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Recognizing CIDP

Ken Gorson, MD, Neurology, discusses diagnosis of CIDP in a 70-year-old male who presented with tingling hands.

Transcript

Recognizing CIDP

Ken Gorson, MD, Neurology, discusses diagnosis of CIDP in a 70-year-old male who presented with tingling hands.

Hello, my name is Ken Gorson and I'm Professor of Neurology at Tufts University School of Medicine, and would like to present a neuromuscular case.

So this patient was a 70-year-old man, who relates a 6-month history of tingling in his hands. About 6 weeks after this, he started to notice similar symptoms in his feet. Four weeks later he went on to develop leg weakness and recurrent falling. Then in the next 2 weeks he lost the ability to walk. A week prior to admission he developed upper limb weakness, noting trouble with dressing and handwriting.

His past medical history is fairly unremarkable, notable only for non-arteritic optic neuropathy diagnosed 3 years previously and treated with prednisone, and a history of hypertension and degenerative arthritis.

If we stop there, which one of these diagnostic conditions would be considered? Is this the POEMS syndrome? Is it CIDP? Is it multifocal acquired demyelinating sensory and motor neuropathy, or MADSAM, a variant of CIDP? Does the case represent Guillain-Barré syndrome? Or can you not determine based on the evidence provided?

Well, in fact, with just the history alone, one cannot determine that this is likely a case of CIDP. Patients with POEMS syndrome tend to have a much more prolonged course in progression. The patient's symptoms are relatively symmetric, making MADSAM neuropathy unappealing, and the course is too long for typical Guillain-Barré syndrome. So on examination, what we find is that he has generalized limb weakness involving proximal and distal muscles and reduced grip. He has normal bulk and tone, and he has generalized areflexia.

Sensory examination showed diminished pin sensation in the legs extending to the knee, with some hypersensitivity in the fingertips. Vibration was reduced to the distal fingertips, and he had loss of vibration extending from the toes to the feet up to the knees. Joint position was impaired, and he was unable to stand without assistance.

Next question: what other studies would be helpful in establishing a diagnosis? Is it clear what the diagnosis is now from the information you have? Would spinal fluid analysis be helpful? Motor nerve conduction studies? Sensory nerve conduction studies? Or some combination of the above? Well, a combination would be helpful. So, clearly, motor and sensory nerve conduction studies would be appropriate, as would spinal fluid analysis.

Moving on in terms of laboratory data, serologies were negative including testing for HIV and hepatitis C. The patient had no evidence of monoclonal protein on serum immunofixation. His spinal fluid showed just 2 white blood cells, with an elevated protein concentration 85 mg per deciliter, and normal glucose.

This is his nerve conduction data, and the abnormalities are highlighted. What one sees is a prolongation of the distal motor latency of the median nerve, although this could represent just carpal tunnel syndrome. However, there's clear conduction block of the ulnar nerve in the forearm segment. There's a markedly prolonged distal motor latency of the right peroneal motor nerve, and there is substantial conduction velocity swelling in multiple nerves in the demyelinating range.

So here you see on the picture to the right evidence of focal conduction block, with amplitude reduction of the ulnar motor nerve in the forearm segment. The F responses for the median and ulnar nerves were greatly prolonged, and on the sensory studies you see a very classic pattern of so-called sural sparing, with the sural amplitude normal, whereas the medial and ulnar sensory nerve action potentials were absent.

So this is a patient who was diagnosed with CIDP and started on appropriate therapy with IVIG, with a loading dose of 2 gm per kilogram administered over 2 days, followed by maintenance therapy with 1 gm per kilogram every 3 weeks. Within the first several weeks of treatment, the patient started to notice a clear improvement in strength and activities of daily living, and after several more cycles he had a virtually complete response with normal strength and reflexes.

In clinical studies, the most common adverse reactions with GAMUNEX-C were headache, pyrexia, hypertension, chills, rash, nausea, arthralgia, and asthenia (in CIDP). The most serious adverse reactions in clinical studies were pulmonary embolism (PE) in 1 subject with a history of PE (in CIDP).

So in summary, this is a classic pattern of CIDP, manifest by subacute progressive symmetric limb weakness and sensory symptoms and findings. The patient had diffused areflexia. Nerve conduction studies showed evidence of widespread acquired demyelination. Spinal fluid protein level was elevated, and the patient had a terrific response to IVIG.

Thank you for your attention, and I hope this information was helpful to you in managing your patients.

Transcript

Recognizing CIDP

Ken Gorson, MD, Neurology, discusses diagnosis of CIDP in a 70-year-old male who presented with tingling hands.

Hello, my name is Ken Gorson and I'm Professor of Neurology at Tufts University School of Medicine, and would like to present a neuromuscular case.

So this patient was a 70-year-old man, who relates a 6-month history of tingling in his hands. About 6 weeks after this, he started to notice similar symptoms in his feet. Four weeks later he went on to develop leg weakness and recurrent falling. Then in the next 2 weeks he lost the ability to walk. A week prior to admission he developed upper limb weakness, noting trouble with dressing and handwriting.

His past medical history is fairly unremarkable, notable only for non-arteritic optic neuropathy diagnosed 3 years previously and treated with prednisone, and a history of hypertension and degenerative arthritis.

If we stop there, which one of these diagnostic conditions would be considered? Is this the POEMS syndrome? Is it CIDP? Is it multifocal acquired demyelinating sensory and motor neuropathy, or MADSAM, a variant of CIDP? Does the case represent Guillain-Barré syndrome? Or can you not determine based on the evidence provided?

Well, in fact, with just the history alone, one cannot determine that this is likely a case of CIDP. Patients with POEMS syndrome tend to have a much more prolonged course in progression. The patient's symptoms are relatively symmetric, making MADSAM neuropathy unappealing, and the course is too long for typical Guillain-Barré syndrome. So on examination, what we find is that he has generalized limb weakness involving proximal and distal muscles and reduced grip. He has normal bulk and tone, and he has generalized areflexia.

Sensory examination showed diminished pin sensation in the legs extending to the knee, with some hypersensitivity in the fingertips. Vibration was reduced to the distal fingertips, and he had loss of vibration extending from the toes to the feet up to the knees. Joint position was impaired, and he was unable to stand without assistance.

Next question: what other studies would be helpful in establishing a diagnosis? Is it clear what the diagnosis is now from the information you have? Would spinal fluid analysis be helpful? Motor nerve conduction studies? Sensory nerve conduction studies? Or some combination of the above? Well, a combination would be helpful. So, clearly, motor and sensory nerve conduction studies would be appropriate, as would spinal fluid analysis.

Moving on in terms of laboratory data, serologies were negative including testing for HIV and hepatitis C. The patient had no evidence of monoclonal protein on serum immunofixation. His spinal fluid showed just 2 white blood cells, with an elevated protein concentration 85 mg per deciliter, and normal glucose.

This is his nerve conduction data, and the abnormalities are highlighted. What one sees is a prolongation of the distal motor latency of the median nerve, although this could represent just carpal tunnel syndrome. However, there's clear conduction block of the ulnar nerve in the forearm segment. There's a markedly prolonged distal motor latency of the right peroneal motor nerve, and there is substantial conduction velocity swelling in multiple nerves in the demyelinating range.

So here you see on the picture to the right evidence of focal conduction block, with amplitude reduction of the ulnar motor nerve in the forearm segment. The F responses for the median and ulnar nerves were greatly prolonged, and on the sensory studies you see a very classic pattern of so-called sural sparing, with the sural amplitude normal, whereas the medial and ulnar sensory nerve action potentials were absent.

So this is a patient who was diagnosed with CIDP and started on appropriate therapy with IVIG, with a loading dose of 2 gm per kilogram administered over 2 days, followed by maintenance therapy with 1 gm per kilogram every 3 weeks. Within the first several weeks of treatment, the patient started to notice a clear improvement in strength and activities of daily living, and after several more cycles he had a virtually complete response with normal strength and reflexes.

In clinical studies, the most common adverse reactions with GAMUNEX-C were headache, pyrexia, hypertension, chills, rash, nausea, arthralgia, and asthenia (in CIDP). The most serious adverse reactions in clinical studies were pulmonary embolism (PE) in 1 subject with a history of PE (in CIDP).

So in summary, this is a classic pattern of CIDP, manifest by subacute progressive symmetric limb weakness and sensory symptoms and findings. The patient had diffused areflexia. Nerve conduction studies showed evidence of widespread acquired demyelination. Spinal fluid protein level was elevated, and the patient had a terrific response to IVIG.

Thank you for your attention, and I hope this information was helpful to you in managing your patients.


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GAMUNEX®-C (immune globulin injection [human], 10% caprylate/chromatography purified) is indicated for the treatment of primary humoral immunodeficiency disease (PIDD) in patients 2 years of age and older, idiopathic thrombocytopenic purpura (ITP) in adults and children, and chronic inflammatory demyelinating polyneuropathy (CIDP) in adults.

Thrombosis may occur with immune globulin products, including GAMUNEX-C. Risk factors may include: advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling central vascular catheters, hyperviscosity, and cardiovascular risk factors. Thrombosis may occur in the absence of known risk factors. For patients at risk of thrombosis, administer GAMUNEX-C at the minimum dose and infusion rate practicable. Ensure adequate hydration in patients before administration. Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk for hyperviscosity.

Renal dysfunction, acute renal failure, osmotic nephrosis, and death may occur with immune globulin intravenous (IVIG) products in predisposed patients. Patients predisposed to renal dysfunction include those with any degree of preexisting renal insufficiency, diabetes mellitus, age greater than 65, volume depletion, sepsis, paraproteinemia, or patients receiving known nephrotoxic drugs. Renal dysfunction and acute renal failure occur more commonly in patients receiving IVIG products containing sucrose. GAMUNEX-C does not contain sucrose. For patients at risk of renal dysfunction or failure, administer GAMUNEX-C at the minimum concentration available and the minimum infusion rate practicable.

GAMUNEX-C is contraindicated in patients who have had an anaphylactic or severe systemic reaction to the administration of human immune globulin. It is contraindicated in IgA-deficient patients with antibodies against IgA and history of hypersensitivity.

Severe hypersensitivity reactions may occur with IVIG products, including GAMUNEX-C. In case of hypersensitivity, discontinue GAMUNEX-C infusion immediately and institute appropriate treatment.

Monitor renal function, including blood urea nitrogen (BUN), serum creatinine, and urine output in patients at risk of developing acute renal failure.

Hyperproteinemia, increased serum viscosity, and hyponatremia may occur in patients receiving IVIG treatment, including GAMUNEX-C.

There have been reports of aseptic meningitis, hemolytic anemia, and noncardiogenic pulmonary edema (transfusion-related acute lung injury [TRALI]) in patients administered with IVIG, including GAMUNEX-C.

The high-dose regimen (1g/kg x 1-2 days) is not recommended for individuals with expanded fluid volumes or where fluid volume may be a concern.

Because GAMUNEX-C is made from human blood, it may carry a risk of transmitting infectious agents, eg, viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

Do not administer GAMUNEX-C subcutaneously in patients with ITP because of the risk of hematoma formation.

Periodic monitoring of renal function and urine output is particularly important in patients judged to be at increased risk of developing acute renal failure. Assess renal function, including measurement of BUN and serum creatinine, before the initial infusion of GAMUNEX-C and at appropriate intervals thereafter.

Consider baseline assessment of blood viscosity in patients at risk for hyperviscosity, including those with cryoglobulins, fasting chylomicronemia/markedly high triacylglycerols (triglycerides), or monoclonal gammopathies, because of the potentially increased risk of thrombosis.

If signs and/or symptoms of hemolysis are present after an infusion of GAMUNEX-C, perform appropriate laboratory testing for confirmation.

If TRALI is suspected, perform appropriate tests for the presence of antineutrophil antibodies and anti-HLA antibodies in both the product and patient's serum.

After infusion of IgG, the transitory rise of the various passively transferred antibodies in the patient's blood may yield positive serological testing results, with the potential for misleading interpretation.

In clinical studies, the most common adverse reactions with GAMUNEX-C were headache, pyrexia, hypertension, chills, rash, nausea, arthralgia, and asthenia (in CIDP); cough, rhinitis, pharyngitis, headache, asthma, nausea, fever, diarrhea, and sinusitis with intravenous use (in PIDD) and local infusion-site reactions, fatigue, headache, upper respiratory tract infection, arthralgia, diarrhea, nausea, sinusitis, bronchitis, depression, allergic dermatitis, migraine, myalgia, viral infection, and pyrexia with subcutaneous use (in PIDD); and headache, ecchymosis, vomiting, fever, nausea, rash, abdominal pain, back pain, and dyspepsia (in ITP).

The most serious adverse reactions in clinical studies were pulmonary embolism (PE) in 1 subject with a history of PE (in CIDP), an exacerbation of autoimmune pure red cell aplasia in 1 subject (in PIDD), and myocarditis in 1 subject that occurred 50 days post-study drug infusion and was not considered drug related (in ITP).

Please see accompanying full Prescribing Information for GAMUNEX-C.

Terms to know

IG=immune globulin, CIDP=chronic inflammatory demyelinating polyneuropathy, PIDD=primary immunodeficiency disease, ITP=idiopathic thrombocytopenic purpura, Sub Q=subcutaneous, IV=intravenous, ICE=10% caprylate-chromatography purified immune globulin intravenous (IGIV-C) CIDP efficacy.

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