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Neurology Grand Round

23.02.17
PATIENT PARTICULARS
Ashik Gharami
47 year , male
Muslim
CHIEF COMPLAINTS
Weakness of all 4 limbs-3yrs
Thinning all 4 limbs-3yrs
Tingling and paresthesia over fingers &
toes- 3yrs
Neck pain -3months
Insidious onset,gradually progressive
History of present illness
Patient was apparently well 3yrs back.To start with he
complained of weakness of all 4 limbs-insidious onset
,gradually progressive,involving distal part of both upper
& lower limbs.He found difficulty in mixing rice, holding
objects firmly,buttoning & unbuttoning shirts and holding
clothes for cutting. Initially he could do with difficulty,but
for last 2-3months,he is not able to do his job because of
the increased severity of weakness.
He also C/O slippage of chapals with awareness for the
same duration.
H/o difficulty in combing,doing overhead activities or
getting up from squatting posture since last 3months.
History of present illness(cont)
H/o neck and trunk weakness.
He found difficulty in lifting head from pillow and turning
sides on bed without assistance.
He also gives h/o thinning of all 4 limbs both proximal
and distal.
Significant weight loss (76kg previously,50kg at present).
Weakness was associated with looseness.
No h/o abnormal twitching,stiffness,flexor spasm.
No h/o diurnal variation
History of present illness(cont)
He complained of intermittent tingling pain over fingers and
toes after prolonged work/exertion.
No h/o burning ,cotton wool or plaster cast like sensation,or
numbness.
However,he C/O sharp,shooting pain over the upper
neck,radiating to both shoulder,down to dorso-lumbar spine
and up to left occipital region.These are not increased by
coughing,sneezing and movement,but precipitated by flexion
of head.pain has decreased in intensity at present.
He c/o unsteadiness of gait after prolonged walking and
difficulty while walking on uneven surface.Not increased at
evening hours.No visual or ear complain.No h/o fall,limb
tremulousness.
History of present illness(cont)
On direct quesioning,he complains of decreased
sweating over the body except the chest region.No h/o
hair loss,cold skin.
He gives h/o constipation for long time.
H/o loss of early morning erection for last 3yrs.
No history suggestive of bladder involvement,syncope.
No h/o diplopia,ptosis,dysphagia,nasal regurgitation of
liquid,nasal intonation of voice,facial asymmetry.
No h/o seizure,LOC,tremulousness,abnormal
posturing or fall.
History of present illness(cont)
On further questioning,he gave h/o tender skin
lesions over chest,trunk,back,left face,
proximal UL,rt LL.These are tender,slight
reddish appearance,no discharge,largest one
near rt axilla(noticed for last 2wks)
Family members noticed a swelling in posterior part of
neck on rt side.
H/O on and off fever(not documented)
No h/o rash,oral ulcer,joint pain, deformity.
No h/o respiratory distress,chest pain,palpitation.
No h/o drugs or toxin exposure
PAST HISTORY
Diagnosed as Type2DM for last 3yrs, on
medication
Normotensive
No thyroid illness,TB
H/O jaundice-30yrs back
H/O proptosis of left eye f/b double vision-
26yrs back,which recovered after
consulting Ophthalmologist.
PERSONAL HISTORY
Educated upto class 7th.
Occupation-Cutting clothes
No addiction
Mixed Indian diet
Married,2 daughter and 1 son

FAMILY HISTORY
No h/o similar illness in family members
Father died of Oral cancer
No major neurological illness in family members
SUMMARY
47year male,with 3yr h/o DM,nonHTN,
without any addiction presented with 3yr
h/o insidious onset,gradually progressive
weakness involving distal part of limbs and proximal
weakness for last 3months along with neck & trunk
weakness,associated with thinning & looseness.He had
tingling pain over fingers and toes,& sharp,shooting
pain over upper neck radiating to both shoulders,D-L
spine.He had generalised weight loss(26kg) and
papular,tender skin lesions over
chest,trunk,back,proximal UL,LL.H/O decreased
sweating ,erectile dysfunction and constipation.No
bldder involement .
ANATOMICAL SUBSTRATES

LMN weakness 1)Peripheral nerve


motor ,large sensory fibres
autonomic fibres
2)Motor ,sensory radicles
2)AHC-asymmetrical involvement
-Thinning++
autonomic-uncommon
No twitching
3)Muscle-less likely
4)NMJ-no diurnal variation
PATHOLOGY
Demyelinating-non length dependent
improving course
Axonal
ETIOLOGY
Metabolic
Inflammatory
Infective
Toxin,drugs
Paraproteinemia
Paraneoplastic
Hereditary(least)
GENERAL EXAMINATION
Pallor++/ Jaundice / clubbing - /cyanosis-/edema-
Lymphadenopathy-B/L cervical,axillary,inguinal 2- 3
cm,variable size,discrete,firm,nontender,not fixed to
underlying str or skin,mobile,no sinus
No thyromegaly or prominent neck veins
No oral ulcer
SKIN-nodular eruptions,tender,1 -2 cm,firmly adherent to
skin,no discharge,largest near rt axilla
Rough ,scaly lesions over distal UL
Localised hyperpigmentation & hypertrichosis over lt
shoulder
No skeletal deformity
CHEST-Decreased BS over lower part of
chest posterorly,other-NVBS,Dullness on
percussion over infraaxillary and
infrascapular areas.No added sound
CVS-S1,S2 heard,no murmur
ABDOMEN-No hepatosplenomegaly,No
LN/mass
NERVOUS SYSTEM EXAMINATION

HMF-Conscious,oriented to time,place,person
Speech-normal
Comprehension-normal
CRANIAL NERVES
EOM-full
Pupil-B/L-NSNR
Fundus-CNBT (hazy media)
No facial asymmetry
No tongue weakness,atrophy,fasciculation
Palatal movement-B/L symmetrical
MOTOR:

BULK
RT LT
ARM 25CM 23.5CM
FOREARM 22CM 21.5CM
THIGH 35.5CM 34CM
LEG 28CM 27CM

Atrophy of thenar,hypothenar eminence,1 st dorsal


interossei
TONE-
UL-Normal
LL-Normal
POWER
UL:Prox-5/5
Distal-5/5
small muscles-normal
LL:Prox- 4+/5
Hip abductor-4-/5
Distal-4+/5
Neck-weak
Trunk-weak
DTR-
BJ 2+, TJ 2 +, SJ 2+
KJ 2+, AJ - abs
Plantar-B/L-withdrawal
SENSORY
Pain,temp-decreased below knee(LL)
decreased distal to wrist (UL)
JPS,vibration-normal
Romberg-negative
CEREBELLAR
FNF-N
Heel shin N
GAIT-normal
No nerve thickening
No meningeal sign
Spine ,cranium-normal
AUTONOMIC TEST

Baseline BP-100/70 mm Hg
HR-100/min
STANDING:1min-110/80mmHg
3min-120/80mmHg (N)
Cold pressure (1min30sec)-DBP-90 (N)
Handgrip at 40mm Hg-DBP-70 (Abn)
SWEAT TEST- No sweating (Abn)
INVESTIGATIONS

Hb 7.6 UREA 17
ESR 145
TLC 5500 CREAT 1.12
DC N71L21M5 Na+ 125.9
B1

TPC 4.49lacs/m K+ 5.1


m3
Bil 0.8 Ca2+ 10.5
ALT 55 MCV 85
AST 88 HCT 17.6
ALP 198 PBS anisocytosis
PROTEIN 7.7 Normocytic,
normochromic

ALBUMIN 3.0
FBS 114
INVESTIGATIONS
ICTC-neg
HbSAg-neg
HCV-neg
PLEURAL FLUID
Cell-1380/c.mm
Mononuclear-37%,PMN-19%,
Mesothelial-44%(reactive)
Gramstain,AFB-neg
Protein-0.95g/dl
Sugar-125mg/dl
ADA-20
INVESTIGATIONS

Slit skin smear-No AFB seen


Serum Protein Electrophoresis:
Monoclonal band not seen
Pattern suggestive of chronic
inflammatory disease
USG of abdomen
B/L pleural effusion(R>L)
NCS OF ALL 4 LIMBS
UL-normal latency,amplitude & velocity
LL-normal latency, velocity,amplitude
Sensory-nonrecordable except rt median
H reflex-absent

IMP:Normal motor conduction study,


Predominantly sensory polyradiculoneuro- -
pathy Involving both UL & LL.
MRI CERVICAL SPINE-
Early cervical degeneration with posterior
bulge C3-C4 & C5-C6 i.v disc indenting
thecal sac & exiting recess.
Generalised signal changes from all
vertebra.
MRI BRAIN
Generalised cerebral and cerebellar atrophic
changes,B/L periventricular ischemic
leukoaraiosis.
BIOPSY OF SKIN LESION
Inconclusive

BM ASPIRATION-Dry tap

BIOPSY OF BONE MARROW


Report awaited
FNAC OF LN (post. Cervical)

Small lymphocytes,Plenty of activated


lymphocytes present,tingible body
macrophages absent-suspicious of a
lymphoproliferative disorder.
FINAL DIAGNOSIS
Sensory motor polyradiculo-neuropathy in
a case of lymphoproliferative
disorder(NHL/CLL) & Type2DM
D/D-POEMS syndrome