Case study- Peripheral Neuropathy (Nerve Care forum)

DR SUDHIR KUMAR MD DM
CONSULTANT NEUROLOGIST
APOLLO HOSPITALS, HYDERABAD
Case Study

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Case Study
Patient presentation
A 49 year old man A Mechanical Engineer by profession, had weakness & wasting of Bilateral hands 6 months back.
History:
 Initially noticed – inability to hold any object tightly or extend hand properly.
 Followed by wasting of thenar muscles in both hands,
 Gradually noticed, difficulty in buttoning shirt & gripping objects tightly,
 Noticed tremors of fingers
No h/o of fasciculation
No h/o LL symptoms
No h/o other potential weakness /wasting
No h/o bowel, bladder complaint, no h/o of neck pain
After clinical examination and laboratory diagnosis for Motor Neuron Disease (MND) ????
No conclusion arrived and patient was referred to tertiary care centre.

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Case Study
Evaluation at tertiary care hospital
Medical history:
 Patient noticed thinning of the muscles at base of thumbs approximately since 6-7 months
 With disturbed hand writing, needed to apply more force while writing
 Can not button/unbutton shirt, can not mix food, cannot hold objects.
 Can comb hair, can wash hair, can wear clothes
Physical examination:
Pulse Rate- 86/ min
BP- 150/90 mm of Hg
CNS: Conscious, oriented
Speech- Normal
Pupils- B/L 3mm RTL
EOM- full range
No facial weakness/ sensation normal
Tongue/Palate/ Uvula- Normal
Motor tone Normal
Wasting
Thenar muscle wasting +
Fasciculation- Biceps +; Triceps +
Ulnar clawing +

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Case Study
Laboratory investigations
CBC and
Electrolytes
Others Urine analysis LFT LIPDS Misc.
Hb- 14
TC- 8.89
Vit B12 – 196 Sugar- Nil Proteins- 7.31 TC- 194 S. Ca- 8.7
DC-
69.1/23.8/2.4/4.6
TSH- 2.735 Albumin- Nil Albumin- 3.5 HDL- 34 S. PO4- 4.47
FBS- 102 Vit D- 7.10 RBC- 0 Bilirubin- 0.45
Direct 0.13
LDL- 142 S. Mg- 1.97
Bu- 19 CPK- 137 WBC- 0 AST- 25 TGL- 116 HCV Negative
ANA
Creat- 0.81 CD19- 14.88 ALT- 44 Rheumatoid A-
Negative
Na+- 140 CD 20- 17.11 ALP- 71 HBsAg- negative
K+- 4.54 GGT- 39 VDRL- negative
Cl– 103 HIV- negative
ESR- 43 *Autoimmune and Ganglioside IgG antibody tests reports negative
Electrophoresis – Hypoalbuminemia with hyperglobulinemia & increase in alpha 1 & Beta 2 globulins

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Case study
What do you think???

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Case Study
Diagnosis
1. Bilateral Anterior Horn Disease
2. Vitamin B12 deficiency
3. Vitamin D deficiency
With Hypertension
Advise on discharge:
- Tab Aten (50 mg) 1-0-0
- Tab Meconerve (1500 μg) 1-0-0
- Neuro D3 (60k) Friday
- Tab shelcal (1500) 0-1-0
Follow up with neuro OPD

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Case Study
Course of action
While arriving at above diagnosis,
 patient was admitted
 Evaluated clinically
 Autoimmune Paraneoplastic work up sent
 Immunofixation & light chain sent
 EMG done
 Patient discharged along with advise on discharge to follow up with reports in OPD
EMG Report: Median, Ulnar, Peroneal, Tibial, Sural (both UL & LL)- Features suggestive of Severe Motor Axonal Neuropathy
in the upper limb’s right › Left.

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Case Study
Discussion
Introduction
Peripheral neuropathy is one of the most prevalent neurologic conditions encountered by physicians of all
specialties.
The primary care physician is presented with 3 distinct clinical challenges in caring for patients with peripheral
neuropathy:
(1) How to efficiently and effectively screen (in less than 2 minutes) an asymptomatic patient for peripheral neuropathy
when they have a disorder in which peripheral neuropathy is highly prevalent
(eg, diabetes mellitus),
(2) how to clinically stratify patients presenting with symptoms of neuropathy to determine who would benefit from
specialty consultation and what testing is appropriate for those who do not need consultation, and
(3) how to treat the symptoms of painful peripheral neuropathy.
James C. Watson, and P. James B. Dyck, Peripheral Neuropathy: A Practical Approach to Diagnosis and Symptom Management. 2015, Mayo Clin Proc. 90(7):940-951

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Case Study
Discussion
Screening for Peripheral Neuropathy (PN)
The recognition of peripheral neuropathy in patients with disorders, where it is highly prevalent,
may affect the management for that disease.
Annual screening for peripheral neuropathy is recommended in diabetic patients
Most recommendations for office screening for neuropathy:
 light touch perception to a 10-g Semmes-Weinstein monofilament,
 vibration testing with a 128-Hz tuning fork,
 superficial pain (pinprick) perception, or
 testing of ankle deep tendon reflexes
If single-modality screening is used, monofilament light touch or vibration testing appears to be more sensitive and
specific than superficial pain (pinprick) or ankle reflex testing.
Importantly, screening is meant to identify whether an asymptomatic patient, at risk
for peripheral neuropathy secondary to a systemic disease, is likely or unlikely to
have a peripheral neuropathy.
Alone, it is insufficient to fully characterize the neuropathy or direct the necessity of
further diagnostic tests or consultations.

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Case Study
Discussion
EVALUATING PATIENTS PRESENTING WITH CLINICAL SIGNS OR SYMPTOMS SUGGESTING PN
 Clinically Stratifying Patients With Peripheral Neuropathy
 Evaluation of Length-Dependent Peripheral Neuropathies
 Serologic Evaluation
 Diabetic Neuropathy
 Impaired Glucose Tolerance
 Vitamin B12 Deficiency
 Dysproteinaemias
 Other Laboratory Tests
 Toxic Neuropathies
 Hereditary Neuropathies
 Other Diagnostic Tests Nerve Conduction Studies and Electromyography
 Nerve Biopsy

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WHEN TO DO NERVE BIOPSY IN A PATIENT WITH PN?
1. When the neuropathy is progressive,
2. Disease is likely to become disabling or debilitating,
3. Other tests have failed to identify the cause,
4. When a treatable cause is expected (it is better to avoid biopsy,
if we are suspecting toxic or diabetic neuropathy)

Sudhir Kumar, Joe Jacob. Neurology India.2004;52:436-8.12
YIELD OF NERVE BIOPSY IN OUR STUDY

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Case Study
Discussion
 Regardless of clinical pattern of involvement, patients with acute or subacute onset of symptoms or progressive or
functionally limiting neuropathies should be considered for neurologic consultation.
 Similarly, clinicians should refer any patient when there is clinical uncertainty.
Neuropathies in Which Specialty Consultation Would Be Beneficial
 Acute, Subacute in onset
 Rapidly progressive
 Severe, functionally limiting
 Length independent (polyradiculoneuropathy)
 Multifocal
 Motor Predominant
 Associated with severe dysautonomia
Regardless of clinical pattern or affected modality

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Case Study
Discussion
Recommended Evaluation of Chronic Length-Dependent PN
Complete blood cell count
Renal function
Liver function tests
Erythrocyte Sedimentation Rate (extractable nuclear antigen if dry eyes/mouth and sensory neuropathy are present)
Fasting glucose or hemoglobin A1c
Thyroid stimulating hormone
Monoclonal protein (serum protein immunofixation electrophoresis)
Vitamin B12 (with methylmalonic acid)
Infectious (if risk factors or endemic region): Lyme disease, human immunodeficiency virus
Family history of peripheral neuropathy, pes cavus, hammertoes

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Case Study
Symptomatic Management of PN
The primary goal in the evaluation of neuropathy:
1. Identify the etiology and if possible treat the underlying cause.
2. However, even when the neuropathy has a treatable etiology (such as diabetes mellitus, vitamin B12 deficiency, or
toxic exposure),
3. Treatment serves primarily to prevent further progression of the neuropathic symptoms.
4. In these cases and in those in which the neuropathy is idiopathic or untreatable, management is symptomatic.
 One of the most limiting symptoms from peripheral neuropathy is neuropathic pain.
 Among diabetic patients with neuropathy, 11% to 26% have neuropathic pain.
 Several consensus algorithms for the treatment of chronic neuropathic pain have
been proposed and compared.
 Only one has focused explicitly on painful diabetic peripheral neuropathy.

Title of Presentation | DD.MM.YYYY16

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Case Study
Conclusion
 Peripheral neuropathy is commonly encountered in the primary care setting.
 In patients with systemic disease such as diabetes mellitus, peripheral neuropathy can be efficiently identified
or ruled out by screening with a combination of vibration and light touch testing.
 Most peripheral neuropathies are length dependent, sensory predominant, and clinically mild to moderate in
severity without notable functional limitations.
 These neuropathies can usually be effectively worked up and managed without specialty consultation.
 The highest-yield screening is for diabetes mellitus, vitamin B12 with methylmalonic acid, serum protein
electrophoresis, and family history suggesting an inherited neuropathy.
 Neuropathies that are length independent (polyradiculoneuropathies), multifocal, severe, functionally limiting, or
rapidly progressive warrant neurologic consultation.
 Neuropathic pain can be effectively treated with an algorithmic approach.

Case study- Peripheral Neuropathy (Nerve Care forum)

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