SLE

Ø  Lupus means wolf ,manifestation like hungry wolf

Ø  It is autoimmune multisystem disease

Ø  Etiology-Unknown

Ø  Pathogenesis-Ab against every component of cell

Ø  Asian 0.03%

Ø  Black 0.2%

Ø  Onset-20-30 years ,gradual

Ø  F:M =8:1

C/F

Ø 
Fever ,malaise ,mayalgia ,arthralgia

Ø  Photosensitivity-Butterfly rash under sun(Discoid rash in DLE)

Ø  Fall of scalp hair

Ø  Signs of arthritis are less common(Jaccoud's deformity)

Ø  Spontaneous abortion

Ø  Pericarditis ,effusion

Ø  Pleurisy ,effusion ,consolidation

Ø  Red urine

Ø  Anemia/leukopenia/pancytopenia

Ø  Psychosis ,seizures ,convulsion

Ø  General lymphadenopathy

Examination-Anemia ,lymphadenopathy ,loss of hair ,rash ,oral ulcer over hard palate

Systemic-Acc. to systemic features

Diagnostic criteria/ARA(11)

Ø  Butterfly rash

Ø  Discoid rash

Ø  Oral ulcer

Ø  Photosensitivity

Ø  Arthritis(Non erosive ,2/more peripheral)

Ø  Serositis

Ø  Renal-Proteinuria >0.5g/day ,3+ ,cellular cast

Ø  Hematologic-Anemia/leukopenia ,4000/cmm/thrombocytopenia <100000/cmm

Ø  Neurologic -

Ø  +ve ANA Ab ,

Ø  +ve Anti DS DNA Ab

[4/more-Definite SLE]

Complication

Ø  Renal-AGN ,NS ,ARF

Ø  CVS-Pericarditis ,HF ,premature MI/due to vasculitis

Ø  CNS-Psychosis ,convulsion

Ø  DVT ,pul embolism

Investigation

Ø  BLD-TC ,DC  WBC ,HB ,ESR ,total bilirubin

Ø  Routine-Total urine protein(>3.5 g/1.3 sq.m/day in adult)

Ø  Urine RME-RBC cast

Ø  CXR

Ø  ECG-IHD/HB

Ø  ANA-80% +ve(So here Anti DS DNA Ab +ve 30%)

Ø  Anti phospholipid Ab(In abortion pt/chance of thromboembolism)

[Prognosis depends on renal system/vital organ involvement]

Histological change of renal involvement

Ø  Every glomerular disease may occurs

DD/+ve ANA

Ø  Dermatomyositis

Ø  Polymyositis

Ø  Mixed connective tissue disease/MCTD

Ø  Sarcoidosis

Ø  Rh arthritis

DD of butterfly rash

Ø  SLE ,DLE

Ø  Dermatomyositis

Ø  MCTD

Ø  Sarcoidosis

Ø  PKDL

Rx

Ø  Nature of disease  must be explained

Ø  Mild to moderate/no renal involvement-For pain give NSAIDs ,hydroxychloroquine,if not controlled  then give prednisolone 30-60mg/day

Ø  If renal involvement

                          Methylprednisolone 10mg/kg/day IV for 3 days

                               Followed by Oral prednisolone 30-60mg/day

                                   Methotrexate 20-30mg/wk

                                          If side effects>Slowly decrease the dose and add chemotherapy

Parkinsonism

Parkinson's  syndrome

Ø  Syndrome consisting  of tremor ,rigidity and hypokinesia(+Postural instability)

Parkinson's disease

Ø  Primary/idiopathic parkinsonism is Parkinson's disease

Ø  Idiopathic neurodegenerative disorder due involvement of basal ganglia ch. by Parkinson's  syndrome

Parkinson's plus

Ø  Parkinsonism with other degenerative diseases

[Others are/atypical parkinsonism syndrome-Shy drager syndrome ,nigrostrial degeneration ,steele -Richardson syndrome]

Parkinsonian tremor

Ø  Involuntary

Ø  Coarse

Ø  4-6Hz

Ø  Present at rest

Ø  Reduce during voluntary movement(Opposite Cerebellar tremor) and sleep

Ø  Initially pill rolling movement of thumb and index finger

Ø  Later ,affects arms ,legs ,jaw , tongue

Ø  Usually unilateral

Parkinsonian rigidity

Ø  Lead pipe-Uniform rigidity in flexor and extensor  limbs(Better seen in elbow and knee)

Ø  Cog-wheel-Rigidity is interrupted by tremor(Better seen in  wrist).Interrupted rigidity

[Rigidity of affected limb is increased  with active movement of opposite limb

Rigidity is increased muscle tone  present throughout the  passive movement caused by extrayramidal lesion

Spasticity is increased muscle tone present  which is maximal at beginning and decreased  as the passive movement continue  due to pyramidal lesion

Hysterical rigidity is the more and more increase in muscle tone during passive movement of affected limb]

Parkinsonian hypokinesia/dyskinesia

Ø  Difficulty in initiating movement

Ø  Slowness of movement

Ø  Can't deliver a quick hard blow

Ø  Alternative movement progressively impedes and blocked completely

Ø  Can't complete rapid ballistic movement

Parkinsonian gait(Festinant gait)

Ø  Rapid ,small ,shuffling step ,hardly raises his foot from ground(?)-To avoid falling

Ø  Flexed attitude(?)-To catch up own centre of gravity

Ø  Less swing f arms

Ø  Difficulty in stopping

Ø  Difficulty in rapid turning(Fractioned gait)

Ø  Others-

·         Propulsion-Pushed from behind>Unable to stop>Forward fall

·         Retropulsion-Pushed from back>Unable to stop>Backward fall

·         Kinesia paradoxica-

o   Unable to initiate ,but once started can complete whole act e.g.ran out from house  during ,run down stairs but can't stop

Parkinsonian reflexes

All jerk/reflex/plantar response  are normal

Plantar response can be extensor if atypical parkinsonism or associated with  UMNL

Parkinsonian pathology

Ø  Progressive dengeration of dopaminergic neurons of substantia nigra with formation of Lewy body(Hallmark)

Ø  Dopamine is decreased so imbalance between dopamine and Ach

Ø  Atypical parkinsonism-MRI>Atrophy of midbrain with preserving pons(Hummingbird sign)

Parkinsonian cause

Ø  Parkinson's disease

Ø  Post encephalitic

Ø  Drugs-Phenothiazine group ,haloperidol ,metochlopromide ,methyldopa

Ø  Trauma(Punch drunk syndrome)

Ø  Wilson's disease

Ø  Huntington's disease

Ø  Parkinson  plus

Ø  Normal pressure hydrocephalus(Triad of incontinence ,gait apraxia ,dementia)

Traits	Post encephalitic	PD
Age	Any	Elderly
Onset	Sudden	Insidious
Previous history	Encephalitis	No
Complaints	Mainly rigidity	Mainly tremor
Symmetry	Fairly symmetrical	Asymmetrical
Oculogyric crisis Ophthalmoplegia Pupil	Present Present Dilated	Absent Absent Normal
Plantar response	Extensor	Flexor

Oculogyric crisis

Ø  Involuntary ,sustained upward and outward deviation of eye

Ø  Causes-Post encephalitic parkinsonism ,phenothiazine ,petit mal epilepsy

Traits	Essential tremor	PD tremor
Stimulus	Starts with action	Starts with rest
Family hist.	Yes	No
Symmetry	Yes	No
Side	Bilateral	Unilateral

Staging of PD

Ø  Stage-1:Unilateral involvement(Hemiplegic PD)

Ø  Stage-2:Bilateral involvement without postural abnormality

Ø  Stage-3:Bilateral involvement with mild postural abnormality

Ø  Stage-4:Stage-3 +requiring help for postural abnormality

Ø  Stage-5:Fully developed disease making pt. restricted to bed/wheelchair

[Rx-Levodopa with carbidopa

Contraindication of levodopa-Narrow angle glaucoma ,PUD]

Types of movement disorder

Ø  Hypokinetic-PD ,PD plus

Ø  Hyperkinetic

·         Rhythmic-Tremor

·         Non rhythmic-

v  Chorea(Lesion in caudate nucleus)-Jerky involuntary purposeless movement

v  Athetosis(Lesion in putamen)-Slow writhing movement of limbs

v  Hemiballismus(Lesion in subthalamic nucleus)Dramatic form of chorea violating flinging movement of one side of body ,or one limb(monoballism)

v  Myoclonus(Lesion in multiple sites of brain)-Isolated random jerk of muscle group(Onset of sleep)

v  Dystonia(Unknown)-Sustained involuntary  muscle contraction causing abnormal posture

v  Tics(Unknown) -Stereotyped repetitive movement(Blink/wink/headshaking/shrugging)

Parkinsonian sign

Ø  Tutibation

Ø  Mask facies(Expressionless face)

Ø  Serpentine stare(Less blinking)

Ø  Blepharoclonus(Eyelid tremor when gently closed)

Ø  Palilalia(Repetition of end of a word)

Ø  Glabellar tap(Repeatedly tap at glabella>Blinking continues which should normally stop after 3/4 blinks)

Ø  Myerson sign(Glabellar tap at  nasal bridge)

Ø  Tremor

Ø  Rigidity

Ø  Hypokinesia

Ø  PD gait