Mononeuropathies and Mononeuritis Multiplex

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1 Mononeuropathies and Mononeuritis Multiplex Dr Ashish Bhutada (MD, DM) Shri Markendey Sahakari Rugnalay, Yashodhara Hospital, Solapur

2 Aim of presentation Causes and Pathophysiology in short Clinical presentation of common diseases Mimics and Red flags Diagnosis Treatment Cases Take home message

3 Mononeuropathy A disorder of a single nerve or nerve trunk. Usually Local Cause entrapment, compression, stretch injury, ischemia, infection, inflammation of a nerve Inappropriate Injections Beginning of a systemic disease: Leprosy, Vasculitis

4 Mononeuritis multiplex (Multiple Mononeuropathies) Inflammation of two or more nerves, typically in unrelated parts of the body. Usually due to Systemic disorders infections, Leprosy AIDS, diabetes mellitus; Autoimmune disorders vasculitis, sarcoidosis; connective tissue diseases, such as rheumatoid arthritis, systemic lupus erythematosus, cryoglobulinemia, and Sjogren syndrome. Hereditary: Hereditary neuropathy prone to pressure palsies

5 History Symptoms Pain, parasthesia, Numbness Unnoticed burns, injury Motor weakness Ask where and how did symptoms start Focal, asymmetric, symmetric Acute, subacute Nerve and skin examination Systemic history and examination

6 Case 1 35 year old female c/o parasthesia in right hand extending upto elbow since 2 months Awakens from sleep, worse at night Symptoms are intermittent but daily

7 What is likely etiology? 1. Cervical radiculopathy 2. Carpal tunnel syndrome 3. Ulnar neuropathy 4. SOL in left sensory cortex with focal seizure

8 Tinel s sign positive ESR, Blood sugars, TSH normal

9 Which investigation first? 1. Nerve conduction study 2. MRI cervical spine 3. MRI brain

10 Nerve conduction study NCS: Prolonged latency in right median CMAP and SNAP with preserved amplitude s/o mild median neuropathy at wrist (carpal tunnel syndrome.)

11 How would you treat this case? 1. Surgery 2. Wrist splint in neutral position 3. Gabapentin 4. Steroids 5. Both 2 + 3

12 Carpal tunnel syndrome Carpal tunnel syndrome is most common mononeuropathy of upper limbs The hallmark of classic carpal tunnel syndrome is pain or paresthesia in a distribution that includes the median nerve territory, with involvement of the lateral portion of the hand The symptoms are typically worse at night and characteristically awaken affected individuals from sleep.

13 Carpal tunnel syndrome Conservative therapy involves wrist splinting in neurtral position, Glucocorticoid treatment and surgical release are options for moderate to severe carpal tunnel syndrome that is refractory to more conservative measures. Most CTS cases respond well to conservative management.

14 Proximal median Neuropathies Rare median nerve syndromes include entrapment where the median nerve passes through the pronator teres muscle. Isolated injury to the anterior interosseous nerve that branches off from the median nerve in the region of the elbow. Nerve conduction helps to localise the lesion MRI or USG helps to look for SOL and nature of compression

15 Ulnar Neuropathy at Elbow: Tardy ulnar Ulnar neuropathy at the elbow is the second most common compression neuropathy affecting the upper extremities. In mild cases, symptoms include sensory loss and paresthesias over digits 4 and 5. Mild ulnar neuropathy at the elbow often improves with conservative measures or remains stable. Those with persistent symptoms of weakness and numbness beyond six months should be referred for consideration of surgical intervention. Do not miss Pure neuritic Leprosy Never forget to palpate ulnar on both sides for thickening

16 Ulnar Clawing

17 Radial Neuropathy The radial nerve is predisposed to compression in spiral groove. ( Saturday Night palsy) The triceps retains full strength but there is weakness of the wrist extensors (ie, wrist drop), finger extensors, and brachioradialis. Sensory loss over the dorsum of the hand, possibly extending up the posterior forearm, may also be present. With posterior interosseous neuropathy, forearm pain and weakness of finger dorsiflexion is typical. Conservative treatment is generally the rule for patients with a one-time compression injury to the radial nerve. Consider possibility of neuralgic amyotrophy in case of posterior interosseous neuropathy

18 Wrist drop

19 Do not miss central lesion ( Stroke) for Radial neuropathy Most central disorders that cause arm weakness tend to affect the extensors greater than the flexors. Thus, weakness in extension of the wrist (ie, wrist drop) and fingers may be an ambiguous finding. Relative preservation of triceps strength and sensory loss isolated to the dorsum of the hand are helpful findings indicative of radial neuropathy at the spiral groove Try to hook finger flexors. Check Thumb movements. Check Dexterity

20 Stroke presenting as wrist drop

21 Other mononeuropathies of Upper limb Proximal focal neuropathies of the upper extremity include suprascapular neuropathy, long thoracic neuropathy, and axillary neuropathy. Additional uncommon peripheral nerve syndromes affecting the upper extremities include focal amyotrophy, multifocal motor neuropathy, and zoster radiculoganglionitis.

22 Facial palsy Most common cranial mononeuropathy Do not miss differential diagnosis Call it Bell s palsy only if you rule out other differentials

23 Case 48 year old male, diabetic Acute onset of facial assymmetry No other symptom

24 Case 1 facial: Video

25 What is your disgnosis 1. Bell s Palsy 2. Herpetic facial palsy 3. Hansen s disease 4. Upper motor neuron facial palsy - stroke 5. Lower motor neuron facial palsy- stroke

26 Clinical syndrome Left facial LMN palsy with left gaze paresis Brainstem Stroke/ demyelination

27 Left pontine paramedian infarct

28 Case 50/ male, diabetic Difficulty in closing right eye since last 15 days

29 What is your disgnosis 1. Bell s Palsy 2. Herpetic facial palsy 3. Hansen s disease 4. Upper motor neuron facial palsy - stroke 5. Lower motor neuron facial palsy- stroke

30 Final diagnosis Hansen s Facial neuropathy It is ususlly a partial facial neuropathy with localised sensory loss In partial facial neuropathies, also look for parotid tumor.

31 Case 52/ male / diabetic Severe left facial weakness

32 What is your disgnosis 1. Bell s Palsy 2. Herpetic facial palsy 3. Hansen s disease 4. Upper motor neuron facial palsy - stroke 5. Lower motor neuron facial palsy- stroke

33 Final diagnosis Herpez zoaster associated facial palsy Herpetic rash may not be evident at the onset of facial palsy

34 How would you treat severe Bell s 1. Oral Stroids alone palsy? 2. Oral Steroids+ Valacyclovir 3. Oral Steroids + Valacyclovir + Facial nerve stimulation 4. IV methyl prednisolone 1 gm for 3 days

35 Lower extremity mononeuropathies

36 Peroneal Neuropathy The typical clinical presentation of common peroneal neuropathy at the fibular neck is acute foot drop. Localisation important for foot drop Partial scitic neuropathy Radiculopathy

37 Tarsal Tunnel syndrome Tarsal tunnel syndrome refers to tibial nerve compression in the region of the ankles as the nerve passes under the transverse tarsal ligament. Patients typically present with aching, burning, numbness, and tingling involving the sole of the foot, the distal foot, the toes, and occasionally the heel.

38 Sciatic neuropathy Sciatic neuropathy occurs due to a variety of causes. In addition to pain, patients with sciatic nerve injury in the sciatic notch/gluteal region complain of weakness affecting most of the lower leg musculature. Common cause is injection

39 Femoral Neuropathy Femoral neuropathy also occurs due to a number of causes Typically results in weakness involving the quadriceps with sparing of adduction.

40 Meralgia Parasthetica Lateral femoral cutaneous nerve entrapment as it traverses below the inguinal ligament produces the syndrome of meralgia paresthetica. Paresthesia and pain that radiates down the lateral aspect of the thigh toward the knee Diabetes, pregnancy, obesity

41 Obturator neuropathy Mononeuropathies of the obturator nerve are uncommon. Pain, weakness in leg adduction, and sensory loss over a small area in the medial thigh.

42 Lumbosacral plexopathy Lumbosacral plexopathies usually present with diffuse weakness of the affected lower extremity, although a patchy distribution of weakness may occur. The causes are numerous and diverse Diabetic amyotrophy and idiopathic lumbosacral radiculoplexus neuropathy often present with asymmetric, focal onset of pain followed by weakness involving the leg Progression is subacute and is followed by partial recovery in most patients.

43 Case 40 year old male Numbness in left 3 rd to 5 th fingers followed by weakness of hand since 3 months One month later weakness and parasthesia in right 1 st and 2 nd finger followed by hand weakness.

44 Over next one month severe burning in both hands Then develoed parasthesia in lateral aspect of right foot. He also has history of repeated Respiratory problems, intermittent nasal bleed and bloody sputum

47 Clinical examination Neurological : Predominantly distal asymmetric neuropathy affecing all 4 limbs No thickened nerves No hypopigmented skin lesions RS: B/l ronchi ++ Rest within normal limits

48 Labs CBC : Hb 10.8, WBC 8900 ( N: 51%, L: 30%, E:14%) ESR: 78mm BSL random 120mg% Creatinine 1.0 mg% LFT Normal HIV, HbSAg, HCV negative Urine Routine normal

49 What is the likely diagnosis? 1. Pure Neuritic Hansen s disease 2. Toxic Neuropathy 3. Vasculitis 4. Nutritional ( B12, thiamine defficiency)

50 Special Investigations P-ANCA positive Elevated serum IgE levels by 4 fold

51 Final diagnosis Vasculitic neuropathy- Churg strauss Disease

52 Case 45 Year old female. Parasthesiae in soles of both feet and bilateral foot drop in AUG ` days later developed parasthesiae and weakness in right ulnar nerve distribution.

53 On Examination (Aug 14) Thickened nerves (R ulnar, Both CP, SP & sural nerves). No skin patch. Trophic changes in the right hand. Motor sensory mononeuritis multiplex involving both the common peroneal nerves and right ulnar nerves. Proprioception and DTR normal.

54 Clinical Impression Motor sensory mononeuritis multiplex Most likely due to leprosy (thickened nerves, normal proprioception and preserved DTRs).

55 Investigations (Aug 2014) ESR: 74 mm at the end of 1 hr CBC, LFT &RFT: Normal ANA, ds DNA & ANCA: Negative RA FACTOR, HBsAg: Negative HIV & VDRL: Negative. SSS: Negative

56 Electrophysiology Suggestive of an axonal mononeuritis multiplex involving right sided median, ulnar & bilateral common peroneal, tibial and sural nerves.

57 Left Sural Nerve Biopsy Marked perineural fibrosis. Mild perineural lymphocytic infiltrate. Two vessels showing lymphocytic vasculitis. Fite- Faracco stain for lepra bacilli- Positive.

58 Fite- Faracco stain for lepra bacilli- Positive

59 Diagnosis Mononeuritis Multiplex Due To Hansen s Disease.

60 Treatment (Aug 14) She was treated with multi bacillary treatment for Hansen's disease for 1 year. She received a short course of oral prednisolone during initiation of her MB therapy. Remained well, neuropathic features improved

61 New Problems.. (Dec 15) Though her neuropathy (sensory component) had improved, she developed dry gangrene of both her feet.

62 On examination: Both the dorsalis pedis pulsations were absent. She still had bilateral foot drop & weakness in right ulnar nerve distribution with thickened nerves.

63 Investigations (Dec 15) ESR: 87 mm at the end of 1 hr. ANA: positive (1:40 dilution). Anti Cardiolipin antibody: Positive (IgG-14.5; IgM-5.5). Anti Cardiolipin antibody repeated after 2 months was positive (IgG).

64 Investigations (Dec 15) Lupus Anticoagulant (PT, aptt, Mixing studies, DRVVT) & β2 GPI antibody were negative (IgG-0.8; IgM-2.4). Protein C, protein S, anti thrombin III & Factor V Leiden: Normal. Sr Homocysteine, FBS, C3, C4, Lipid profile: Normal. ANCA: Negative. 2D ECHO: Normal.

65 Colour Doppler Of Lower Limbs Revealed absent flow in the right dorsalis pedis A. with diffuse intimal thickening in all the arteries.

66 Diagnosis Anti- Phospholipid Antibody Syndrome (Apla): Secondary To Hansen`s Disease.

67 Treatment (Dec 15) Started on LMWH followed by warfarin. She however required amputation of her gangrenous toes.

68 APLA Syndrome Anti phospholipid antibodies (apl): A heterogeneous group of antibodies which have been reported in the setting of autoimmune disease and also infections. The syndrome can occur in its primary form or secondarily in association with other autoimmune or infectious diseases.

69 APLA In Infections In infections, antibodies are often transient & can disappear after treatment of the infection. First described in a patient with lepromatous leprosy by Cole et al (1979). In patients with leprosy, acl are reported to be raised in 37 78%, anti-ß2gpi in 3 19% and LA up to 69% [Lupus 2000].

70 Final Diagnosis. Leprous neuropathy with APLA syndrome.

71 Never miss DM, Thyroid, Hansens.. Potentially treatable

72 Take Home message Careful history, detailed neurological symptom and systemic examination remains most important for evaluation of any neuropathy Always ask how and where the first symptom start in a patient who has come with advanced neuropathy NCS and imaging important for diagnosis. Do not miss central lesions in case of suspected mononeuropathies. It can be life threatening.

73 Take Home message Do not miss Leprosy in any neuropathy. Examine for thickened nerves in all cases. Entire skin examination needs to be done in natural sunlight to look for Leprosy skin lesions. Chronic infections like leprosy can trigger immunological dysfunction to give arthritis/ vasculitis

74 Acknowledgement Dr. Satish Khadilkar Dr. Girish Soni Dr. Chetan Chaudhari Dr. Abhinay Huchche Dr. Kaumil Kothari Dr. Bhagyadhan Patel

75 Thank You

Year 2004 Paper one: Questions supplied by Megan

Year 2004 Paper one: Questions supplied by Megan QUESTION 47 A 58yo man is noted to have a right foot drop three days following a right total hip replacement. On examination there is weakness of right ankle dorsiflexion and toe extension (grade 4/5).