Download Pattern Recognition of Neuropathic Disorders

Pattern Recognition of Neuropathic
Disorders
Richard J. Barohn, M.D.
Chair, Department of Neurology
Gertrude and Dewey Ziegler Professor of Neurology
University Distinguished Professor
Vice Chancellor for Research
University of Kansas Medical Center
Kansas City, KS
Pattern Recognition of Neuropathic Disorders
Pattern NP1:
• Symmetric Proximal and Distal Weakness
with sensory loss
• Consider:
•
GBS/CIDP
Dimachkie MM, Barohn RJ, Katz J. Neurol Clin 2013;31(2):533-555;
Dimachkie MM, Barohn RJ. Neurol Clin 2013;31(2):491-510.
Pattern Recognition of Neuropathic Disorders
Pattern NP2:
• Symmetric Distal Sensory Loss With or Without Weakness
• Consider:
• cryptogenic sensory polyneuropathy (CSPN)
• metabolic disorders
― diabetic (DSPN) + IGT
• drugs/toxins
• Sjogren’s, sarcoid
• hereditary
̶ amyloid, Hereditary Sensory Neuropathy (HSN), Fabry’s, others (?
Nav1.7)
̶ CMT – but usually weakness, + sensory signs, no sensory symptoms
• If acute/subacute with pain/weakness – consider vasculitis
• If slow NCS – consider DADS
Pasnoor M, Dimachkie MM, Barohn RJ. Neurol Clin 2013;31(2):463-476; Pasnoor M, Dimachkie MM, Kluding P, Barohn RJ. Neurol Clin 2013;31(2):425-445.
Pasnoor M, Dimachkie MM, Barohn RJ. Neurol Clin 2013;31(2):447-462.
Pattern Recognition of Neuropathic Disorders
Pattern NP3:
• Asymmetric Distal Weakness With Sensory Loss
– Single Nerves/Roots, consider:
• compressive mononeuropathy and radiculopathy
– Multiple Nerves, Consider:
• vasculitis
• HNPP (hereditary neuropathy with pressure palsy)
• infectious (leprosy, lyme, HIV, sarcoid, hepatitis)
• Multifocal Acquired Demyelinating Sensory And Motor (MADSAM)
Neuropathy/Lewis-Sumner
Pattern Recognition of Neuropathic Disorders
Pattern NP4:
• Asymmetric Proximal and Distal Weakness With Sensory Loss
―Consider:
 polyradiculopathy
 plexopathy
 radiculoplexopathy
― Due to:
 diabetes: lumbosacral radiculoplexopathy (DLSRP “amyotrophy”)
 Neoplasia direct invasion
 idiopathic (? dysimmune) – Parsonage–Turner syndrome
 hereditary (HNPP-PMP, HNA-Sept1)
Pasnoor M, Dimachkie MM, Barohn RJ. Neurol Clin 2013;31(2):447-462
Pattern Recognition of Neuropathic Disorders
Pattern NP5:
Asymmetric Distal Weakness Without Sensory Loss
• Consider:
A. With upper neuron findings
1. motor neuron disease/ALS
2. only UMNPLS
B. Without upper motor neuron findings
1.
Progressive muscular atrophy
2.
3.
4.
5.
Multifocal motor neuropathy
Multifocal acquired motor axonopathy (MAMA)
Juvenile monomelic amyotrophy
Polio/post-polio/polio-like
• Brachial amyotrophic diplegia (BAD)
• Leg amyotrophic diplegia (LAD)
• West Nile virus
• Enterovirus 68
Statland JM, Barohn RJ, McVey AL, Katz JS, Dimachkie MM. Neurol Clin 2015;33(4):735-748; Liewluck T, Saperstein DS. Neurol Clin 2015;33(4):761-773;
Jawdat O, Statland JM, Barohn RJ, Katz JS, Dimachkie MM. Neurol Clin 2015;33(4):775-785; Statland JM, Barohn RJ, Dimachkie MM, Floeter MK, Mitsumoto
H. Neurol Clin 2015;33(4):749-760
Pattern Recognition of Neuropathic Disorders
Pattern NP6:
• Symmetric Sensory Loss (With or Without Distal Weakness) and Upper
Motor Neuron Signs
– Consider causes of acquired combined system degeneration with neuropathy:
• B12 deficiency
• Copper deficiency
• End stage liver disease
• Inherited disorders
̶
Adrenomyeloneuropathy
̶
Metachromatic leukodystrophy
̶
Friedreich’s
• 2nd lesion, ex. Cervical spondylosis
Hammond N, Wang Y, Dimachkie MM, Barohn RJ. Neurol Clin 2013;31(2):477-489
Pattern Recognition of Neuropathic Disorders
Pattern NP7:
• Symmetric Weakness Without Sensory Loss
– Proximal and distal
 Spinal Muscular Atrophy
– Only distal
 Hereditary Motor Neuropathy
“Distal SMA”
 CMT can present pure motor but sensory deficits on exam
• Overlap pattern with myopathy/NMJ
Kolb SJ, Kissel JT. Neurol Clin 2015;33(4):831-846.
Pattern Recognition of Neuropathic Disorders
Pattern NP8
• Focal Midline Proximal Symmetric Weakness
– Neck or trunk extensor weakness
• ALS
• Overlap pattern: MG, INEM, ITEM
– Bulbar weakness
• ALS/PLS
• Isolated Bulbar ALS (IBALS)
• Kennedy’s syndrome; X-linked, bulbospinal SMA
• Grunseich C, Fischbeck KH. Neurol Clin 2015;33(4):847-854
• Overlap pattern: MG, OPD
– Diaphragm weakness (SOB)
• ALS
• Overlap pattern: MG, Pompe
Pattern Recognition of Neuropathic Disorders
Pattern NP9:
• Asymmetric Proprioceptive Loss Without Weakness
– Consider sensory neuronopathy due to:







cancer (paraneoplastic)
Sjögren’s syndrome
vitamin B6 toxicity
cisplatinum
HIV-related
Idiopathic
Vitamin E deficiency (probably nerve, not cell body)
• Consider chronic immune sensory polyradiculopathy (CISP)
(Neurology 2004; 63:1662)
Pattern Recognition of Neuropathic Disorders
Pattern NP10:
• Autonomic Dysfunction (ex. Orthostasis, impotence)
– Consider:









diabetes mellitus
amyloidosis (familial & acquired)
Guillain-Barré syndrome
acute autonomic ganglionopathy
Sjögren’s syndrome
Fabry’s
porphyria
HIV-related autonomic neuropathy
idiopathic pandysautonomia
o Nav 1.7 mutation
 paraneoplastic
Clinical Patterns of Neuropathic Disorders
Weakness
Proximal
Distal
+
Asymm
Symm
Sensory
Symptoms
+
+
+
GBS/CIDP
NP2 - Distal sensory loss
with/without weakness
+
+
+
CSPN, metabolic, diabetes,
drugs, hereditary, DADS
NP3 - Asymmetric distal
weakness with sensory loss
+
+
+
Multiple – vasculitis, HNPP,
MADSAM, infection
Single - Mononeuropathy,
radiculopathy
+
+
+
Polyradiculopathy, plexopathy,
DLSRP, cancer, idiopathic,
infection
NP5 - Asymmetric distal
weakness w/out sensory loss
+
+
NP6 – Symmetric sensory
loss & upper motor neuron
signs
+
+
+
+
PATTERN
NP1 - Symmetric prox &
distal weakness w/sensory
loss
NP4 - Asymmetric prox &
distal weakness w/sensory
loss
+
NP7 - Symmetric weakness
without sensory loss*
+\-
NP8 - Focal midline proximal
symmetric weakness*
+ Neck/trunk
extensor
or
+ Bulbar
+ Diaphragm
NP9 – Asymmetric
proprioceptive loss w/out
weakness
NP10 – Autonomic
dysfunction
*Overlap patterns with myopathy and NMJ disorders
+
+
Severe
Proprioceptive
Loss
+
UMN
Signs
Autonomic
Symps/Signs
Diagnosis
+/-
+ UMN – ALS/PLS
- UMN – MMN
+
B12/Copper defic;
Friedreich’s,
ALD
Prox & Distal
SMA
Distal
Hereditary motor neuropathy
+
+
+
+
+
ALS
ALS/PLS
+
Sensory neuronopathy
(ganglionopathy)
CISP
+
Diabetes, GBS, amyloid,
prophyria
Adapted from Barohn RJ, Amato AA,. Neurol Clin 2013;31(2):343-361