Numbness, Tingling, and More Simon Oh, M.D. Colorado Neurology Center 4/10/2015 Approach to Neuropathy History “Listen to the patient. He is trying to tell you what is wrong with him.” - Sir William Osler Approach to Neuropathy History “Listen to the patient. He is trying to tell you what is wrong with him… Unless the patient has everything checked positive on a 14-point review of systems.” - Sir Simon Oh Approach to Neuropathy History nature of the complaints sensory or motor or both? distribution mononeuropathy? multiple mononeuropathies? symmetric polyneuropathy? time course acute onset or insidious onset? rapid progression or slow progression? associated symptoms Location • “just like real estate” – location, location, location Clinical Approach Neurological Examination cranial nerves motor strength tone muscle bulk reflexes sensory modalities distribution coordination gait Clinical Approach Sensory Pattern Dermatomal distribution? Clinical Approach Sensory Pattern Nerve distribution? Clinical Approach Motor Unit What part of the motor unit is involved? Clinical Approach Motor Unit What part of the motor unit is involved? Motor neuron disease? Radiculopathy? Neuropathy? Neuromuscular junction? Myopathy? Case 1 Simon Oh, M.D. Colorado Neurology Center History The patient is a 66-year-old right-handed woman who has had falling problems over the past 2 years. She first fell about 2 years ago. She was in a hurry and she thinks that she may have tripped. She almost dropped her grandchild, but was able to break her fall. She ended up tearing her right rotator cuff which required surgery. Since September 2014, she has been falling more frequently. No dizziness or prodrome. No focal weakness. She thinks that she may trip or stumble. The falling does not occur on a daily basis, but occurs frequently. She has had 4 significant falls and she may have rolled her right ankle once. History She has mild numbness and tingling in her feet. No burning. She has balance problems. When rinsing her hair in the shower, she sometimes has to hold onto the wall for balance. She has occasional muscle cramps, but no focal weakness. She has been diabetic for about 15-20 years. Her last hemoglobin A1c was ~6.0%. Past Medical History 1. 2. 3. 4. 5. diabetes mellitus hypertension gastroesophageal reflux disease restless leg syndrome gout Medications 1. 2. 3. 4. 5. 6. 7. allopurinol 300 mg daily aspirin 81 mg at night losartan 50 mg daily metformin 500 mg twice daily omeprazole 20 mg daily ropinirole 2 mg at night supplements include glucosamine Allergies/Adverse Reactions 1. pregabalin 2. statins, including atorvastatin Social History • • • • married originally from Canada rare alcohol use no tobacco or illicit drug use Family History • no family history of any neurological disorders ROS • • • • • • • • • • • • • • General: No fevers or chills. No fatigue or weakness. No weight change. Eyes: No blurred vision or double vision. No eye pain. ENT: No hearing loss or ringing in the ears. No nosebleeds. No hoarseness or dysphagia. Cardiovascular: No chest pain or palpitations. She has leg swelling. Respiratory: No asthma, chronic cough, or shortness of breath. GI: No nausea or vomiting. No diarrhea or constipation. Genitourinary: No blood in urine. No dysuria or incontinence. Musculoskeletal: No lower back or neck pain. No arthritis. She has muscle cramping. No fasciculations. Skin: No rashes currently. No nail changes or hair changes. Neurologic: No headaches. No seizures. No history of stroke. No speech problems. She has numbness or tingling. No memory loss. No loss of consciousness. Psychiatric: No depression or anxiety. No insomnia. No hallucinations. Endocrine: She has diabetes mellitus. No history of thyroid disorder. Hematologic/lymphatic: No bleeding, bruising, or lymph node swelling. Allergies/immunologic: No hay fever. She has had a drug rash to statins. Physical Exam Vitals: Wt= 154 lbs. Ht= 5’0” BP= 118/74 HR= 71 RR= 20 General: She is well groomed, well appearing, and in no acute distress. HEENT: Normocephalic and atraumatic. No scleral icterus. No conjunctival injection. No ptosis or lid lag. Mucous membranes are moist and the oropharynx is clear. Cardiovascular: Regular, rate, and rhythm. 2+ pedal pulses. Respiratory: Non-labored. Musculoskeletal: Full range of motion. No joint deformities. No clubbing or cyanosis. Skin: No rashes. The skin in the lower extremities is dry. There is some hyperemia in the toes and soles of the feet. Normal skin turgor. Psychiatric: She is euthymic and makes good eye contact. Thought is linear. Hematologic/Lymphatic/Immunologic: No peripheral edema in the lower extremities. Neuro Exam Mental Status: She is alert and has a clear sensorium. Speech is fluent. No dysarthria. Cranial Nerves: Pupils are equal, round, and reactive to light. Extraocular movements are intact with smooth pursuit and no nystagmus. Visual fields are full to confrontation. There is normal facial sensation to light touch and good strength in the muscles of mastication. There is no facial asymmetry or weakness. Hearing is intact to finger rubs bilaterally. The soft palate elevates symmetrically and uvula is midline. There is normal strength in the trapezius and sternocleidomastoid muscles bilaterally. The tongue is midline with appropriate strength. Motor: 5/5 strength in all proximal and distal muscle groups of all 4 extremities. Muscle tone is normal throughout with no cogwheeling or spasticity. There is no muscle atrophy. Reflexes: 2+ biceps, brachioradialis, and triceps reflexes. 1+ patellar and absent ankle reflexes. Sensation: Sensation is intact to light touch throughout. Vibration is decreased in the toes compared to the fingers. A Romberg sign is present. Coordination: Finger-to-nose is normal bilaterally with no tremor or dysmetria. Gait: The gait is normal with normal arm swing. Diagnosis? Evaluation • laboratory testing • EMG/nerve conduction study Evaluation At the initial visit, there was clinical evidence of a peripheral neuropathy. Laboratory testing to look for other etiologies for peripheral neuropathy were ordered. Her B12 level was low at 225. SPEP did not show a monoclonal protein. ESR was normal at 2.0. Her TSH was slightly high at 5.90. EMG/NCS small myelinated fibers (Ad) and unmyelinated C fibers are not assessed by the nerve conduction study despite extensive evaluation, the etiology of a peripheral neuropathy may be unknown in 25-50% of the cases technical factors Nerve Conduction Study Electromyography EMG/NCS • The EMG/nerve conduction study showed evidence of a length-dependent sensory and motor polyneuropathy. EMG/NCS Left Sural SNAP Left Superficial Peroneal SNAP EMG/NCS Left Peroneal CMAP Left Tibial CMAP EMG/NCS Left Radial SNAP Discussion • diabetic polyneuropathy – most common cause of peripheral neuropathy in Western countries – 8% had neuropathy at the time of diagnosis – 50% had neuropathy after 25 years – risk factors: • • • • • glycemic control hypertension smoking obesity high triglycerides Discussion • diabetic polyneuropathy – distribution • usually symmetric with a length-dependent pattern – sensory and motor • injury to nerve axons • initially the toes are affected with numbness and tingling with a slow progression proximally • ~10% report pain (burning, stabbing, or shooting Discussion • diabetic polyneuropathy – autonomic features • • • • • autonomic instability erectile dysfunction bladder dysfunction gastroparesis sweating abnormalities Discussion • B12 deficiency – cofactor for methionine synthase and 1methylmalonyl-coenzyme A mutase – can lead to axonal loss and some demyelination in peripheral nerves – can cause dorsal column damage in the spinal cord • hypothyroidism – associated with a length-dependent sensory polyneuropathy – large-fiber modalities are decreased – decreased reflexes Discussion • iatrogenic causes – metformin • B12-intrinsic factor complex uptake is calcium dependent • metformin affects calcium-dependent membrane action – allopurinol • may cause a sensory neuropathy Follow Up She was started on a low dose of thyroid medication by her PCP. She was also put on B12 injections. She was put on alpha lipoic acid. She has not had any further falls. The paresthesias are better. Case 2 Simon Oh, M.D. Colorado Neurology Center History The patient is a 24-year-old woman with no significant past medical history who has had intermittent lower back pain for the past 2 years. Her achy pain ranges between 5-7/10 in intensity. The pain is exacerbated with high-impact workouts, as well as flexion at the hip. She has gotten some improvement with massage and a lower back injection. No focal weakness in her lower extremities. No muscle cramps or fasciculations. No bowel or bladder dysfunction. She has some mild numbness on the bottom of her feet. The numbness has been occurring for more than 2 months. There were no antecedent illnesses, such as diarrhea or a respiratory infection. She underwent MRI of the lumbar spine without contrast on 7/2/2014 that showed mild degenerative disc disease at L5-S1. Past Medical History • none Medications 1. diclofenac as needed 2. methocarbamol as needed Allergies • NKDA Social History • • • single rare alcohol use no tobacco or illicit drug use Family History • no family history of any neurological disorders Physical Exam Vitals: Wt= 126 lbs. Ht= 5’4” BP= 110/80 HR= 71 RR= 20 O2 sat= 98% RA General: She is well groomed, well appearing, and in no acute distress. HEENT: Normocephalic and atraumatic. No scleral icterus. No conjunctival injection. No ptosis. Mucous membranes are moist and the oropharynx is clear. Cardiovascular: Regular, rate, and rhythm. 2+ radial and pedal pulses. Respiratory: Non-labored. Musculoskeletal: Full range of motion. No joint deformities. No clubbing or cyanosis. Skin: No rashes, lesions, or ulcers. The skin in the lower extremities is not dry. There is hyperemia in the toes of the feet. Normal skin turgor. Psychiatric: She is euthymic and makes good eye contact. Thought is linear. Hematologic/Lymphatic/Immunologic: No peripheral edema in the lower extremities. Neuro Exam Mental Status: She is alert and has a clear sensorium. Speech is fluent. No dysarthria. Cranial Nerves: Pupils are equal, round, and reactive to light. Extraocular movements are intact with smooth pursuit and no nystagmus. Visual fields are full to confrontation. There is normal facial sensation to light touch and good strength in the muscles of mastication. There is no facial asymmetry or weakness. Hearing is intact to finger rubs bilaterally. The soft palate elevates symmetrically and uvula is midline. There is normal strength in the trapezius and sternocleidomastoid muscles bilaterally. The tongue is midline with appropriate strength. No tongue fasciculations. Motor: 5/5 strength in all proximal and distal muscle groups of all 4 extremities. Muscle tone is normal throughout with no cogwheeling or spasticity. There is no muscle atrophy. Reflexes: Absent biceps, brachioradialis, triceps, patellar, and ankle reflexes. Sensation: Sensation is intact to light touch throughout. Vibration is decreased in the toes compared to the fingers. A Romberg sign is present. Coordination: Finger-to-nose is normal bilaterally with no tremor or dysmetria. Gait: The gait is normal with normal arm swing. Diagnosis? Evaluation • EMG/nerve conduction study – electrodiagnostic findings consistent with a demyelinating peripheral neuropathy, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) EMG/NCS Right Sural SNAP Right Superficial Peroneal SNAP EMG/NCS Right Peroneal CMAP Right Tibial CMAP Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) • an acquired immune-mediated neuropathy with demyelinating features • characterized by progressive sensory loss and weakness, affecting proximal and distal limbs Typical CIDP • chronically progressive, stepwise, or recurrent symmetric proximal and distal weakness and sensory dysfunction in all extremities • > 2 months duration • cranial nerves may be affected • reduced or absent DTRs Atypical CIDP • chronically progressive, stepwise, or recurrent in nature – – – – – predominantly distal asymmetric focal pure motor pure sensory • > 2 months duration • reduced or absent DTRs (may be normal in unaffected limbs Epidemiology • rare – affects ~3-8/100,000 – may be underdiagnosed • males more affected – male: female ratio is 2:1 • age – affects patients of all ages – average age is ~50s Epidemiology • can occur with concurrent illness – – – – – – – – – – diabetes mellitus HIV infection connective tissues disorders thyrotoxicosis monoclonal gammopathy of undermined significance chronic hepatitis liver transplantation lymphoma melanoma carcinoma Evaluation • EMG/nerve conduction study – conduction blocks • any drop in CMAP amplitude of 20% – temporal dispersion • any increase in CMAP duration of >15% Evaluation • laboratory testing – CSF protein may be elevated with a normal WBC count • MRI – may show enhancement or enlargement of nerve roots • nerve biopsy Treatment • IVIG • plasmapheresis • steroid therapy Follow Up An EMG/nerve conduction study on 7/26/2014 and it was markedly abnormal due to the presence of diffuse slowing, temporal dispersion, and absent/markedly delayed F-responses. It was felt that she had CIDP. She travelled to Mayo Clinic where the diagnosis was confirmed. She began IVIG in early August 2014. She got 2 g/kg divided over 4 days. She had maintenance doses of 1 g/kg over 1 day. She got headaches and flu-like symptoms after her treatments. She felt like she had gotten weaker and she had been falling. She still had numbness in her legs. She was empirically put her on B12 injections and she felt much better on the daily and weekly doses. Follow Up On a follow up evaluation on 9/18/2014, she had 5/5 strength in all proximal and distal muscle groups of the upper extremities except she had 4-/5 strength with hip flexion and ankle dorsiflexion. She could not stand from a crouched position. Muscle tone was normal throughout. There was no muscle atrophy. Her gait was abnormal due to a steppage gait. Follow Up On a follow up evaluation on 11/29/2014, she had 5/5 strength in all proximal and distal muscle groups of the upper extremities except she had 4-/5 strength with hip flexion and 2/5 with ankle dorsiflexion. She could not stand from a crouched position. Muscle tone was normal throughout. There was no muscle atrophy. Her gait was very abnormal due to a significant steppage gait. A follow up EMG/NCS showed progressive worsening of demyelination. Follow Up IVIG therapy was stopped. She was put on prednisone 60 mg daily. 4 weeks later, she had 5/5 strength with hip flexion and 4-/5 with ankle dorsiflexion. She could stand from a crouched position. Muscle tone and bulk were normal throughout. Her gait was better, but still slow compared to her normal baseline. She was started on methotrexate 15 mg weekly with folic acid. Over the next 2 months, her prednisone was decreased slowly to 10 mg daily. Case 3 Simon Oh, M.D. Colorado Neurology Center History The patient is a very pleasant 31-year-old right-handed woman with a Chiari I malformation who underwent decompression in June 2011. Prior to her surgery, she had weakness in her limbs. She states that she developed weakness in her left hand during her freshman year in college. At that time, she had problems with typing and holding objects. She essentially developed a left wrist drop. She then developed a wrist drop and weakness in her right upper extremity. She did not have any visual disturbances or dysphagia. She was worked up by a another neurologist. She had an “abnormal” EMG/NCS. She apparently also had a muscle biopsy of a left wrist flexor in 2005 and the results were not clear. In the last year and a half, she developed a right foot drop followed by weakness of her left ankle. She has mild numbness and tingling intermittently in her forearms and legs. She has some fasciculations when she is tired. She also has some mild tremors in her hands. Past Medical History 1. Chiari I malformation 2. s/p Chiari decompression surgery in June 2011 3. history of C-sections in 2007 and 2010 Medications 1. Reclipsen oral contraception as directed Allergies • NKDA Social History • • • married 1-2 glasses of wine weekly no tobacco or illicit drug use Family History • no family history of any neurological disorders or muscle disease Physical Exam Vitals: Wt= 113 lbs. Ht= 5’2” BP= 98/68 HR= 86 RR= 16 O2 sat= 99% RA General: She is well groomed, well appearing, and in no acute distress. HEENT: Normocephalic and atraumatic. No scleral icterus. No conjunctival injection. No ptosis. Mucous membranes are moist and the oropharynx is clear. Cardiovascular: Regular, rate, and rhythm. 2+ radial pulses. Respiratory: Non-labored. Musculoskeletal: Full range of motion. No joint deformities. No clubbing or cyanosis. Skin: No rashes. The skin in the lower extremities is not dry. There is no hyperemia in the toes and soles of the feet. Normal skin turgor. Psychiatric: She is euthymic and makes good eye contact. Thought is linear. Hematologic/Lymphatic/Immunologic: No peripheral edema in the lower extremities. Neuro Exam Mental Status: She is alert and has a clear sensorium. Speech is fluent. No dysarthria. Cranial Nerves: Pupils are equal, round, and reactive to light. Extraocular movements are intact with smooth pursuit and no nystagmus. Visual fields are full to confrontation. There is normal facial sensation to light touch and good strength in the muscles of mastication. There is no facial asymmetry or weakness. Hearing is intact to finger rubs bilaterally. The soft palate elevates symmetrically and uvula is midline. There is normal strength in the trapezius and sternocleidomastoid muscles bilaterally. The tongue is midline with appropriate strength. There are no fasciculations or wasting of the tongue. Motor: 4+/5 strength in the deltoids and biceps bilaterally. 5-/5 strength in the triceps bilaterally. 4-/5 with wrist flexion. 3/5 strength with wrist extension and interossei muscles. There is decreased muscle bulk in the wrist extensors in her forearms. 5/5 strength with hip flexion, hip adduction/abduction, knee flexion/extension, and ankle plantarflexion bilaterally. 3/5 strength with ankle dorsiflexion bilaterally. There is decreased muscle mass in the extensor digitorum brevis and abductor hallucis muscles. She has decreased bulk in the anterior tibialis muscles. Tone is slightly decreased. There is no cogwheeling or spasticity. There is no clinical myotonia. No fasciculations are seen on examination. Neuro Exam Reflexes: 1+ biceps, brachioradialis, triceps, patellar, and ankle reflexes. There is no percussion myotonia with percussion of the thenar muscles or the wrist extensors. Plantar responses show downgoing toes bilaterally. Hoffmann’s signs are not present bilaterally. Sensation: Sensation is intact to light touch throughout. Vibration is decreased in the toes compared to the fingers. A Romberg sign is unequivocally present. Coordination: Finger-to-nose shows no dysmetria. There is a terminal tremor bilaterally. Gait: There is a mild steppage gait with both legs. Evaluation • laboratory testing – 2-hour fasting glucose tolerance test, TSH, SPEP, and ESR were within normal limits – B12 was on the lower side of normal at 315 – anti-GM1 Abs were negative • EMG/nerve conduction study – electrodiagnostic findings showed a polyneuropathy, motor with conduction block, demyelinating and axonal – all sensory nerves were normal EMG/NCS Right Sural SNAP EMG/NCS Right Peroneal CMAP Right Tibial CMAP EMG/NCS Right Peroneal CMAP Right Tibial CMAP EMG/NCS Right Radial SNAP EMG/NCS Left Median CMAP Left Ulnar CMAP EMG/NCS Left Radial CMAP Right Radial CMAP Diagnosis? Multifocal Motor Neuropathy (MMN) • an acquired immune-mediated neuropathy with demyelinating features • characterized by progressive asymmetrical weakness with no sensory involvement • restricted to signs of lower motor neuron disease – – – – weakness wasting fasciculations absent or reduced reflexes Epidemiology • rare – affects ~1/100,000 • males more affected – male: female ratio is 3:1 • age – affects patients from 20-80 – average age is ~40s Clinical Presentation • progressive weakness – distal muscles usually affected first • wrist drop • foot drop • asymmetrical presentation – rare to have cranial nerve involvement or respiratory involvement • insidious and slow • other symptoms – muscle cramps and fasciculations – no sensory involvement Examination • weakness – – – – – radial nerve median nerve ulnar nerve musculocutaneous nerve peroneal nerve • reflexes – reduced – no plantar responses or clonus Findings • laboratory testing – anti-GM1 Abs • EMG/NCS – conduction blocks • any drop in CMAP amplitude of 20% Follow Up An EMG/nerve conduction study on 4/3/2012 was markedly abnormal due to the presence of conduction blocks in a number of motor nerves. All sensory nerves were completely normal. It was felt that she had MMN. Follow Up She began IVIG in early May 2012. She got 2 g/kg divided over 5 days. She then had maintenance doses of 1 g/kg over 2 days. Follow Up The patient continues to get better. On 12/18/2014, she had 5/5 strength with neck flexion and extension. 5/5 strength in the deltoids, biceps, and triceps bilaterally. 5-/5 with wrist flexion. 4/5 strength with wrist extension and interossei muscles. 5/5 strength with her finger flexors. There was decreased muscle bulk in the wrist extensors in her forearms. 5/5 strength with hip flexion, hip adduction/abduction, knee flexion/extension, and ankle plantarflexion bilaterally. 4-/5 strength with right ankle dorsiflexion and eversion. 5-/5 strength with left ankle dorsiflexion and eversion. There was decreased muscle mass in the extensor digitorum brevis and abductor hallucis muscles. Tone was slightly decreased. There was no cogwheeling or spasticity. Her gait was improved with less steppage. Questions?
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