Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)_1
Short Summary

43-year-old male experienced numbness in both hands. An EMG showed conduction block both on the ulnar and median nerves at the elbow level on both sides, and the clinical examination found sensory loss affecting mainly light touch on both hands. EMG confirmed the presence of nerve conduction blocks, contrasting with apparent preservation of motor strength. The patient was diagnosed with chronic inflammatory demyelinating polyneuropathy and was then put on high doses corticosteroids for several months. A hypocholesterolemizing drug was also introduced, which seemed to coincide with relapse of paresthesias and onset of reduction of hand motility.

Patient's Questions
1)         Do you confirm the diagnosis?
 
2)         What therapy do you suggest? In case the therapy performed is the best one could you advise eventual experimental therapies?
 
3)         Centers of excellence in Italy and/or in Europe?
 
4)         Prognosis?
Medical Background

Age: 43, Sex : Male
Diagnosis: Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)  
Anamnesis:  Family hypercholesterolemia.
                        No other relevant disease.
Case history:
Symptomatic beginning in April 2006 with the appearance of parestesies at distal upper limbs, much more on the right side and more in the median nerve territory. As the clinical picture persisted, electromyography (EMG) was performed with evidence of a demyelinating neuropathy at the upper limbs with the ulnar and median nerve motor conduction block at the elbow, bilaterally.
The patient was, therefore, hospitalized at the Legnano Hospital from for medical tests.
The neurologic exam at admission highlighted: distal hypoesthesia of upper limbs, much more evident for the tactile modality, less evident for the thermodolorific and vibratory one, asymmetric as more marked in the right side and in the median nerve territory.
An EMG was repeated, it confirmed that the demyelinating neuropathy picture, with motor conduction blocks of the upper limbs, remained unchanged.
Among the biohumoral tests performed, serum protein electrophoresis, Reumafactor test, ERS, oncogenic markers, C-reactive protein, anti-Borellia antibodies were within norm limits. On the contrary, these values were altered: cholesterol: 269 mg/dl, triglycerides: 292 md/dl.
Instrumental examinations performed:
ECG: synusal rhythm 64/min.
Chest X-ray: no parenchymal lesions.
Diagnostic rachicentesis with injection needle 22 after local anesthesia with lidocaine 3 cc: the chemical and physical outline was within norm limits (proteins 42.7 mg/dl, 1 cell).
Therefore, the diagnostic conclusion of hospitalization were indicative of:
“the instrumental clinical picture is compatible with a diagnosis of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
in the upper limb demyelinating variant (Upper limb demyelinating CIDP). The presence of symptoms and sensitive objective signs leaves out a multifocal motor neuropathy (MMN)”.
The patient was followed by the specialist in neurology and a therapy with Prednisone 100 mg daily was therefore diagnosed at the patient’s discharge, highlighting the importance of a close longitudinal follow up for possible further medical tests and therapies.
The steroid dosage was gradually reduced in time: 50 mg daily from January 2007; 25 mg daily from May 2007; in July 2007 the dose of 25 mg every other day was reached.
In addition, from mid-June 2007 liponorm for hypocholesterolemizing therapy was introduced. It seemed to accentuate the hand paresthesias, forcing the endocrinologist to recommend the suspension of that medication from July 2007.
After a brief improvement during the therapy with the steroid, the symptoms came back with involvement of both hands, especially of the1st -2nd-4th finger and reduction of the hands motility.
Therefore, in July 2007, the patient decided to contact neurologist at the Negrar Hospital (VR), who asked him to undergo further medical tests:
-           cervical spine and encephalon MRI turned out negative, with the exception of a small discopathy C6/C7.
-           EMG upper limbs with evidence of partial block of the ulnar nerve motor conduction, bilaterally and small block on the median nerve on the right, SAP within norm limits.
The results were substantially comparable to the previous ones, so the patient continued the treatment with deltacortene 5 mg daily without any relevant symptomatic improvement.
In December 2007 EMG was repeated, the results were always the same and the patient continued the treatment with Deltacortene untill May 2008 when at the Borgo Roma Hospital (VR) an intravenous cortisone drip was performed for 1 week. After an improvement lasted 1 month, the symptoms reappeared involving ad worsening the lower limbs too.

Expert's Opinion

Although some improvement occurred after withdrawal of the hypocholesterolemizing drug, but I don’t believe in the responsibility of this drug in recurrence of paresthesias. It is clear to me that relapse actually coincides with reduction of the daily dose of corticosteroids, which was actually unavoidable. During the following months administration of increased doses of corticosteroids induced only partial and transient improvement showing the illness was now resistant to corticosteroids.
I would recommend now to try treatment with high doses intraveinous immunoglobulins (0.4 g/kg per day during 5 consecutive days). This treatment should be repeated every 4 weeks for several months. I don’t think that immunosuppressive therapy is needed.
The patient should be reassessed by an expert in peripheral nerve disorders every six months.
In conclusion:
1.    I confirm the diagnosis of CIDP, but the patient must be closely followed.
2.    Treatment suggested: intraveinous immunoglobulins
3.    Centers of excellence in Italy: 
a.    Dr Ed Nobile-Orazio in Milan
b.    Hopital de la Salpétrière, Paris.
4.    Prognosis: The prognosis is extremely variable: Approximately 75% of the patients improve after treatment. Some of them recover completely; unfortunately it may take years.

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