Peripheral Neuropathies - Signs, Symptoms, and Examinations - PDF

Peripheral Neuropathies by Baris Isak PhD, MSc Marmara University Hospital Department of Neurology Istanbul, Turkey 1 [email protected] A poster for infrared therapy in DM-PNP...

Peripheral Neuropathies by Baris Isak PhD, MSc Marmara University Hospital Department of Neurology Istanbul, Turkey 1 [email protected] A poster for infrared therapy in DM-PNP 107 million NA; diabetic 70% suffer DM-PNP PNP 1st cause of hospitalization in DM 100000 ampututed limbs each year 2 [email protected]  Unfold the cube Laboratory Signs Examination Associated Symptoms findings findings Diagnosis ? Neurophysiological evaluation Biopsy 3 [email protected]  What are the signs and symptoms ? 4 [email protected]  The clinical response to motor nerve injury Loss of function Disturbed function “- symptoms” “+ symptoms” Motor nerves Wasting Fasiculations Hypotonia Cramps Weakness Hyporeflexia Orthopedic deformity 5 [email protected]  6 [email protected] www.neuro.wustl.edu/neuromuscular/pics/people/patients/Hands/handatrophymnd3.jpg The clinical response to sensory nerve injury Loss of function Disordered function “- symptoms” “+ symptoms” Sensory ↓ Vibration Paresthesias “Large Fiber” ↓ Proprioception Hyporeflexia Sensory ataxia Sensory ↓ Pain Dysesthesias “Small Fiber” ↓ Temperature Allodynia 7 [email protected] The clinical response to autonomic nerve injury Loss of function Disturbed function “- symptoms” “+ symptoms” Autonomic nerves ↓ Sweating ↑ Sweating Hypotension Hypertension Urinary retention Impotence Vascular color changes 8 [email protected]  Diabetic neuropathy http://www.neuro.wustl.edu/neuromuscular/nother/skel.html#nosteo 9 [email protected] Associated findings 10 [email protected]  Check for deformities.. https://upload.wikimedia.org/wikipedia/commons/b/b0/Kyphoscoliosis_hereditary_sensory_autonomic_neuropathy_III.jpg https://upload.wikimedia.org/wikipedia/commons/e/ed/Charcot-marie-tooth_foot.jpg Check family history... https://www.ndsu.edu/pubweb/~mcclean/plsc431/mendel/hum-ped-rec.gif Check signs/symptoms of metabolic, systemic, vasculitic diseases, and intoxications... https://upload.wikimedia.org/wikipedia/commons/d/d5/Clubbed_thumb.jpg http://ichef.bbci.co.uk/news/624/media/images/78139000/jpg/_78139409_poisoning.jpg 11 [email protected] Neurological Examination 12 [email protected] Sensory Network 2 major pathways Lemniscal system mechanoreception proprioception Spinothalamic system nociception thermoreception visceroception [email protected]  Peripheral Sensory Examination PNP Lemniscal Senses (Deep Spinothalamic Senses sensation) (Superficial sensation) 1. Position sensation 1. Pain 2. Vibration 2. Temprature 3. Romberg 14 [email protected]  Motor dysfunclion/loss Weakenss Atrophy Sensory loss-LF Loss of Position/ vibration -SF Loss of Heat/Pain sensations DTR loss Autonomic symptoms Flushing, Dizziness, Ulcerations Bowel-bladder dysfunction 15 [email protected] Laboratory 16 [email protected]  Laboratory Investigations CBC Uric acid Fasting blood sugar BUN Creatinine TFTs B12 ESR Methylmalonic acid Homocysteine CRP Folate 17 [email protected]  Laboratory- screening for treatable neuropathies B12 LFN> DM SFN & LFN ANA Screen for connective tissue diseases TSH PNP in long course ESR Check malignancies HIV Very painful PNP Medications Chemotherapy 18 [email protected] Neurophysiological Procedures 19 [email protected]  Electrodiagnosis High specifity- moderate sensitivity Validated Useful for screening of Distribution Progression SEPs Nerve conduction studies Electromyography 20 [email protected]  21 [email protected] Motor-NCSs 22 [email protected]  Sensory-NCSs Median Sensory NCS Medial Plantar NCS Sural NCS Dorsal Sural NCS 23 [email protected]  SEPs 24 [email protected]  25 [email protected]  Where is the injury ??? The clues: what modalities are injured which fibers are injured whether axon or myelin (or both) injured. 26 [email protected] what modalities are injured ? 27 [email protected]  Plan of the Nervous System UMN Crt. vv Th vv MS v drg T1-L2 c ^ Lemn < ^ o r > Sy d ^ Spth r. III,VII,IX,X LMN g. S2-4 n. ^ ^ PSy ^ Motor Sensory Autonomic 28 < [email protected]  Theoretical approach... Only LMN SMA, radiculopathies Only Spth and/or Autonomic SFN Only Lemniscal LF-sensory PNP Lemniscal + LMN LF- sensorimotor PNP Spth + Lemniscal Mixed type Sensory PNP LMN+ Spth+ Lemniscal Sensory motor PNP 29 [email protected] which fibers are injured..? 30 [email protected]  Fibre Types Lmns. SpTh All Mod 31 [email protected]  Fibre Types Group Name Diameter (microns) CV (m/s) Function A α 1-20 70-110 Motor Proprioception β 5-10 30-60 Vibration Propriocepton γ 3-6 20-30 Fusimotor Spindles δ 2-5 20-30 Sharp pain B arms EMG: Signs of denervation (acute, chronic) and reinnervation 38 [email protected]  Demyeilinization Hypertrophic nerves on exam NCS: Proximal CMAPs ↓ Distal motor lat↑ (>125%) Global areflexia, weakness CV↓(> sensory deficits w/o much denervation 39 [email protected]  Use the 6 D’s…. 1. What is the distribution of the deficits? 2. What is the duration? 3. What are the deficits (which fibers are involved)? 4. What is the disease pathology (axonal or demyelinating or mixed) 5. Is there an inherited (developmental) neuropathy? 6. Is there drug/toxin exposure? 40 [email protected]  1. What is the distribution of the deficits? Asymmetry 1. Mononeuropathy 2. Mononeuritis multiplex – e.g. vasculitis Symmetric (glove/stocking) = polyneuropathy 41 [email protected]  2. What is the duration? Ask: Acute or Chronic? Most polyneuropathies are chronic – ++months-yrs Acute polyneuropathies e.g. Guillain Barre syndrome Vasculitis Relapses and remissions e.g. Intermittent toxin exposure 42 [email protected]  3. What are the deficits (which fibers affected)? If predominant motor fibers think of: Guillain Barre syndrome Lead toxicity Charcot-Marie-Tooth disease If pure sensory/ severe proprioceptive deficit, think of sensory neuronopathy: Carcinoma (paraneoplastic) Vitamin B6 toxicity If autonomic nerves involved (small fiber) think of: Diabetes Amyloid Drugs like vincristine, ddI, ddC 43 [email protected]  4. What is the disease pathology? The vast majority are axonal. Demyelination a key finding because its causes are relatively few. If demyelination uniform the cause is hereditary. e.g. Charcot-Marie Tooth type I (HMSN) If otherwise unremarkable chronic sensorimotor axonal polyneuropathy… exclude alchohol, diabetes, hypothyroidism, uremia, B12 deficiency & monoclonal gammopathy 44 [email protected]  5. Is there an inherited (developmental) neuropathy? Among the most common! Clues – orthopedic deformities (feet, spine) – long duration – indolent progression – few “positive” symptoms – examine/question the family members! 45 [email protected]  6. Drug or toxin exposure? Demyelinating Axonal e.g. e.g. Glue sniffing Cancer drugs like vincristine Arsenic and paclitaxel Antibiotics like chloroquine, ethambutol, isoniazid and metronidazole Cardiac medications like amiodarone 46 [email protected]  Folding the sides of the cube The 3 questions of clinical neurology… 1. Where is the lesion? 2. What is the etiology? 3. What is the treatment? 47 [email protected]  1. Where is the lesion? 2. What is the etiology? Peripheral Neuropathies Roots Plexus Nerves. 48 [email protected]  Patterns of peripheral neuropathy-Dorsal Root http://enerjigunlugu.net/images/r/guney-kore-ampul--HI-642864.jpg http://enerjigunlugu.net/images/r/guney-kore-ampul--HI-642864.jpg Asymmetrical S One extremity ≥2 nerves Combinations of different nerves innervated by the same dermotome e.g., Ganglionopathy /Avulsion 49 [email protected]  Patterns of peripheral neuropathy-Anterior Root http://enerjigunlugu.net/images/r/guney-kore-ampul--HI-642864.jpg Asymmetrical M One extremity ≥2 nerves Combinations of different nerves innervated by the same myotome E.g., Avulsion/Radiculopathy 50 [email protected]  Patterns of peripheral neuropathy-Plexopathy Asymmetrical S&M One extremity ≥2 nerves Combinations of different nerves innervated by the same trunc- cord Demyelinating E.g., Tm invasion) Axonal E.g., radiation plexopathy 51 [email protected]  Patterns of peripheral neuropathy-Mononeuropathy Asymmetrical S&M 1 extremity 1nerve E.g., Carpal Tunnel syndrome Peroneal Entrapment at fibular head 52 [email protected] Patterns of peripheral neuropathy-Multiple mononeuropathies Asymmetrical S&M ≥2 extremities ≥2 nerves * Non-contagious Demyelinating in entrapment sites HNPP Axonal in non-entrapment sites Vasculitis 53 [email protected]  Patterns of peripheral neuropathy Chronic Length Dependent PNP Symmetrical S&M ≥2 extremities ≥2 nerves Axonal *Contagious Typically length dependent (“stocking-glove”) Associated findings Polyneuropathy is common! 2.4% (8% over 55 years) E.g., Diabetic PNP, systemic diseases, metabolic Disorders, exogenous toxins 54 [email protected] Patterns of peripheral neuropathy-CIDP Symmetrical S < M ≥2 extremities ≥2 nerves Demyelinating Proximal weakness 55 [email protected] Patterns of peripheral neuropathy-MADSAM Asymmetrical S&M ≥2 extremities ≥2 nerves Demyelinating MADSAM is a CIDP variant 56 [email protected] Patterns of peripheral neuropathy-MfMN Asymmetrical M ≥2 extremities ≥2 nerves Demyelinating Almost always in hands and wrists Severe involvement in ulnar inn.muscles but sparing median inn. muscles Weak muscles No Atrophy No pathological reflexes 57 [email protected] Patterns of peripheral neuropathy-MND Asymmetrical M ≥2 extremities ≥2 nerves Axonal Hands / wrists Drop foot Bulbar Split hand Weak muscles Atrophy (SMA,ALS) No atrophy (PLS) DTR↓(SMA) ↑(ALS; PLS) 58 [email protected]  Treatment-Etiology based Diabetes Renal insufficiency Hypothyroidism Vitamin B12 deficiency Systemic vasculitis 59 [email protected]  Treatment-Symptom based Pain Antiepileptic drugs Antidepressants Tramadol 60 [email protected]  Treatment- Preventive and Palliative Weight reduction Foot care Wound care- ulcer care Good shoes Ankle-foot othoses if needed 61 [email protected]  A rough overview.. Entrapment 62