case study- peripheral neuropathy (nerve care forum)

DR SUDHIR KUMAR MD DM

CONSULTANT NEUROLOGIST

APOLLO HOSPITALS, HYDERABAD

Case Study

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Case StudyPatient 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 StudyEvaluation 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

WastingThenar muscle wasting +Fasciculation- Biceps +; Triceps +Ulnar clawing +

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Case StudyLaboratory investigations

CBC and Electrolytes

Others Urine analysis LFT LIPDS Misc.

Hb- 14TC- 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- 0.45Direct 0.13

LDL- 142 S. Mg- 1.97

Bu- 19 CPK- 137 WBC- 0 AST- 25 TGL- 116 HCV NegativeANA

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 studyWhat do you think???

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Case StudyDiagnosis

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 StudyCourse 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 StudyDiscussion

Introduction

Peripheral neuropathy is one of the most prevalent neurologic conditions encountered by physicians of allspecialties.

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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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 riskfor peripheral neuropathy secondary to a systemic disease, is likely or unlikely tohave 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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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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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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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 StudySymptomatic 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.


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Case StudyConclusion

Peripheral neuropathy is commonly encountered in the primary care setting.

In patients with systemic disease such as diabetes mellitus, peripheral neuropathy can be efficiently identifiedor 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 inseverity 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, orrapidly progressive warrant neurologic consultation.

Neuropathic pain can be effectively treated with an algorithmic approach.


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case study- peripheral neuropathy (nerve care forum)

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