17 - Toronto Notes 2011 - Nephrology

NP

Nephrology Katie Connolly, Melanie Ostrekher and EliAa Rennert-May, chapter editors Doreen Ezeife and Nigel Tan, associate editors Steven Wong, EBM editor Dr. Ramesh Prasad, Dr. Martin Sc:hreiber and Dr. Gemini Tanna, staff editors Basic Anatomy Review ................... 2 Anatomy of the Kidney Renal Structure and Function Renal Hemodynamics

Tubulointerstitial Disease ............... 27 Tubulointerstitial Nephritis (TIN) Acute Tubular Necrosis (ATN) Analgesic Nephropathies

Differential Diagnoses of Common Presentations . . . . . . . . . . . . . . . . . . . . . . . . . . 4 Azotemia Proteinuria Hematuria

Vascular Diseases of the Kidney .......... 30 Large Vessel Disease Small Vessel Disease

Assessment of Renal Function ............. 6 Measurement of Renal Function Urinalysis Urine Microscopy Urine Electrolytes Electrolyte Disorders. . . . . . . . . . . . . . . . . . . . . 9 Sodium Homeostasis Hyponatremia Hypernatremia Potassium Homeostasis Hypokalemia Hyperkalemia

Systemic Diseases and the Kidney ........ 32 Hypertension (HTN) Hypertensive Nephrosclerosis Renovascular Hypertension Renal Parenchymal Hypertension Multiple Myeloma Malignancy Diabetes and the Kidney ................ 34 Cystic Diseases of the Kidney ............ 36 Adult Polycystic Kidney Disease Medullary Sponge Kidney Autosomal Recessive Polycystic Kidney Disease Common Medications ••••••.••••••.•••• 38

Acid-Base Disorders .................... 16 Metabolic Acidosis Metabolic Alkalosis

Landmark Nephrology Trials •.••••••.•••• 39 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40

Renal Failure .......................... 19 Presentation of Renal Failure Acute Kidney Injury (AKI) ................ 20 Approach to AKI Chronic Kidney Disease (CKD) ••••••••••.• 21 Management of Chronic Kidney Disease Renal Replacement Therapy ............. 22 Dialysis Renal Transplantation Glomerular Disease .................... 23 Terminology of Glomerular Changes Presentation of Glomerular Disease Investigations for Glomerular Disease Secondary Causes of Glomerular Disease Infections and Glomerular Disease

Toronto Notes 2011

Nephrology NPI

1'oroDio

NPl Nephrology

2011

Basic Anatomy Review Anatomy of the Kidney















Renal Structure and Function The Nephron • basic structural and functional unit ofthe kidney, approximately I million per kidney • 2 main components: glomerulus and attached renal tubule (Figure I) • direction of blood flow: afferent arteriole -+ glomerular capillaries -+ efferent artuiale -+ vasa recta (the capil.laries surrounding the tubules) -+renal venules

0

Osmatic diuratics

( ) L.oapdiul'ltics

0

Q

liEzide diu191ics K+ Sparing diantics o.-.lingII!Dolloop ·. alllonil

I



1!1!

!:z: N1+ K•

i

i·;a 2

I I I

N1+-K- 2CI-

l

H,D -

Madula

J

0 [

z

li

I 0

1. N1p1Jnm Compa1ants

Tlble 1. MaJor Fu1dons of the Kidneys 1. Willa EKrltiGn

Ghmarular fi1nl1ion

Eiu:mian of nitragnJUs prDib:ls of pratailrnelllbalism

(urea, Cl)

Tubailr secretion Tub.-r calllxllism

Excretion af organic (!Ne) and Dfllllnic bases (Cr) llnllkdawn end ucratian af drugs {..tDalicl, diul'llicll) end peplida hlmlanes (mast pituillry harmCIIIIS, ilsUin.

Tubailr tllaCI and Wllllr rubsGiptilll Tubaar Kseaetion Tub.-r HaecratiCII HCO, synlheais ..:1 rBaa!ption Tubular Ca, Mg. P04 lnllspart

Clllllds vduma s1a1us and osmolar balance Clllllds pllillssium cai!CI!nlnllian Al:id-basa balance Al:id-basa balance .Min Ca. Ma-1'04 harnaastasis

Elytlqail!lil pracb:lian lcorttxl Vitamin Ddvation [25[0H)D 1,25(0HJDI Ranil praductian (JG applfiiUi)

....aod ............... Na IIKL18tian Ranil praductian Gluconeogereis tfmm lacbde, py!\MII8 end smila acids)

Red lilad eel proU;Iian

Calcium hiDII!IISialis

Allin VIICUI.-ra&i&Wlcellllaldastlmle sacratian Allin ECF Allin VIICUiar rnista1ce Glucose !llp!iY lllllintailed in prnlanged staiYIIIion

Buic Anatomy Review

'IbroDlo Nota 2011

Nephrology NP3

The Glomerulus • site where blood constituents are filtered through to the kidney tubules fur excretion or reabsorption • consistB of following cell types 1. capillary endotbelial cells and podocytes • support the glomerular basement membrane (GBM) and furm the plasma filtration apparatus 2.mesangialcells • have contractile properties and produce YllSoad:i.ve substances to help control blood flow 3. parietal epithelium • covers the interior of Bowman's capsule • filtration occurs aaoss the GBM Into Bowman's space (Figure 2) • filtration barrier: conaists ofcapillary endothelium, GBM. podocyte filtration alit& • particles are selectively filtered by size (60 kDI is NOT affected• Thus, edema is partly secondary to salt and Wlll8r l'lltllntion 2.Giom.-r • No111111lly, the 111ntion barrier is selectively penneable to SIZE {2-3 RBCs/HPF on microscopy

H1111aluria

•

I

•

...

-ve dipstick. no RBCI

+ve dipsticll.- +ve RBCs

+ve dipllick, no RBCI

• Pseudohematurill • Food (basts), dyes, madicati011 (rifampin)

[1rue hematuria) • Hemoglobin (hemolysis)

• Myoglobin (rhabdomyolysis)

"

Hematological • Coaguloplllhy, sickle cell

Prilllllry • • • • • •

I

..

GN Post-51rap. GN Rapidly-progressive GN nephritis (ICuta and chronic) Papillary necrosis lgA naphroplllhy

s

"

Urologic • Nephrolithiasis, 1rauma, tumour. pro.tatitis, urethritis • Dysuria or flank pain common • Isomorphic RBCs, no casts • Blood at beginning (lni!Yitis) or end [prostate, bladder) of stre1111

Second1ry

..

• Comective tissue diseases (CTDJ • Waganar's, Goodpastura's, SLE, Ct.Jrg-Stnluss, HSP • Infection • Pyelonephritis • Heneditary • AIport's, polycystic kidnsy disaasa (PCKD)

Figura 6. An Approach to Hamll'lllria

Investigations for Hematuria • Hx and Px: family history of nephrolithiasis, hearing loss (Alpert's), cerebral aneurysm (PCKD), diet, recent URTI, irritative and obstructive urinary symptoms (UTI) • urine R&M, C&S, urea, Cr • 24-hr urine stone workup: calcium, oxalate, citrate, magnesium, uric acid, cysteine • further workup (if casts and/or proteinuria): CBC, electrolytes, 24-hr urine protein and Cr, serology (.ANA, RF, C3, C4, p-ANC.A, c-.ANCA, .ASOT), abdo/pelvic ultrasound, cystoscopy ± urology consult

Assessment of Renal Function

,,



er-=cr_

[Cr),.,,.. x GFR = [CrJ..t..., x urin flow ms(ml/min) GFR - ICrJ.m. x urjne !low r11!: [Cr)-

.....

Measurement of Renal Function



,,t----------------.

There is an inverse relati011ship between sarum Cr concentration and CrCI at steady state.

• • • • • • •

Glomerular Filtration Rate (GFR) = rate of filtration of plasma by the glomeruli most renal functions decline in parallel with a decrease in GFR GFR is often estimated using serum creatinine concentrations [ Cr] creatinine (Cr) is a metabolite of creatine (intermediate in muscle energy metabolism) Cr is freely filtered at the glomerulus with no tubular reabsorption and minimal secretion (10%) rate of production determined by muscle mass Cr excreted= Cr filtered (at steady state)

Ways to Estimate GFR I. Calculate creatinine clearance (CrCl) • calculation provides reasonable estimate of GFR • measure plasma [Cr], 24-hr urine volume and urine [ Cr] • GFR= (urine [Cr] xurinevolumeinmL)/(plasma [Cr] xdurationofurine collection in minutes) • two major errors limiting the accuracy of CrCl • increasing Cr secretion can overestimate true GFR, particularly in azotem.ic patients • incomplete urine collection can underestimate true GFR; over-collection of urine overestimates it

Toronto Notes 2011

Nephrology NP7

Assessment of Renal Function

2. Cockcroft-Gault formula • serum Cr used along with age, gender and weight (kg) to estimate GFR (see sidebar) • nonnal range is >90 ml/min (> 1.5 ml/s) 3. MDRD (Modification of Diet in Renal Disease) formula • most common way in which GFR is estimated • complex formula incorporating age, gender, serum Cr, Mrican descent • GFR is reported as ml/min/L73m2 body surface area Limitations of Using Serum Cr Measurements I. must be in steady state • constant GFR and rate of production of Cr from muscles • sudden injury may reduce GFR substantially, but it takes time for Cr to accumulate and then re-establish steady state 2. GFR must fall substantially before plasma [CrI rises above normal laboratory range • with progressive renal failure, remaining nephrons compensate with hyperfiltration • GFR is relatively preserved despite significant structural damage 3.plasma [CrI is influenced by the rate of Cr production • lower production with smaller muscle mass (ie. female, elderly, low weight) • e.g. consider plasma [Crl oflOO f!InOl!L (1.13 mgldL) in both of these patients • 20 year-old man who weighs 100 kg, GFR = 144 mUmin • 80 year-old woman who weighs 50 kg, GFR = 30.6 mUmin 4. contribution of tubular secretion to Cr excretion is increased when GFR is low • CrCl overestimates GFR • certain drugs (cimetidine, trimethoprim) interfere with Cr secretion 5. errors in Cr measurement • very high bilirubin level causes [Cr] to be falsely low • acetoacetate (a ketone body) and certain drugs (cefoxitin) create falsely high [Cr]

... ,



Cockcroft-Gault Fonnlll

CrCI (mVmin] = I11!H!!fll X wt (kg) X 12 I X 0.85 in W0111111] (Crl,.... (umol!ll

....

,

·}-----------------,

C&nical Settings in which Urw. Lenl Ia MfKt.d lndaptlmlont llf Rn..

Function incruH in Urwa Volume depletion (prarenalezotemial Gl hemonllage High protlin diet

Sepsis

Celllbolic lllrt8 with tilsuu bnlakdown Cortic:osteroid or cytotoldc agents

In lira

Low protein diet Livar disalsa

Measurement of Urea Concentration • urea is the m.aj or end product of protein metabolism • plasma urea concentration is a measurement of renal function but should not be use alone as it is modified by a variety of factors • urea production reflects dietary intake of protein and catabolic rate; increased protein intake or catabolism (sepsis, trauma, GI bleed) causes urea level to rise • ECF volume depletion causes a rise in urea independent of GFR or plasma [CrI • in addition to filtration, a significant amount of urea is reabsorbed along the tubule • reabsorption is increased in sodium-avid states such as ECF volume depletion • typical ratio of urea to [Cr] in serum is 1:12 in Canadian units (using mmol/L for urea and !llllol!L for Cr), and 14:1 in US units (urea expressed as BUN in mgldl and Cr in mgldL)

Urinalysis • use dipstick in freshly voided urine specimen to assess the following: 1. Specific Gravity • ratio of the mass of equal volumes of urine/H 20 • normal range is 1.001 to 1.030 • values 1.020 reflect concentrated urine • value usually 1.010 in end stage renal disease (isosthenuria) 2. pH • urine pH is normally between 4.5-7.0; if persistently alkaline, consider: • renal tubular acidosis • UTI with urease-producing bacteria (e.g. Proteus)

3. Glucose • freely filtered at glomerulus and reabsorbed in proximal tubule • causes of glucosuria include 1. hyperglycemia >9-11 mmol/L (>160-200 mg!dl) leads to filtration that exceeds tubular resorption capacity 2. increased GFR (e.g. pregnancy) 3. proximal tubule dysfunction (e.g. Fanconi's syndrome) 4. Protein • dipstick only detects albumin; other proteins (e.g. Bence-Janes, lg, Tamm-Horsfall) may be missed • microalbuminuria (defined as 30-300 mglday) is not detected by standard dipstick (see Diabetes and the Kidney, NP34) • sulfosalicylic acid detects all protein in urine by precipitation • gold standard: 24-hr urine collection for total protein

.....



,

·}----------------,

Z4 hour Urinll Callectian 1. lliscllld first morning $JI&Cim&n 2. Collect all subsequent urina for lila next24 hi"$ 3. IWripra.. bltwnn voids 4. Collect second morning CIIJIIy: Cloudilass may indicate infection Colour: usually pllla yallow or lllllbar. but may be colourtess (dillllellls insipidus, excess Wlt8r intalal], bright yallow (due to ribofiiMn ingestion or vitamin ..bllllll). or dlllt yaUow (conciiTII'IDd urina in inlnMiscu!er volume depletion]

....

,



Eslinwting Urillll DIIIIDI..ity Lut 2 digits of lila specific g111Yity x 30 = urine osmolality approximatEly e.g. specific gravity of 1.020

= 600m0sm

NP8 Nephrology

Assessment of Renal Function

Toronto Notes 2011

5o Leukocyte Esterase • enzyme found in WBC and detected by dipstick • presence ofWBCs indicates infection (e.g. UTI) or inflammation (e.g. AIN)

6. Nitrites

• nitrates in urine are converted by bacteria to nitrites • high specificity, low sensitivity for UTI • +ve dipstick for leukocyte esterase and nitrites is 94% specific for diagnosing a UTI

.. ,

....

7. Ketones



T•nninolagy

Aly 111 or

•nil

... lllllwilfl

o

o



B. Hemoglobin Red 1)111 calls WlitiCIIcasts QllllljwQt



•inoopr

• positive in alcoholic/diabetic ketoacidosis, prolonged starvation, fasting

o

o

ltplne •

20 mmolJL) in the setting of acute renal failure: indicates renal disease vs. pre-renal • high urine Na (>40 mmolJL) in the setting of hyponatremia: generally from causes such as diuretics, tubular disease (e.g. Bartter's syndrome), SIADH • additionally, urine pH is useful to grossly assess renal acidification • "low" pH ( 100 cc,lhr, < 1DD mOsmiL) in lha sstting of hyponalr8mia is u.uaUy thefi!$1: sign of dangerously rapid C01111Ction of serum &Odium

.

... ' .-----------------. , Conection of Na in hyponab8mia should

not exteed BmmoVI/24 hrs Ldess

dlfinitlly known to t. 5.0 mEq!L Approach to Hyperkalemia 1. emergency measures: obtain ECG, if life threatening begin treatment inunediately 2. rule out factitious hyperkalemia; repeat blood test 3. hold exogenous K, and any K retaining medications 4. assess potential causes of transcellular shift 5. estimate GFR (calculate CrCl using Cockcroft-Gault) 6. if normal GFR, calculate TTKG = (Uk/PJ4 weeks

Treatment 1. preliminary measures • pre-renal • correct prerenal factors: optimize volume status and cardiac performance, hold ACEI/ARB • renal • exclude reversible renal causes: die nephrotoxic drugs, treat infection, and optimize electrolytes • post-renal • consider obstruction: structural (stones, strictures) vs. functional (neuropathy) • treat with Foley catheter, indwelling bladder catheter, nephrostomy, stenting 2. treat complications • fluid overload • NaCl restriction • high dose loop diuretics • hyperkalemia (refer to Treatment of Hyperkalemia, NP16) • adjust dosages of medications cleared by kidney 3. definitive therapy depends on etiology • note: renal transplant is not a therapy for AKI

.Acute Kidney Injury/Chronic Kidney Disease (CKD)

Toronto Notes 2011

Nephrology NP21

Prognosis • high morbidity and mortality in patients with sustained AKI and multi-organ failure

Chronic Kidney Disease (CKD) Definition • abnormal markers (Cr, urea) • GFR 3 months or • kidney pathology seen on biopsy or • decreased renal size on U/S (kidneys 50%) • focal: some glomeruli affected • terms applying to an indMdual glomerulus • global: entire glomerulus abnormal • segmental: only part of the glomerulus abnormal Types of Changes • proliferation: hyperplasia of one ofthe glomerular cell types (mesangial. endothelial, parietal epithelial), with or without inflammatory cell infiltration • membranous changes: capillary wall thickening due to immune deposits or alterations in basement membrane • crescent formation: parietal epithelial cell proliferation and mononuclear cell infiltration from crescent-shape in Bowman's space

Presentation of Glomerular Disease Important Points To Remember • each glomerulopathy presents as one of 4 major glomerular syndromes • acute nephritic • nephrotic • rapidly progressive glomerulonephritis • asymptomatic urinary abnormalities • each glomerulopathy can be caused by a primary disease OR can occur secondary to a systemic disease • some glomerulopathy can present as more than one syndrome at different times 1. ACUTE NEPHRITIC SYNDROME

Clinical/Lab Features • proteinuria (but 3.5 Qfdl 2. Hypollbumilemia 3. Edama 4. Hyperlipidemia, lipiduria 5. Oval fat bodies (microscopy( 6. Hypereoag!Jahle stile (antithrombin Ill, protein Cand pnrtein S lost in urineI

Low Complement Laval

Nonnll Camplamant Lartl

Postinfectious GN Mambranoprolifarativa GN

lgA nephropathy Anti.{iBM disaasa

Slf

PolyariErilis nodosa Wegener's granulomatosis Henoch.Schonlein pLrpura Goodpasue's synrtome

Endocarditis Abscess or shunt neplritis Cryoglobulinemia

2. NEPHROTIC SYNDROME Clinical/Lab Features • heavy proteinuria (>3.5 g/1.73m2/d) • hypoalbuminemia • edema • hyperlipidemia (elevated LDL cholesterol),lipiduria (fatty casts and oval fat bodies on microscopy) • hypercoagulable state (due to antithrombin III, Protein C and ProteinS urinary losses) • patient may report frothy urine • glomerular pathology on renal biopsy: • minimal change disease (or minimal lesion disease or nil disease) - i.e. glomeruli appear normal on light microscopy • membranous glomerulopathy • focal segmental glomerulosclerosis (FSGS) • membranoproliferative glomerulonephritis • nodular glomerulosclerosis • each can be idiopathic or secondary to a systemic disease or drug (sirolimus can cause proteinuria without obvious glomerular pathology) Tabla 11. Naphrotic Syndroma Minimal

Change

Hodgkin's lymphoma

Secondary CIUSIS

Membranous Glomeruloplllly

Focal S..-nlll Mambranoprolifaratiwe Glomeeulosderosis Glomeeulonephrilil

HBV, SLE, solid

Reflux ne(h'opathy, HIV; HBV, obe&ity



HCV, malaria. SLE, leukemia, lymphoma, inlecl8d &hunt

Nodular

Diabetes mellitus, amyloidosis

breast. Gil DI\IIICIIUIIS

NSAIDs

Gold, penicillamine Heroin

Th. .py

Steroids

Recllce BP, ACB, &teroids

Steroids, ACEVARB fur proiBinuria

Aspirin, ACEI, dipyridamole Treat undBriying controversial cause

The Nephritic-Nephrotic Spectrum • glomerular pathology can present with a clinical picture anywhere on a spectrum with pure nephritic and pure nephrotic syndromes at the extremes (see Figure 15) Naphratic

lntarmadiate

Hamatu.ria, "-

[ Protainara FSGS Membranous gtomarulopathy Minimal change

Naphritic

Membranoproliferative GN Focal proliferative GN • lgA nephropllhy • ldioplllhic membranoprolifenrtive GN

• HBV, HCV •SL.E • Cryoglobulinamia

Figura 15. Tha Spectrum of Glomerular Pathology



Diffuse prolifen.tive GN Crescentic GN

Toronto Notes 2011

Glomerular Disease

3. RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS (RPGN) Clinical/Lab Features • a subset of nephritic syndrome in which renal failure progresses in weeks to months • crescent formation usually seen on renal biopsy • RBC casts and/or dysmorphic RBCs in urine • classified by immunofluorescence staining (see Table 12) • treatment: underlying cause for postinfectious; corticosteroids + cyclophosphamide or other cytotoxic agent + plasmapheresis in select cases • prognosis: 50% recovery with early treatment, depends on underlying cause Tabla 12. RPGN Classification

RPGNTpl: AJrti.GBM m..iltld %111RPGNC.• 15% of cases lmmuno.ftuorncence Linear pattern due to lgG 111d SUing Pattem C3 deposition along capillary loops Antibodies against type IV collagen in GBM

RPGN Type II: Immune Complex m•iltlll

RPGN Type Ill: Non-immUDI lllldillld (i.e. piuci-immune)

24% of cases

60'J(, of cases

Granular pattern due to subendlllhelial or subepithelial deposits DllgG and C3

No immune stailing

Most often di&sa&e

z• to systemic

Vasculitis of glomerular capillaries

Primuyc....

Idiopathic anti.{lBM disease

lgA nephropathy

Idiopathic

s-nduvc....

Goodpasture's disease

Post-infectious GN, SLE, Cryoglobulinemia, Henoch-Sclxlnleil pLrpura

Wegener's {c-ANCA +vel Microscopic polyangiitis (p-ANCA +vel Cilurv-Stn!uss (ANCA -vel

4. ASYMPTOMATIC URINARY ABNORMALITIES Clinical/Lab Features • isolated proteinuria (usually 60 years old • infection over lumbar puncture (LP) site • uncooperative patient Complications • tonsillar herniation • post-LP headache (5-40%) -clear pattern: worse when upright, better supine; generally onset within 24 hrs • prevention: smaller gauge (ie. 22) needle, reinsert stylet prior to needle removal, blunt ended

needle

.....

,..,



The needle for a lumbar punclin is insertBcl into one of L3·4, L4-5, or L5.S1 inlnpacas.

\•, The volume of CSF removed during a lurnb11r puncture i& ruplsni&hed within one hour.

.....

,..,

































Do nat delay antibiotics while waiting

fer alumb11r puncture if suspicion of

• symptomatic treatment: caffeine and sodium benzoate injection • corrective treatment: blood patch • spinal epidural hematoma • infection

infactionl

What to send LP for • Tube #I: Cell Count and Differential: RBCs and WBCs and differential • xanthochromia [yellow bilirubin pigmentation) implies recent bleed into cerebrospinal fluid (CSF)I • Tube #2: Chemistry: Glucose (compare to serum glucose) and protein • Tube #3: Microbiology: Gram stain and C&S • specific tests depending on clinical situation/suspicion • viral: PCR for herpes simplex virus (HSV) • bacterial: polysaccharide antigens of H. injluenzae, N. meningitidis, S. pneumococcus • fungal: Cryptococcal antigen, India ink stain (cryptococcus), culture • TB: Acid-Fast stain, TB culture, TB PCR • Tube #4: Cytology (for evidence of malignant cells) • Tube #5: RBCs: compare RBC cell count to that of tube #l Tabla 5. Lumbar Puncture lntarpral:ltion (Normal vs. Various Infectious Causes)

Condition

Colour

Proteil

Glucose

Cells

NORMAL

Cle.-

3.0mmoL'l

O..SWBC, DRBC 0 neutrophills

Viral Infection

Cle.- or opaiBSCent

Normal or slightly increased >#5 -1r8umatic tap RBC in tube #1



SAH





N8 Neurology

Toronto Notes 2011

Seizure Disorders and Epilepsy

Seizure Disorders and Epilepsy Seizure Definitions

11•, Mldlcll EmetgiiiCyl S1atus Epilepticus can cause irmtanible brain damage without traatmant

• seizure - transient neurological dysfunction caused by excessive activity of cortical neurons, resulting in paroxysmal alteration of behaviour and/or EEG changes • epilepsy - chronic condition characterized by two or more unprovoked seizures • ictal- during seizure • post-ictal- period following a seizure when there may be a state of confusion/somnolence • inter-ictal- period between seizures during which epileptic discharges may be seen on EEG • status epUepticus- seizure lasting >30 minutes without spontaneous cessation or recurrent seizures without full return to consciousness inter-ictally

Classifieation SeiZU111 I

...

...

Provoked

UnpriiVOksd

I

+'. Panial- -

...

I

Simpla

I Motor Sensory

Stroke is the most common cause of late-onset (>50 'f8lll' of age) seizures, accounting for 53-BO'K. of c__

...

Complex

+

Autonomic



t.

can secondarily become - - - - - - - - - - - - - - - - - •Generahzad

...

Pwychiatric

Figura 1D. Classification of Saizuras

I

...

...

Convulsive



l

Abet nee

Fevar Metabolic Trauma

•

Clonic

I

+ +

Tonic

Tonio-Cionic

+ ...

Myoclonic

Atonic

Etiology • idiopathic • identifiable etiology: vascular, congenital, neurodegenerative or other neurologic disorders, neoplasm, trauma, childhood epilepsy syndromes, infection, metabolic, toxins, genetic • cryptogenic

Signs and Symptoms • generalized seizures • tonic-clonic (grand mal): • prodrome of unease or irritability hours to days before the attack • tonic ictal phase: tonic muscle contractions, arm flexion and adduction, leg • extension, 'cry' as respiratory muscle spasm and air is expelled; lasts 10-30 seconds • clonic ictal phase: clonus involving violent jerking of face and limbs, tongue biting, incontinence; 90% of essential tremor does not need treatment.

11•, Most common cause of chorea is drug therapy for Parkinson's disease.

© Lucy Zhang 2011

Figure 21. Horizontal Section of Basal Ganglia

Approach to Movements Disorders 1. Describe the movements. Classify each as hyperkinesias or hypokinesias 2. Name the movements (see Table 16) 3. Consider the differential diagnoses for the movements named

Differential Diagnoses 1. Tremor: a. Postural: physiologic, anxiety, sedative/alcohol withdrawal, drug toxicity, heavy metal poisoning, carbon monoxide poisoning, thyrotoxicosis, benign essential tremor, cerebellar, Wilson's disease • benign essential tremor is a common autosomal dominant trait that presents as a bilateral postural tremor of the vertical axis, especially in the upper extremities b. Intention: brainstem lesion, cerebellar lesion, alcohol, anticonvulsants, sedatives, Wilson's disease c. Resting: Parkinsonism, Wilson's disease, mercury poisoning Table 17. Approach to Tremors Resting

Postural

Intention Anywhere

Body Part

Distal UE

Uf/head/voice

Characteristics

3-7Hz pill rolling

6·12Hz fine tremor

sensory) slowing, decreased

F-wave • subtypes 1. Acute inflammatory demyelinating polyneuropathy (AIDP) 2. Acute motor-sensory axonal neuropathy (AMSAN) 3. Acute motor axonal neuropathy (AMAN) • treatment • disease specific: IVIg or plasmapheresis • nonpharmacologic: admit and monitor vital signs and vital capacity due to risk of respiratory failure, manage dysautonomia, manage pain • prognosis • nadir of symptoms at 2-3 weeks, with resolution at 4-6 weeks • 5% mortality (higher ifiCU), 7-15% permanent substantial deficits Diagnostic Approach to Peripheral Neuropathies 1. Differentiate: motor vs. sensory vs. autonomic l. Pattern of Deficit: symmetry, focal vs. diffuse, upper vs. lower limb, cranial nerve involment 3. Tempo: acute to chronic, relapsing remitting vs. constant 4. Good History: PMH, detailed family tree, exposures (e.g. insects, toxins, sex. travel), systemic symptoms 5. Detailed Peripheral Neuro Bum: LMN findings, differentiate between root and peripheral nerves, check cranial nerves, check respiratory status

GBS is a neurological emergency due to risk of imminent raspinrtory failtn.

....

,,



Miller-RICIH!r Y11rimt af GBS - Triild 1. Ophthalmoploqill

2. Ataxia 3. Arvllexil

....

,,



Mg and pi1Ui111apilemillalld tD morv rapid improvement, less intensive care

and less ventillllion, but do not change mortality or ralaps1 ram.

N32 Neurolo8Y

Neuro-oncology/Neurom115Cular Junction Diseaaa

Toronto Notes 2011

Neuro-oncology Paraneoplastic Syndromes Definition • uncommon complication of cancer; often is the presenting complaint

Pathophysiology • likely an autoimmune attack on the nervous sym:em by tumour antigens

Associated Neoplasms • small cell lung cancer: cerebellar degeneration, encephalitis, opsoclonus-myoclonus, retinopathy, neuropathy, Lambert-Eaton syndrome • breast: cerebellar degeneration, encephalomyelitis, opsoclonus-myoclonus • thymoma: myasthenia gravis • other syndromes: necrotizing myelopathy, motor neuron syndrome, neuropathies, mononeuritis multiplex, polymyositis and dermatomyositis, encephalitis

Investigations • antibodies commonly ordered include anti-Hu, anti-Ri and anti-Yo

Treatment • unsatisfactory and often palliative. Options to consider are steroids, IVIg, plasmapheresis and treatment of malignancy

Tumours of the Nervous System • see















NS9

Neuromuscular Junction Diseases Clinical Approach to Disorders of the Neuromuscular Junction Tabla 19. Common Disorders of the Neuromuscular Junction Butuli1m

.....

',..



Dis1un of 1h1 niUilllllllscular junction typically feature prominent fatiguability.

OculluAiulblr pamis

+

Limb WIIDISS

+ +

++(early)

+ + +

+ +

+

++

Thymoma

Small cell carcinoma

GISSx

"'

1' (rapid sti'I'IJiation)

1' (rapid stimulation)

htiguablity

Post-exen:ile enhlncement

+

N

Reflexes ANS anti:hulin. .ic Sx

SansarySx

Associated conditions Rapatilivll EMG stimulatilll

-.1- (slow



>lr (slow stimulation)

Myasthenia Gravis (MG) Etiology and Pathophysiology • damage and blockade of post-synaptic acetylcholine receptors by specific antibodies • 15% of patients with myasthenia gravis have associated thymic neoplasia, 85% have thymic

hyperplasia • autoimmune disorder

Epidemiology • bimodal age of onset - 20's (mostly women) and 60's (mostly men)

Toronto Notes 2011

Neurology N33

Neuromusc:ula.r Junction Diseasea

Signs and Symptoms • see also Table 19 • fatiguability and weakness of skeletal muscles without reflex, sensory, or coordination abnormalities • typically ocular (diplopia/ptosis) -+bulbar (dysarthria/dysphagia) -+ necldlexors/extensors -+ proximal limbs • respiratory muscle weakness may lead to respiratory failure

Myasthenia Gravis is a neurological emergency due to 1he risk of imminent rnpillllory failure I

....



Investigations • edrophonium (Tensilon•) test -can result in respiratory difficulty so have crash cart nearby • assess for improvement over 2 minutes following edrophonium injection ·EMG • repetitive stimulation -+ decremental response • single fibre electromyography shows increased jitter (80-10096 sensitivity) • anti-acetylcholine receptor antibody assay (70-80% sensitivity) • MUSK antibody may be used if seronegative for AChR antibody • CT/MRI to screen for thymoma/thymic hyperplasia

Treatment

,,

Tensilon• is a drug 1hat inhibit$ acltylcholinestlrllsl. It improvM mJscll function immadilltaly in my811henia

gravis, but not in cholinergic crisis.

....

,,



zClinical Forms rrf Mpltllenil GriVis

1. Ocular [15"'} 2. Gene1111ized (85%1

• thymectomy • 8596 of patients show improvement or remission • symptomatic relief • acetylcholinesterase inhibitors (e.g. pyridostigmine) • does not affect primary pathologic process -+ rarely result in control of disease when used alone • immunosuppression • steroids are mainstay oftreatment - 70-80% remission rate • azathioprine, cyclophosphamide and mycophenolate as adjuncts to steroids or as steroid sparing therapy • short-term immunomodulation (for crises) • IVIg and plasmapheresis

Prognosis • 3096 eventual spontaneous remission

Lambert-Eaton Myasthenic Syndrome (LEMS) Etiology and Pathophysiology • downregulation of presynaptic voltage-gated Calcium channels 2° to specific channel binding antibody causing decreased amounts of ACb released into the synaptic cleft • 50-6696 are ultimately associated with small cell carcinoma of the lung

Signs and Symptoms • • • •

weakness of skeletal muscles without sensory or coordination abnormalities reflexes are diminished or absent, but increase after active muscle contraction bulbar and ocular muscles affected in 25% prominent anticholinergic autonomic symptoms (dry mouth >impotence> constipation > blurred vision)

Investigations • • • •

edrophonium test (see Myasthenia Gravis) -+ no response EMG: rapid (> 10Hz) repetitive stimulation -+ incremental response screen for malignancy, especially small cell lung cancer post-exercise facilitation- an incremental response to repetitive stimulation due to presynaptic calcium accumulation

Treatment • tumour removal • acetylcholine modulation • increased acetylcholine release (3-4 diaminopyridine) • decreased acetylcholine degradation (pyridostigmine) • immunomodulation • steroids, plasmapheresis, IVIg

....

,,



Lambert-Eaton myas1hanic syndrome can be differentiated from myasthenia Qlllvis, by 1ha phenomenon of postexercise facilitlltion.

N34 Neurolo8Y

Toronto Notes 2011

Myopathiea

Myopathies Clinical Approach to Muscle Diseases Table 20. Myopathies

Ksr lnvatigations

Etiology lnlllmmiiDry

....

',

Polymyositis

Mya[gies Pharyngeal involvement

1' CK Biopsy: endomesial Necrosis

Dermatomyositis

Mya[gias Similar to polymyositi& Characteri&tic r.&les Can be paraneoplastic

1' CK Biopsy: parifasciculll' atrophy

Sarcoido8is



ACE IIMII Biopsy: IJliiUIOIIllls

Inclusion body myositis

Weak quads and deep finger flexors

1' CK Biopsy: ilclusion bodias

Thyroid (1' or -1-) Cushing's syndrome Parathyroid (1' or -1-)

See Endocrinology

TSH, serum cortisol, calcium panel

Medication

Medication or toxin history

Toxicology

Critical illness myopathy

ICU patient Hx steroid& and nondepolarizing palltfzing agents Faiure to ween from ventilation

Biopsy: selective loss of thick Myosin filaments

Parasitic, bactErial, or vinll

Mya[gies Inflammatory myopathy

1' ITI'fl9obin



rn-

lmporu,nt lnfanution to Reganllnf Myapllthilll • Weakness: proximal > distal

• Pain: myalgias, but no impaired sen.tion

• MyotDnil [difficulty with relaxldioo)

.....

',

Endoc:rin1



Myoplllllisl1118 chlllle1llrizad by prominent symmetric proximal

weakness end lbsant SIIIIIO!'f chlngal.

.....

',

Good Cll..ti- to Alina Proximal

wen-s

',



Common Mellicalio1111bat Cauu Mpptllhy Steroids, mrtins and IIIT!mnmrBis









Duchenne

onset {Duchenne and

Becksr

Prograssiw proxi11111l muscle -knass pseudohypertrophy

Staining

Myotonic dystrophy

Distal myopathy Myotonia Genetic anticipation

Genetic testing

Hereditary Mltlbolic

McArdle's

Exercise-related rnyalgias, cramping, and myoglobumimria

1' lactate 1' serurnturinary myoglobil Pllst-sxen:ise

Heredililry Periodic Parllylil

Periodic paralysis

Episodic W8ilkness between attacks

1'cr-I-K

Heredililry

MERRF MELAS ICI!ImsSayre

Ptosis, conmon Proximal > distal myopathy Exercise intolerance Rhabdomyolysis

Increased lactate Biopsy: ragged red fibres

Hereditary Dystrophy

• Legs: climbing slllirl, stand from sit • Anna: n111ch above hlllld, wash hair

.....



Mitochandriil

•Abbreviltion5: MBliiF -ITilochoncnl encephlllomyoplllhy slrulie-like episodes

rauged llld fibe11;

Becker)

Biopsy: abnormal dyttrophin

MELAS- mitochondrial encepllllomyopathy, lactic I!Cifbis, and

Polymyositis/Dermatomyositis • see RH13

Myotonic Dystrophy Etiology and Pathophysiology • unstable trinucleotide repeat in DMK gene (protein kinase} at 19ql3.3 • number of repeats correlates with severity of symptoms; autosomal dominant Epidemiology • most common adult muscular dystrophy • prevalence 3-5/100 000 Signs and Symptoms • appearance: ptosis, bifacial weakness, frontal baldness {including women), triangular face giving a drooping/dull appearance

Toronto Notes 2011

Myopathies/Cerebellar Disorders

• physical exam • distribution ofweakness: distal greater than proximal (in contrast to other myopathic disorders) • myotonia: delayed relaxation of musclc:s after exertion (elicit by tapping on thenar muscles with hammer) • cardiac: 90% have conduction defects ( 1° heart block; atrial arrhythmias) • respiratory: hypoventilation 2° to muscle: weakness • ocular: subcapsular cataracts, retinal degeneration, decreased intraocular pressure • EMG: subclinical myotonia -long runs with declining frequency and amplitude

Treatment • no cure • management of myotonia: phenytoin

Duchenne and Becker Muscular Dystrophy • see Pediatrics, P46

Cerebellar Disorders Clinico-Anatomic Correlations • vermis: trunk/gait ataxia • cerebellar lobe (i.e. lateral): tremor, rebound phenomenon, dysarthria, dysdiadochokinesis, nystagamus Symptoms and Signs of Cerebellar Dysfunction • nystagmus: observe on extra-ocular movement testing (most common is gaze-evoked nystagmus) • dysarthria (ataxic dysarthria): abnormal modulation of speech velocity and volume- elicit scanning/telegraphic/slurred speech on spontaneous speech (see Dysarthria, Nl9) • ataxia: broad-based, uncoordinated, lurching gait • dysmmetria: irregular placement ofvoluntary limb or ocular movement • dysdiadochokinesis: unable to perform rapid alternating movements (e.g. pronationsupination task) • postural instability: look for truncal ataxia on sitting (titubation =rhythmic rocking of trunk and head); look for difficult tandem gait and broad based gait • intention tremor: elicit on finger-to-nose testing- typically orthogonal to intended movement, and increases as target is approached • hypotonia: decreased resistance to passive muscular extension- occurs immediately after injury to lateral cerebellum • pendular patellar reflex: knee reflex causes pendular motion ofleg occurs after injury to cerebellar hemispheres • rebound phenomenon: overcorrection after displacement of a limb (with both arms extended --+ pushing both will cause one to rebound up if there is lesion on that side)

Wernicke-Korsakoff Syndrome • deficiency of thiamine due to alcohol abuse • acute: apathy, confusion, decreased EOM, ataxia (truncal and gait) • without treatment progresses to encephalopathy and ultimately death • treatment: thiamine 100 mg • Korsakoff's syndrome: progressive decline ofboth anterograde and retrograde memory • note that alcohol can also cause a cerebellar ataxia separate from thiamine deficiency. The ataxia can be due to cerebellar atrophy or alcohol polyneuropathy

Cerebellar Ataxias Congenital Ataxias • early onset nonprogressive ataxias associated with various syndromes as well as development abnormalities (e.g. Arnold-Chiari malformation, Dandy-Walker cysts) Hereditary Ataxias • autosomal recesaive: includes Friedreich's ataxia, ataxia telangiectasia, vitamin E deficiency • Friedreich's ataxia: prevalence 2/100 000; onset between 8 and 15 years • signs: gait and limb ataxia, weakness, areflexia, extensor plantar reflex, impaired proprioception and vibration • death in 10-20 years from cardiomyopathy or kyphoscoliotic pulmonary restriction • autosomal dominant: spinocerebellar ataxias (SCA.s) of which 30 exist, most are CAG repeats

Neurology N35

N36 Neurolo8Y

Cerebellar Disorden/Vertigo/Gait Diaturbances/Pain Syndromes

Toronto Notes 2011

Acquired Ataxias • neurodegeneration (e.g. multiple system atrophy) • systemic: alcohol, celiac sprue, hypothyroidism, Wilson's, thiamine deficiency • toxins: carbon monoxide, heavy metals, lithium, phenytonin, solvents • vascular: infarct, bleed, basilar migraine • autoimmune: MS, Miller-Fischer (GBS) • children: tumours, post-viral

Vertigo • see Otolaryngology, OT12

Gait Disturbances Approach to Gait Disturbances I. Length of stride • if small paces - look at posture • if stooped with no armswing- Parkinsonian gait • look for other signs of extrapyramidal disorders • if upright with exaggerated armswing - Marche a petit pas • due to diffuse infarction of both cerebral hemispheres (lacunar)

r-t•,

CENTRAL MOTOR SYSTEMS 3 compn•nb tu til• cantrol af pit 1. Pyramidal: main ouUiow from cor1ex to spinal cord 2. Extrapyramidal: bani ganglia inhibita BJa:llll mCMimanb; 3. Clrlblllum: afflcts coordination of

2. If normal stride length, look at width between feet • if wide-based- ataxia • if high stepping and positive Romberg- sensory ataxia • loss of joint position sense (+ve Romberg) • if wide based without high stepping - cerebellar ataxia • veers to side of the lesion • if scissoring of legs or toe walking- spastic gait • bilateral circumduction due to spastic paraparesis from cerebral palsy, multiple sclerosis or cord compression 3. If normal width, look for height of step • if high stepping bilaterally- bilateral foot drop • if feet barely leave ground or disjointed movement - magnetic/apraxic gait • frontal lobe pathology due to normal pressure hydrocephalus or cerebrovascular disease 4. If no high stepping, lookfor stabllity of pelvis • if rotation of pelvis - waddling gait

• proximal muscle weakness due to congenital deformity or myopathy 5. If no waddling, look at symmetry • if asymmetric - antalgic gait, deformity or hemiparetic gait • antalgic gait is due to pain from an MSK problem • hemiparetic gait involves a foot drop and circumduction of spastic leg due to UMN lesion

6. If movement is elaborate and inconsistent, especially when being observed. conaicler functional pit • rule out an odd gait due to chorea from Huntington's disease

Pain Syndromes Approach to Pain Syndromes

...._,,

• Pinprick CIIUUI sharpnns rnldimd byJIIJfibani • Pain to damage is mediated by Cfibres

Definitions • Nociceptive pain: pain arising from normal activation of peripheral nociceptors • Neuropathic pain: pain arising from direct injury to neural tissue. bypassing nociceptive pathways • Spontaneous pain: unprovoked burning, shooting, or lancinating pain • Paresthesiae: spontaneous or evoked abnormal nonpainful sensations (e.g. tingling) • Dysesthesiae: spontaneous or evoked pain with inappropriate quality or excessive quantity • Allodynia: a dysesthetic response to a nonnoxious stimulus • Hyperalgesia: an exaggerated pain response to a noxious stimulus

Toronto Notes 2011

Pain Syndromes

Medical Pain Control • primary analgesics: OTCs, opiates • adjuvants: antidepressants (TCAs, SSRis), anticonvulsants (gabapentin, carbamazepine), baclofen, sympatholytics (phenoxybenzamine), a2-adrenergic agonists (clonidine, pregabalin)

Surgical Pain Control • direct delivery: implantable morphine pump • central ablation: stereotactic thalamotomy, spinal tractotomy or dorsal root entry lesion • peripheral ablation: nerve blocks, facet joint denervation • deep brain stimulation (DBS) or dorsal column stimulation

Neuropathic Pain Definition • pain resulting from a disturbance of the central or peripheral nervous system

Symptoms and Signs • hyperalgesia/allodynia • subjectively described as -burning, heat/cold, pricking, electric shock, perception of swelling, numbness (Le. stocking/sock distribution) • can be spontaneous or stimulus evoked • distribution may not fall along classical neuro-anatomicallines

Associated Issues • sleep difficulty • anxiety/stress/mood alteration • sexual dysfunction

Causes of Neuropathic: Pain • peripheral neuropathy • systemic disease - diabetes, thyroid disease, renal disease, rheumatoid arthritis • nutritional/toxicity- alcoholism, pernicious anemia, chemotherapy • infectious - HN • trauma - post surgical, nerve injury • nerve root: post-herpetic neuralgia, cervical and lumbar radiculopathies, tic douloureux (see Trigeminal Nerve, Nl8), plexopathies • central: MS, post-stroke, phantom limb, spinal cord injury • Complex Regional Pain Syndromes (see N38) •malignancy

Treatment • pharmacotherapy: TCA. SNRI, anticonvulsant, long acting opiate, topical lidocaine, capsaicin cream, intrathecal opioid or clonidine, Botox, nerve block • surgical therapies: dorsal column neurostimulator, DBS (thalamus) • other therapies: • neuropsychiatry - cognitive behavioural theraphy, psychotherapy • rehabilitation - physiotherapy • CAM - acupuncture, meditation, massage therapy, TCM

Tic Douloureux (Trigeminal Neuralgia) • see Trigeminal Nerve, N18

Postherpetic Neuralgia (PHN) Definition • pain persisting beyond 3 months in the region of a cutaneous outbreak ofherpes zoster

Etiology and Pathogenesis • destruction of the sensory ganglion neurons (e.g. dorsal root, trigeminal, or geniculate ganglia) secondary to reactivation of herpes zoster infection

Epidemiology • 10-15% of all patients with cutaneous herpes zoster • >80% of herpes zoster infected patients >80 years old

Neurology N37

N38 Neurolo8Y

Pain Syndromes

Toronto Notes 2011

Signs and Symptoms • types of pain: constant deep ache or burning. intermittent spontaneous lancinating/jabbing pain, allodynia • distribution: thoracic > trigeminal > cervical > lumbar > sacral Treatment • acute herpes zoster • early treatment with antiviral agents (acyclovir; longer-acting famciclovir and valaciclovir more effective) may prevent PHN in patients over 50 years • PHN • medical: TCA, pregabalin, gabapentin, opiate, lidocaine patch, intrathecal methylprednisolone • surgical: spinal tractotomy, dorsal root entry zone lesion

Complex Regional Pain Syndromes (CRPS) Definitions • CRPS is a pain syndrome characterized by the following 1. presence of an initiating noxious event 2. continuing pain, allodyrua, or hyperalgesia with pain disproportionate to inciting event 3. evidence during the course of symptoms of edema, changes in skin blood flow, or abnormal vasomotor activity 4. absence of conditions that would otherwise account for degree of pain and dysfunction Classification • CRPS type I (reflex sympathetic dystrophy): minor injuries of limb or lesions in remote body areas precede onset of symptoms • CRPS type II (causalgia): injury of peripheral nerves precedes the onset of symptoms Signs and Symptoms • stage I (acute) • pain: burning or aching disproportionate to initial injury • autonomic: edema and temperature inequality • stage II (dystrophic) • pain: constant and increased by stimulus to affected part • autonomic: osteoporosis, cool hyperhydrotic skin, hair loss, cracked/brittle nails • stage III (atrophic) • pain: paroxysmal spread • autonomic: thin, shiny skin, thickened fascia with contractures, bony demineralization Investigations • diagnosis is clinical • trial of differential neural blockade may be helpful Treatment • medical: phenoxybenzamine (sympatholytic) • surgical: paravertebral sympathetic ganglion blockade

Thalamic Pain (Dejerina Roussy Syndrome) ---Definition • hypersensitivity to pain as a result of damage to the thalamus Etiology and Pathogenesis • injury to ventral posterolateral (VPL) and ventral posteromedial (VPM) nuclei of the thalamus • ischemic stroke • hypertensive vascular hemorrhage Signs and Symptoms • begins with hemianesthesia • then persistent spontaneous burning contralateral to lesion • altered response to light cutaneous and deep painful stimuli Treatment • medical: amitriptyline, anti-convulsants • surgical: stereotactic thalamic stimulation (may increase sensory deficit)

Headache

Toronto Notes 2011

Neurology N39 a.lllliaal cmc.J Eumilllllign: O.llil Pllilnt wilb 11Hdde .... a..,.• • Nllll

N......,

Headache

JWA 2006; 2!1&:1 274-83

Clinical Approach to Headaches Investigations • good history and physical to rule out serious causes of headache • important aspects of neurologic exam: LOC and MSE, pupils (symmetry), fundi (papilledema, retinal hemorrhages), pronator drift, meningismus, deep tendon reflexes and Babinski, gait • indications for a • new-onset headache, worst headache of life, thunderclap headache, headache with worrisome symptoms (fever, meningismus, altered LOC, focal neurologic deficits, trauma, papilledema, morning headache) • if CT is negative but suspicion of SAH or meningitis, perform a lumbar puncture

cr

Table 21. Headaches- Primary TCIIIioa-Typa

Migrma

Clustar

nnmanic: p-



a - 4inl1ion of 4-72

u-

UnilideriiiOCIIion

N - Nlu• or-womiling D- Disllblingint!llsily

ThaiR far dafiW or polllil1i n-i11111inl dill!jlllllii VlrillwM!I U.lllmblr rlfiiUM pr8I8IE Mdl 3111d feiW U.Llls ... 24 {1.5-3181. 3.5 {1.3-9.2111111 0.41 {0.32-0.521 TllpiC1ivltf. Dnlllil , . . .... h...... IIIII

..............,

The prawlence rl ii'Uicllnill pllllology {!111111 problliiitylwrias lnlll011 v.1lh dlronic helldldle 1he plellllence is l.l'J. {0.77-1.11\).ln Ylll niglint-typl lllldlclw the prMiince is Howavll. inlhose prasal1ing wi1h new lllldlclw the prMiince is 32'Jo • in 1hose jiiUidiiQ Ylilll t!UIIIarcllp hlllldlchl the prMimct is 43\ {211-611\). Ill th8le dilllrant popullbs. 110 clinical flllfln was found lo hlllllful inlllivJ imcrlrill pethoQ in a 1118111iniiUMYHawM!. -a indivi!UI c1inicll IIU.ns Will luund 1o 1xt ]IIICictive of lignili:lnt

Prevalence

70%

12%

M

F>M

M>F

Family HistaiY

Nona

+++

+

l.ocatiCII

Bilateral frontal

Retroorbilll



Uniateral>bilateral Fronto-tenwal

Dul'llion

MinLIII!s-days

Haurs-days

10mh-2. haurs

Onset/CGune

lndual; worse in PM

Gradual; worse in PM

Daily headache for weeks, months, nocturnal

c:QI8r-tp haadache

petholoQr.

Band-like; constant

Tlrobbing

Constant, aching. stabbing

111ddneli-typl hlldlch1

Severity

Mild-modarata

Modarata-severa

S8\111'8 (waklls from sleep)

lllldlclwv.tii-

Noise Hunger Slaap deprivation

Noise Light Strainilg Coujing Activity

Light

Pallating

Rest

Rest

Walking around

ARoc:iltad Sx

No vomiting No photophobia

Nausi!I1/VDmiting Photo/phonophobia Aura

Red watery eve Nasal congestion or rhinoi'Thea Unilateral Homer's

Muscle tension in scall)'neck Tandar scalp artaias

Red watery eye, rhinorrhea Eyaid !hop

AcuteRx •MA • NSAIDS • Triptans • Ergotamine Prophylaxis • l'ropnllolol • TCA • Anticonvulsents

Acute Rx • Oz • Sumatriptan (IIIISII or injection) Prophylaxis • Verapamil • Lithium • Methylsergide • Pnmisolone

Depression Anxiety

Pllysicll Signs

Muscle tension i'1



MIRII!IIdllnt

Non1)harmacological • Psychological counseling • Physical modalities (e.g. heat, massage) Phannacological • Simple analgesics • Tricyclic antidepressants

EtOH

Table ZZ. Headaches- Serious Incidence

Meningllllrrilatilll

...creased lllraa'anill Pressure

Te11poral Arteritis