brain causing a persistent clinical deficit at 24 hours. Even mild residual deficits are classified as strokes. The severity of the deficit is not the determinant, only that the deficit is present at 24 hours.

Transient Ischemic Attacks (TIA) are ischemic neurological deficits that have completely resolved by 24 hours, regardless of their severity or relative duration (seconds or hours). Recent guidelines have proposed shortening the 24 hours to well within 1 hour since short TIAs, resolving within minutes, do not impart any permanent damage to the central nervous system, but a longer TIA is actually associated with cell death.

Age. The risk goes up about 10 times every twenty years of life.

Although women have a few special risk circumstances, the overwhelming effect of basic atherosclerosis makes men a significantly higher risk cohort. Women start to catch up after menopause.

HTN, lipid disease, smoking, homocysteinuria, obesity, DM, EtOH, inactivity

Cardiac. In addition to the more common causes of cardiac embolization (atrial fibrillation, CHF, and valvular disorders) the occasional patient with an atrial septal defect (PFO, for example) can allow an embolus to bypass the lung to the brain. Episodic arrhythmias (such as atrial fibrillation) can similarly lead to embolization in an otherwise normal heart. This can be from atherosclerosis, or from stimulants and hyperadernergic states of withdrawal. Intracardiac tumors (such as atrial myxoma) can also cause stroke. Atrial myxoma questions are popular, for no clear reason, on licensure or board exams.

Infectious endocarditis can lead to infected embolic stroke. This can create an infected, or mycotic aneurysm

firbromuscular dysplasia (FMD is associated with arterial dissection and with intracranial saccular aneurysms), moya-moya (Focal occlusion of the middle cerebral artery is an unusual cause of stroke. In children, this is typically non-atherosclerotic, and called Moya-Moya disease. Women in their 30’s and 40’s sometimes get this too, for unclear reasons. The pathology is intimal hyperplasia), arterial dissection (The occlusion or stenosis of the artery is typically well tolerated, but emboli from the injured artery can move into the distal artery territory and cause significant strokes. Therapy is with anticoagulation until the dissection heals, although intra-arterial stenting or surgery occasionally have a role.)

Familial deficiencies of blood components that help to prevent thrombosis such as Protein C, Protein S, Antithrombin (formerly Antithrombin III), Factor V Leiden, and Prothrombin Gene 20210, are associated with venous thrombosis largely, including CNS venous thrombosis.

Antiphospholipid antibodies cause a triad of spontaneous miscarriage, thrombocytopenia, and recurrent large vessel thrombosis, arterial or venous. Modern laboratory testing now identifies these antibodies in many patients. The mechanism appears to be an attack on the phospholipid membranes in the chorion, platelet, and endothelium, respectively. Treatment is directed at the individual problem, and can involve anticoagulation with warfarin.

Intraparenchymal Hemorrhages are often due to hypertension and age. They typically occur in the putaman, thalamus, pons and cerebellum deep gray matter. No further investigation is needed when typical hemorrhages occur in these areas. When in atypical locations, such as the deep white matter ("lobar hemorrhages") they can still be from age/HTN, but can also be from AVMs (or other vascular malformations), aneurysm, vasculitis, bleeding disorders, or even hemorrhage into tumors.

In amyloid angiopathy, patients get recurrent lobar hemorrhages that lead to progressive dementia and disability. These lobar hemorrhages can be curiously well tolerated in many. Amyloid deposition is found in the vessels (intramural). This disorder is different from systemic amyloidosis and the amyloid plaques of Alzheimer’s Disease

Ischemic stroke can be resuscitated with thrombolytic agents (i.e. tissue plasminogen activator or TPA) given IV or by IA catheter as long as you get this done within 3 hours, or 3-6 hours in some patients. Keeping fluids up, maximizing cardiac output, and resisting the temptation to lower blood pressure (i.e. stepping on the hose) help, too. Research into the use of mechanical IA techniques (angioplasty, stenting, etc.), hypothermia, and neuroprotective drugs continues.

For hemorrhages, surgical decompression is often needed immediately for subdural and epidural hemorrhages. Subarachnoid hemorrhage requires an angiogram to determine the cause of the bleeding, and thereafter may benefit from a surgery or other procedure (embolization, coiling, focused radiation, etc.). Intraparenchymal hemorrhages only sometimes need surgery. They can often be medically treated, or if severe, surgery has little benefit.

The single agents that reduce the long term risk of recurrent ischemic stroke are aspirin, 9 ticlopidine, clopidogrel, 10 and warfarin

Remember that these agents may require 5-7 days before effective platelet aggregation is achieved.

Warfarin is highly effective in primary prevention of stroke with atrial fibrillation 11. Most noticeably, the relative risk of stroke is reduced by about 70%, compared to 20-30% with antiplatelet agents. Similar robust benefit is seem in patients with mechanical heart valves treated with warfarin. Furthermore, the use of lower international normalized ratio (INR) targets for anticoagulation has resulted in lower bleeding complication rates then expected. Patients with previous history of embolization, hypertension, and heart failure are factors that identify the highest risk patients. The type of atrial fibrillation (paroxysmal or prolonged), and the duration of atrial fibrillation have no effect.

anticoagulation

antiplatelet

First, optimal control of blood pressure and other vascular risk factors will reduce the risk of virtually any intracranial hemorrhage. Second, some intracranial hemorrhages are caused by an initial ischemic event, followed by hemorrhagic transformation. If this is the mechanism, then an anti-platelet or anticoagulants will paradoxically help prevent recurrent hemorrhage.

important- high grade stenosis >70% = good outcomes with surgery. Improves with addition of medical therapy

In order to minimize iatrogenic intracerebral hemorrhage, TPA is contraindicated in patients with abnormal CT scans (indicating stroke has progressed beyond recovery), hypertension, coagulation difficulties, or a coma (indicating another diagnosis).

Ideally, the prehemorrhage blood pressure of the individual patient should guide therapy. This must be estimated in most cases. In general, systolic readings above 160 probably are associated with a higher risk of recurrent hemorrhage, but may be necessary for adequate brain perfusion.

Treatment with nimodipine helps reduce the amount of ischemic damage from vasospasm, a process caused by irritation of blood vessels by the blood in the subarachnoid space. After surgical obliteration of the bleeding source, hypervolemic hypertensive hemodilution (HHH) therapy can be used to reduce the effects of vasospasm.

Lumbar puncture is useful to exclude neurosyphilis and vasculitis. Otherwise, it is not a routine part of the evaluation of ischemic stroke. Perhaps the greatest utility of lumbar puncture is in the determination of subarachnoid hemorrhage, which can occur with a normal CT scan in two situations.

ASA

NMJ diseases, motor neuron disease, muscle disease

peripheral nerve, spinal root, plexus

ALS

muscle atrophy, cramps, fasciculations/muscle twitches (look at the tongue).

spastic tone, hyperactive tendon reflexes, pathological reflexes (Snout, Hoffman, Crossed Adductor, Babinski)

no bladder/bowel involvement. No sensory compaints(rare vibration in distal LE)

ALS

1/4 of these families see gene mutation on CHROMOSOME 21. Codes for an Superoxide Dismutase (SOD-1). SOD-1 plays a role in cell survival

IN NEWBORN OR NEONATE: See respiratory difficulty; floppy, hypotonic child, with death in many within one year (Werdnig-Hoffman). IN INFANTS: See slow milestones, continued hypotonia. IN CHILDHOOD: See loss of motor skills, easy fatigue and tripping. IN ADOLESCENT: Proximal arm and leg weakness(KugelbergWelander)

Chromosome 5 defect of Survival Motor Neuron Protein (SMN) (rarely in gene for NeuronalApoptotic Inhibiting Protein (NAIP). Autosomal recessive (1 in 4 for subsequent pregnancies)

entrapment at head of the fibula -Tendency to "stub toe" when walking or running due to weakness of foot dorsi-flexion, numbness over toes, top of foot, and lateral lower leg

entrapment at the elbow “Numbness” in the little and ring fingers- Examination reveals sensory loss over medial 4th and all of 5th Fingers, weak interossei.

entrapment at the wrist (carpel tunnel) Numbness in the thumb and index finger tips. Pain in hand, forearm particularly at night. Examination reveals sensory loss to pin over tips of thumb, index and middle finger

such as diabetes, hypothyroidism, collagenvascular disease (necrotizing vasculitis), leukemia, or amyloidosis.

AD- Patient has high arch in both feet, foot dorsi-flexion weakness , “glove and stocking sensory loss, absent deep tendon reflexes. Demyelinating disease- MP122 myelin protein

Patient complaints of numbness and tingling initially in feet, then hands, ascending. Progressive weakness of leg muscles, then hands and rarely facial paralysis . Breathing can be compromised early on . Nerve conductions: slow/demyelination. CSF = high protein, no cells, sugar normal. Treat with immune globulin (IVIG) or plasmapheresis. Steroids do not work. Pulmonary intervention as needed.

MULTIFOCAL MOTOR NEUROPATHY—Focal myelin loss due to unknown antibody attacking myelin—may be GM-1 Antibody against ganglioside in peripheral nerve. Clinically may see focal, distal muscle wasting and weakness with no sensory abnormalities. Purely motor weakness in the distribution of peripheral nerves. Nerve Conductions are characteristic with conduction block. Treatment is IVIG or cyclophosphamide IV.

PERIPHERAL POLYNEUROPATHY - distal "glove/stocking" sensory loss. Often burning/stinging distally. This is very common. AUTONOMIC NEUROPATHY - Diarrhea, postural hypotension, Tachycardia, dilated, flaccid bladder, impotence, Charcot joints (painless degeneration) and loss of sweating in hands and feet. MONONEUROPATHY Cranial nerve involvement (III & VI commonest), Entrapped nerve (median)

AXONAL NEUROPATHY = LOW AMPLITUDE COMPOUND MOTOR ACTION POTENTIAL, NORMAL CONDUCTION RATE DEMYELINATING NEUROPATHY = SLOW CONDUCTION

muscular dystrophy, inflammatory myopathies

myoclonic dystrophy, inclusion body myositis

metabolic myopathies, genetic myopathies

myasthenia gravis, oculopharyngeal dystrophy

X-linked- In Duchenne there is no Dystrophin; In Becker’s there is a low MW Dystrophin and/or a reduced percentage of Dystrophin

CK 5,000-20,000. biopsy,

perdnisone- slightly

FASCIO-SCAPULO-HUMORAL DYSTROPHY (fsh)

PROXIMAL WEAKNESS

inclusion body myositis

inflammatory myopathies/polymyositis (lupus, polyarteritis nodosum)

dermatomyositis - autoimmune

myotonic dystrophy, Autosomal dominant, 90% have chromosome 19 defect—too many CTG repeats in non coding region (intron).

oculopharyngeal dystrophy

Slurred speech, double vision, drooping lid(s), facial weakness, shortness of breath, neck muscle weakness, proximal arm/leg weakness, can progress to quadriplegia, respiratory arrest.

Acetylcholine receptor antibody titer elevated in 85%; alternatively a few have a different MuSK antibody, “Tensilon (edrophonium) test”—rapid (seconds) improvement in IV cholinesterase inhibitor.

Prednisone and/or Azathioprine to immunosuppress; thymectomy (usually) to remove presumed antigenic source

dark

white

T2, but dark CSF