N4N

18 September 2015

4 steps

Approach neuromuscular weakness

Overview

Step 1
Step2
Step 3
Step4

: Symptom defined
: Anatomical diagnosis
: Pathological diagnosis
: Clinical diagnosis

Step 1

-> Step2

-> Step 3

Neuromuscular problem

CNS

A. AMSAN
C. Alcohol
Length dependent
Pinprick loss

H. CMT type 2
A. Acute PAN
C.Amyloidosis
H. HSAN

MND
Early autonomic

Nerve
NMJ
Symmetrical
distal

Motor

-> Step 4

Sensory ataxia

A. SCCA lung
C. Sjogren

Muscle disease
A. AIDP
C. CIDP
H. CMT type 1

Symmetrical
Proximal and distal
Sensory
with/without motor
Vascutitic neuropathy
Nerve
Asymmetrical

Subarachnoid
infiltration

Demyelination
Multiple entrapment

A. Polyarthritis nodusa
A. Tumor infiltrative

C. MMN
H. HNPP

D
M

Step 1: Symptom define

Motor
Negative symptom :
Plegia = 0, Paresis >0
Hyporeflexia
Atrophy
Positive symptom:
Fasciculation
Myotonia

Subtle motor deficit test

Heel walking vs Toe walking

Sciatica S1
Femoral L5

 Sensory : Anterolateral spinothalamic

Negative symptom :
Anesthesia =0 Hypoesthesia > 0
Positive sympom:
Paresthesia
- Spontaneus tingling sensation
- Aberrant activity of large fiber

Dysesthesia
- unpleasant sensations when exposed to touch
- Hyperexcitability of small fiber nerve terminal

Allodynia
- Painful with stimuli which do not normally provoke pain
- Central sensitization

 Sensory : Dorsal corlumn

Negative symptom :
Joint position sense
- Pseudoathetosis
- Positive rhomberg test
Vibration sense
- Tunning fork 128 Hz

 Autonomic
Orthostatic hypotension
Constipation, Urinary incontinence
Early satiety
Impotence
Anhidrosis
Erythromelalgia

Step 2: Anatomical diagnosis

Note:
Preganglionic fiber myelinated
Postganglionic unmyelinated small fiber
Ach receptor of autonomic ganglion

Pure motor
MND
atrophy -> weak -> reflex
Nerves
reflex -> weak -> atrophy

NMJ
not impaired unless repetition

Muscle
weak -> atrophy -> reflex


Polyneuropathy with
dominant motor

Porphyria
Lead
GBS (AMAN)
CMT (Charcot-Marie-Tooth type I)
Diptheria/ Dapsone
HNPP (Hereditary liability pressure palsy)
MMN (Multifocal Motor neuropathy)

Sensory
with/without motor, autonomic
Nerves
Root

No dissociation of sensory loss

Radicular pain
Early hyporeflexia
Root or
Early sensory ataxia

Peripheral nerve
Each nerve function
Confluence -> polyneuropathy

Nerve ending
Dysesthesia

Nerves?

Step 3 : Pathological diagnosis

Pattern
1. Symmetrical -distal
Axonopathy

2. Symmetrical -distal and proximal

Demyelination

3. Asymetrical -distal and proximal

Axonopathy (Mononeuritis multiplex)
Demyelination (Multiple entrapment)

Symmetrical length dependent

1

Progression

Symmetrical distal proximal

2

Progression

Asymetrical (Mononeuritis multiplex)

3

Progression

Pattern of axonopathy
Large fiber
most common: DM, Drug, Deficiency etc...

Painful small fiber neuropathy

2nd common : IFG, HIV etc

Small fiber with autonomic

Sensory ganglinopathy (ataxia)
Mononeuritis multiplex

Autonomic neuropathy

Autoimmune autonomic ganglionopathy

Ab to Achr of autonomic ganglion
GBS ,Autoimmune, Paraneoplastic

Porphyria
Amyloidosis ( familial and primary)
HSAN

6 Pack So Hit
Sensory ganglinopathy

6
Vitamin B6 toxicity
Pack Paraneoplastic anti Hu, Cisplatin
So
Sjogrens syndrome
Hit
HIV, HTLV-1

Idiopathic

Asymmetrical
polyneuropathy
Vasculitic neuropathy
Vasculitis
Diabeic polyradiculoplexopathy
Bruns-Garland syndrome (diabetic amyotrophy)

Subarachnoid infiltration
Multiple entrapment
HNPP

Demyelination
MADSAM
MMN

Progression
Acute (4 wks) to subacute (4-8 wks)
GBS, Paraneoplastic
Chronic (>8 wks to year)
CIDP ,Paraproteinaemic
Hereditary
(definite onset no paresthesia)
CMT, HSAN

Step 4: Clinical diagnosis

Clinical clues

Facial diplegia -> GBS

Pes cavus -> CMT
Shoulder fat pad -> Amyloidosis
Palpable purpura -> vasculitic neuropathy
Mees line -> Lead intoxication
Enlarge nerve-> Leprosy, CMT, Amyloidosis

Host status
Multiple presenatation :
Atypical presentation :
Medication associated :
Special situation
:

DM, HIV, Lymphoma

HIV, Immunosuppress
Cancer, HIV
ICU, ESRD

Take home message 4 steps

Step 1 : S-A-M (Sensory, Autonomic, Motor)
Step 2 : Motor : MND, nerves, NMJ, Muscle
Sensorimotor : Nerves
Step 3 : Pattern

: Sym distal, Sym distal&Prox

Asymetrical
Progression : Acute, Chronic, Hereditary

Step 4 : Clinical clues

Host status

Reference
.
2557 780-798
Adams and Victors principle of clinical
neurology 10th edition 2014