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Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017
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Images courtesy of Sumaira Nabi, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
The term peripheral neuropathy is applied to any disease or injury that leads to dysfunction and damage affecting the peripheral nerves. Peripheral neuropathy can be either acute, with rapid onset and progression, or chronic, with slow onset and progression. Signs and symptoms depend on the type of nerve fibers involved. Sensory involvement can give rise to either negative symptoms (eg, numbness or imbalance) or positive symptoms (eg, burning, itching, tingling, pain, or pins-and-needles sensations). Motor involvement can give rise to symptoms such as muscle atrophy and loss of bulk, weakness, falls, and gait impairment. In severe or advanced neuropathy, there is foot drop, which causes a high-stepping or neuropathic gait.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Neuropathies can be classified in several different ways. One classification is based on the type of nerve fiber affected: motor, sensory, sensorimotor, or autonomic.[1] Another is based on the number of nerves involved and the pattern of involvement: A single nerve may be affected (mononeuropathy), or multiple nerves of the body may be affected symmetrically (polyneuropathy). These conditions can be divided into acute and chronic forms, depending on onset and progression. Histopathologically, neuropathies are classified as large-fiber and small-fiber neuropathies. Electrophysiologically, they are classified as demyelinating, axonal, and mixed varieties.
Image courtesy of Sadaf Khattak, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
A 25-year-old male typist presents with a 3-week history of numbness and tingling of the thumb and index finger of the right hand. These symptoms are worse at night and during early morning. The patient also experiences these symptoms while typing during work. There is no loss of power or manual dexterity. On examination, there is no wasting of the thenar muscles. Grip strength is normal. There is reduced pinprick sensation in the right hand over the thumb, index, and middle fingers. The Tinel sign and the Phalen sign, depicted on slide 4, are positive on the right side. Nerve conduction studies (NCSs) reveal slowing of conduction velocity of the right median sensory nerve across the wrist (shown).
Which of the following is the most likely diagnosis?
Carpal tunnel syndrome (CTS)
Cubital tunnel syndrome
Saturday night palsy
Tarsal tunnel syndrome
Images courtesy of Muhammad Sharoz Hassan, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: A. Carpal tunnel syndrome (CTS).
CTS, the most common compressive mononeuropathy, is due to involvement of the median nerve in the carpal tunnel of the wrist. It is characterized by a peculiar constellation of symptoms and signs, including numbness, tingling, paresthesias, and pain in the median nerve territory of the hand.[2] In advanced cases, the examination may reveal altered sensations in the median nerve distribution and loss of power and manual dexterity. Certain characteristic signs are typically noted. The Tinel sign (left) is elicited by light percussion of the median nerve at the wrist with a reflex hammer. The Phalen sign (upper right) and the reverse Phalen sign (lower right) are demonstrated by flexing the wrist at 90° and holding it for 60 seconds. These maneuvers may produce paresthesias in the median nerve territory in cases of active compression. NCSs may show only sensory nerve changes in mild cases, as on slide 3, whereas motor abnormalities are seen in cases with severe compression.
Image courtesy of Sadaf Khattak, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
A 17-year-old male patient presents with a 10-year history of difficulty in walking and frequent tripping. His family medical history is significant for his elder brother having "stork legs." On examination, he has high-arched feet (pes cavus; yellow arrow) and hammer toes (pink arrow), with bilateral foot drop. When he walks, overlifting and slapping of feet are apparent. The results of NCSs and electromyography (EMG), as seen on slide 6, are consistent with a chronic axonal sensorimotor polyneuropathy.
Which of the following is the most likely diagnosis?
Lumbosacral plexopathy
Deep fibular (peroneal) neuropathy
Charcot-Marie-Tooth (CMT) disease
Muscular dystrophy
Image courtesy of Sumaira Nabi, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: C. Charcot-Marie-Tooth (CMT) disease.
CMT disease is the most common inherited neurologic disorder and is characterized by inherited neuropathies.[3] CMT type 2 is an axonal neuropathy that presents with a slowly progressive distal weakness and muscle atrophy (inverted champagne bottle appearance). The morbidity in CMT disease is mainly due to distal muscle weakness and foot deformities. Pes cavus (high-arched foot) is common in this disorder. The gait is a typical steppage or foot drop gait. Needle EMG in these cases reveals neuropathic motor units with increased duration and amplitude and reduced recruitment pattern with polyphasia, as shown in the slide.
Image courtesy of Sumaira Nabi, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
A 22-year-old man presents with a 5-year history of recurrent painless ulcers of the digits of his hands and feet. During this period, he experienced autoamputation of the distal phalanges of some of his toes and fingers. He has been sustaining bruises and ulcers without any pain and has been noticing the wounds later. The family history is unremarkable. On examination, the patient exhibits a glove-and-stocking pattern of loss of response to temperature and painful stimuli. Touch, position, and vibration senses are intact. Power, deep tendon reflexes, and plantar responses are normal. There is no cranial nerve palsy. Evidence of amputated toes is noted, and an infected ulcer on the sole of the right foot is evident (arrow). The patient has Charcot joints (ankles). On NCS, as shown on slide 8, sensory nerve action potentials are absent, and motor nerve conduction is normal.
Which of the following is the most likely diagnosis?
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: A. Hereditary sensory neuropathy (HSN).
Chronic neuropathies in children are mostly hereditary in nature. HSNs are in fact very rare. Their hallmark features are as follows[4]:
Progressive distal sensory loss with painless ulcers
Infections
Autoamputation of phalanges
Charcot arthropathy
Mild motor involvement may or may not be present. Treatment is supportive and includes foot care and wound prevention. Daily inspection of the feet is recommended. Wounds must be cleaned and aggressively treated to prevent osteomyelitis and consequent amputation. Arthrodesis is recommended for Charcot joints and ankle-foot orthoses (AFOs) for foot drop (if present).
Image courtesy of Sumaira Nabi, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
An 18-year-old female patient presents with a 5-day history of progressive ascending weakness of all limbs. Two weeks previously, she had a diarrheal illness. On examination, she is conscious and oriented with respect to time, place, and person. Cranial nerve examination reveals facial diparesis. The patient has flaccid quadriparesis with a power of 3/5 in all limbs, areflexia, and mute plantar responses. NCSs reveal a demyelinating type of polyneuropathy with prolonged distal latencies (pink arrows), slow conduction velocities, and temporal dispersion (green arrows).
Which of the following is the most likely diagnosis?
Motor neuron disease
Hypokalemic periodic paralysis
Guillain-Barré syndrome (GBS)
Stroke
Images courtesy of Sayed Sultan Emad, MBBS (left), and Hanin Bashar Mustafa, MBBS (right inset).
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: C. Guillain-Barré syndrome (GBS).
GBS is a rapidly progressive, immune-mediated polyneuropathy that classically presents with ascending motor weakness.[5] Many GBS patients have sensory symptoms without any definite objective sensory impairment. Cranial neuropathies may also be seen, the most common of which is facial weakness (either unilateral or bilateral). Other complications include autonomic dysfunction and respiratory insufficiency. In most cases, there is a history of an antecedent event, such as an upper respiratory tract infection or a diarrheal illness (as in this case), 2-4 weeks before the onset of weakness. The diagnosis is mainly clinical and is confirmed by means of electrophysiologic studies. NCSs may show features of a demyelinating or axonal polyneuropathy. There are several variants of GBS, of which the most common is acute inflammatory demyelinating polyneuropathy (AIDP). Immunomodulatory therapy, in the form of either plasmapheresis (left) or intravenous immunoglobulin (IVIg) (inset,right), is administered to hasten recovery.
Image courtesy of Umair Hassan, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
A 55-year-old man presents with a 6-month history of progressive weakness of all limbs. On examination, he is conscious and oriented with regard to time, place, and person. Flaccid quadriparesis is noted, with wasting of small muscles of the hands (eg, the first dorsal interosseous muscle of the right hand; arrow) and feet, hyporeflexia, and mute plantar responses. Sensory examination reveals impaired pinprick sensation, proprioception, and sense of vibration. NCSs reveal significantly prolonged distal latencies and slow conduction velocities in the demyelinating range, as highlighted on slide 12. Conduction block is seen in the left tibial nerve. All F waves are prolonged. Needle EMG reveals chronic neuropathic changes.
Which of the following is the most likely diagnosis?
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: B. Chronic inflammatory demyelinating polyneuropathy (CIDP).
CIDP is an acquired immune-mediated condition that involves both motor and sensory nerves.[6] It can have either a chronic and progressive course or a relapsing and remitting one. Symmetric muscle weakness is noted, and both proximal and distal muscle groups are usually involved. On examination, in addition to motor weakness and attenuation of reflexes, there is objective sensory loss that is due to large-fiber involvement. CIDP may be idiopathic, or it may be associated with HIV infection, plasma cells disorders, or lymphoma. Cerebrospinal fluid (CSF) analysis reveals cytoalbuminologic dissociation in nearly 80% of cases. Findings from NCS and needle EMG are consistent with a chronic demyelinating polyneuropathy. The mainstay of treatment is administration of IVIg or high-dose corticosteroids.
Image courtesy of Sumaira Nabi, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
A 55-year-old man presents with a 1-year history of numbness, tingling, and pain in both lower extremities, as well as a 5-month history of similar symptoms in both upper extremities. He also complains of experiencing progressive asymmetrical weakness in both legs for 2 months, without sphincteric dysfunction. On physical examination, the patient is alert and oriented to time, place, and person. He has a left foot drop (shown) and paraparesis with reflexia and bilateral mute plantar responses. He has glove-and-stocking sensory loss with normal perianal sensation. He has a high-stepping gait. On laboratory analysis, the complete blood count (CBC) and erythrocyte sedimentation rate (ESR) are both normal. The results of NCS and EMG are consistent with a chronic demyelinating polyneuropathy. Bone marrow biopsy, bone scanning, skeletal survey, and urine evaluation for Bence Jones protein yield negative results. Serum protein electrophoresis, as shown on slide 14, reveals a discrete narrow 1.96 g/L spike in the gamma globulin region, consistent with monoclonal protein. Serum immunoglobulin (Ig) M (IgM) levels are markedly elevated, at 13.6 g/L (normal range, 0.4-2.3 g/L).
Which of the following is the most likely diagnosis?
Waldenström macroglobulinemia
Lymphoma
Multiple myeloma
Monoclonal gammopathy of unknown significance (MGUS)
Image courtesy of Sumaira Nabi, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: D. Monoclonal gammopathy of unknown significance (MGUS).
MGUS is the most common of the plasma cell dyscrasias.[7] It is more common in men, and its incidence rises with advancing age. MGUS is typically detected incidentally when patients undergo protein electrophoresis as part of an evaluation for unrelated indications, or it may be diagnosed in the course of a workup for polyneuropathy. Paraproteinemic neuropathies are associated with the presence of M-protein (a so-called M spike) in the serum and constitute approximately 10% of idiopathic polyneuropathies. About two thirds of all paraproteinemic neuropathy cases are believed to be associated with MGUS. Some M-proteins behave as antibodies for components of the myelin sheath and axolemma. Nerve damage from M-proteins may also be secondary to deposition of the amyloid byproduct of the circulating paraprotein. Optimal therapy for MGUS neuropathies has not been established. About one third of patients with IgG or IgA MGUS improve symptomatically within days or weeks of the administration of high-dose IVIg, plasma exchange, or corticosteroid therapy (often in combination with immunosuppressants).
Images courtesy of Medscape (left) and Sumaira Nabi, MBBS (right).
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
A 30-year-old man presents with a 3-year history of numbness and weakness in the upper and lower extremities. He has a history of recurrent ulcerations with autoamputation of digits (right), as well as a history of painless hypopigmented skin patches. On examination, he is conscious and oriented with respect to time, place, and person. Thickening of the right ulnar and posterior auricular nerves is noted. The distal phalanges of fingers on both hands are amputated. Multiple painless hypopigmented skin lesions are evident. Power is 5/5 in all limbs. Reflexes are intact, with mute plantar responses. Sensory examination reveals impaired pinprick sensation in a glove-and-stocking pattern, with intact proprioception and sense of vibration. NCSs reveal a severe sensory polyneuropathy. Skin smear (left) demonstrates acid-fast bacilli (AFB).
Which of the following is the most likely diagnosis?
Sarcoidosis
CIDP
Dermatomyositis
Leprous polyneuritis
Image courtesy of Sumaira Nabi, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: D. Leprous polyneuritis.
Leprosy, or Hansen disease, is a chronic granulomatous systemic disease caused by Mycobacterium leprae. It mainly involves the skin and peripheral nerves. It is one of the most treatable causes of peripheral neuropathy and is quite common in the developing world. The hallmark features are hypopigmented insensate skin patches and thickened peripheral nerves. Peripheral neuropathy is frequently seen.[8] Sensory nerves are affected more, with patients commonly complaining of paresthesias, numbness, and pain. Severe sensory neuropathy often leads to the development of recurrent painless fissures and ulcers with poor wound healing and autoamptutation of phalanges. Motor manifestations appear later and manifest as claw hands and foot drop. In addition to polyneuropathy, mononeuropathy and mononeuritis multiplex may also be seen in leprosy. The diagnosis is mainly clinical, in conjunction with the detection of AFB on skin smear or nerve biopsy. Patients must be educated about meticulous care of their hands and feet in order to prevent wounds and ulcers. If a wound becomes infected, appropriate antibiotic therapy must be promptly initiated. Leprosy itself is treated with a multidrug regimen consisting of rifampin, dapsone, and clofazimine.
Images courtesy of Sayed Sultan Emad, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
A 47-year-old man presents with a 6-month history of burning sensations in the hands and feet, as well as a 2-month history of weakness in all limbs. He has experienced skin changes, onychomycosis, and recurrent skin ulceration, and he has long-standing diabetes mellitus that is poorly controlled. On examination, higher mental functions and cranial nerves are intact. Motor examination reveals a power of 4/5 proximally in the lower and upper limbs and 2/5 distally. Skin changes, ulcers, and onychomycosis are apparent. Deep tendon reflexes are hypoactive. Glove-and-stocking sensory impairment is noted, with loss of vibratory and pinprick sensations. NCS findings indicate a chronic axonal sensorimotor polyneuropathy.
Which of the following is the most likely diagnosis?
Diabetic polyneuropathy
Myopathy
Mononeuritis multiplex
Motor neuron disease
Image courtesy of Sumaira Nabi, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: A. Diabetic polyneuropathy.
Diabetes mellitus is frequently complicated by neuropathy resulting from microvascular damage to the vasa nervosa.[9] Such neuropathy is the most common complication of diabetes and is thought to affect 50% of patients. A number of different neuropathic conditions are observed in this setting, including cranial neuropathies (eg, diabetic third nerve palsy), mononeuropathies (eg, CTS), mononeuritis multiplex, peripheral sensory or sensorimotor polyneuropathies, and the painful diabetic amyotrophy. The diagnosis is clinical, in conjunction with electrodiagnostic studies. Tight glycemic control and meticulous foot care are essential to management. Physiotherapy and neuropathic pain management also provide symptomatic relief.
Images courtesy of Sayed Sultan Emad, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
A 20-year-old man presents with a 4-month history of burning pain and numbness in his right foot, followed by weakness. Within 20 days of the onset of these symptoms, he developed a similar burning sensation and numbness in his left hand, with weakness of hand grip and the wrist. He has a history of joint pain. On examination, higher mental functions and cranial nerves are intact. Motor examination reveals right foot drop and left wrist drop. All deep tendon reflexes are normal, except for the right ankle jerk, which is absent. In the right lower extremity, there is sensory loss on the lateral aspect of the shin and the dorsum of the foot. NCSs show no recordable response in the right peroneal motor and sensory nerve and the left radial motor and sensory nerve. Axonal neuropathy is noted in the left ulnar nerve. Testing for rheumatoid factor yields positive results. Nerve biopsy findings are consistent with vasculitic neuropathy.
Which of the following is the most likely diagnosis?
Mononeuritis multiplex
Saturday night palsy
GBS
L5 radiculopathy
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: A. Mononeuritis multiplex.
Mononeuritis multiplex is a painful asymmetrical motor and sensory peripheral nerve disease that involves damage to two separate nerve areas. Various systemic disorders are implicated in its etiology. The most common cause is an underlying vasculitic neuropathy.[10] In addition to mononeuritis multiplex, asymmetric sensorimotor polyneuropathy is also seen in vasculitis. The workup of mononeuritis multiplex is extensive and includes autoimmune antibody screening, histopathologic evaluation, and electrophysiologic studies. NCSs reveal multiple asymmetric axonal sensory and motor mononeuropathies, with neuropathic findings on needle EMG.
Image courtesy of Spotmatik | Dreamstime.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
A 27-year-old woman presents with a 2-month history of weakness in the lower and upper extremities. She is experiencing difficulty in walking, weakness of hand grip, and loss of manual dexterity. No swallowing abnormality or sphincteric involvement is apparent. Antituberculous therapy was prescribed 4 months previously, after the patient had been diagnosed with pulmonary tuberculosis. On examination, higher mental functions and cranial nerves are intact. Motor examination shows a power of 4/5 proximally in the lower and upper limbs, 3/5 distally in the upper limbs, and 2/5 distally in the lower limbs. Deep tendon reflexes are absent. There is no sensory loss. NCS and needle EMG findings indicate a chronic axonal motor neuropathy, as shown on slide 22. Routine laboratory tests all yield normal results. Isoniazid-induced polyneuropathy is suspected.
Which of the following patients are not more prone to develop peripheral neuropathy due to isoniazid?
Obese patients
Patients who are slow acetylators
Patients with HIV infection
Alcoholic patients
Image courtesy of Sayed Sultan Emad, MBBS.
Peripheral Neuropathy: More Than a (Loss of) Feeling
Sumaira Nabi, MBBS, FCPS Neurology | October 25, 2017 | Contributor Information
Answer: A. Obese patients.
Certain drugs and toxins are known to cause neuropathy. Among these is the antituberculous agent isoniazid (INH), which can cause a severe predominantly motor neuropathy. INH-related peripheral neuropathy is thought to be potentially reversible if treated early with pyridoxine[11]; however, morbidity is high if treatment is not initiated in a timely manner. Susceptibility to this neuropathy is increased in older individuals, those with malnutrition and low body weight, those with chronic alcoholism, those with HIV infection, and those who have the slow acetylator genotype. Pyridoxine (vitamin B6) prophylaxis is especially recommended in these patients. Moreover, patients receiving antituberculous therapy must be educated regarding the potential side effects so that they will be more likely to report complications early on.
During the elegant and complex development of the genitourinary system, a variety of congenital abnormalities can arise. Learn more about how to recognize and address these conditions.Slideshows, September 2017
Neuropathies are characterized by a progressive loss of nerve fiber function. A widely accepted definition of diabetic peripheral neuropathy is "the presence of symptoms and/or signs of peripheral nerve dysfunction in people with diabetes after exclusion of other causes."Diseases/Conditions, October 2017
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Contributor Information
Sumaira Nabi, MBBS, FCPS Neurology
Senior Registrar and Consultant Neurologist
Department of Neurology
Pakistan Institute of Medical Sciences
Islamabad, Pakistan
Disclosure: Sumaira Nabi, MBBS, FCPS Neurology, has disclosed no relevant financial relationships.
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References
Misra UK et al. Diagnostic approach to peripheral neuropathy. Ann Indian Acad Neurol. 2008 Apr;11(2):89-97. [PMID: 19893645]
Lewis RA. Chronic inflammatory demyelinating polyradiculoneuropathy. Medscape Drugs & Diseases. October 2, 2017; Accessed: October 11, 2017. Available at: http://emedicine.medscape.com/article/1172965.
Nabi S et al. Pain, tingling, and numbness in the extremities with foot drop and progressive weakening of the legs. Medscape Drugs & Diseases: Case Challenges. May 19, 2014; Accessed: October 11, 2017. Available at: http://reference.medscape.com/viewarticle/824942.
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