That is why we have set up the Centers of Excellence program. If treated early, most CIDP patients respond well to therapy that can limit the damage to peripheral nerves and contribute to improved function and quality of life and at times can cure the disorder altogether.
Typical CIDP is a symmetrical motor and sensory progressive neuropathy affecting proximal and distal muscles with loss of deep tendon reflexes. Multifocal Motor Neuropathy is a pure motor disorder in which there is asymmetric weakness in the distribution of individual nerves that can be confirmed by diagnostic nerve conduction results.
Lewis-Sumner syndrome is a sensory-motor disorder in which there is sensory loss and weakness in the distribution of individual nerves. Diagnostic nerve conduction studies confirm the focal nerve involvement. Pure sensory CIDP presents with sensory loss, pain, and poor balance with abnormal gait or walking. There is no weakness but frequently motor nerve conduction studies are abnormal in addition to sensory conduction studies. There are other less well established variants most of which would fall into the category of CIDP.
Post-treatment life depends on whether the disease was caught early enough to benefit from treatment options. Patients respond in various ways. The gradual onset of CIDP can delay diagnosis by several months or even years, resulting in significant nerve damage that may limit and delay the response to therapy. The chronic nature of CIDP requires long-term care of patients. Accommodations in the home may be needed to facilitate daily living activities. For your reference, you can access a full 1 hour 45 min presentation on CIDP in the video below.
We encourage anyone who is able to attend an event in person to do so, as lecture content will be consistently updated to include the most recent information. Guillain-Barre syndrome GBS is an acute inflammatory disease of the peripheral nerves. An autoimmune attack on the myelin insulation around individual nerve fibers, called axons results in demyelination. Loss of myelin can occur in sensory, motor or autonomic nerves.
Most patients recover spontaneously, but the recovery can be hastened by plasma exchange or intravenous immunoglobulins. In a small number of patients the inflammation in the nerve can be severe enough to cause degeneration of the whole nerve fibers.
In those patients, the recovery is often slower and incomplete. A chronic form of this illness may present with progressive symptoms and result in CIDP Chronic inflammatory demyelinating polyneuropathy.
GBS patients develop rapidly progressive sensory symptoms such as unusual sensations paresthesias and numbness, and motor symptoms such as weakness and cramping in their legs followed by their arms. Patients may also develop weakness of their breathing and difficulty chewing and swallowing. Difficulty breathing may create a neurological emergency as the patients can develop respiratory arrest. A sizeable number of patients also develop autonomic dysfunction where they experience fluctuations in their blood pressure and cardiac arrhythmias.
GBS is one of the true neurological emergencies. Patients need to be monitored closely during the initial acute phase of the illness. The ultrasound analyses have been performed by AG and NW, blinded to the diagnoses. The interrater ICC was 0. Between May and December , overall 67 patients have been included. In the latter, however, the maximum of disability was reached in between 8 weeks. Altogether, clinical stages ranged from severely affected to mild symptoms in both groups according to the clinical scores used.
The clinical data at onset did not significantly differ between the groups with several therapeutic concepts. One patient with GBS received steroids alone, who was first treated by our department of internal medicine because of initially suggested graft-versus-host disease. As this patient improved spontaneously, no further IVIG was administered. The detailed data are shown in Table 2. Detailed data are shown in Table 3. All patients with GBS revealed hypoechoic class 1 In the GBS patient, the echointensity of the fascicles is hypo- to isoechoic, only the median nerve MN is slightly enlarged—only in the upper arm—regionally restricted 13 mm 2 , cut-off 12 mm 2 , whereas ulnar and fibular nerves UN and FN are not enlarged.
In CIDP II, the MN is enlarged with 25 mm 2 and reveals increased echointensity, and the UN is enlarged at two sites—homogeneously 18 mm 2 in the upper arm B1 with reduced echo signal and 13 mm 2 in the forearm with increased echo signal B.
Nerves are surrounded by white circles; the mentioned fascicles are marked with asterisk symbols. The combination of the electrophysiological sural and ultrasonic sensory nerve sparing pattern therefore showed a positive predictive value for the diagnosis of GBS in Overall, 12 patients 9 with GBS [ The amount of improvement did not differ between the distinct therapeutic groups. Fourteen None had more than 6 points.
A1 and A2 reveal the course in a GBS patient with a significant decrease of nerve root enlargement cervical root 6, C6; 4. Here, the cervical root 6 B1 and B2 5. Treatment options for immune-mediated neuropathies differ depending on subtype. Therefore, a distinction of both—sometimes similarly starting—entities is crucial.
Nerve enlargement is the most intriguing finding in immune-mediated neuropathies in ultrasound, in acute as well as in chronic types. Whereas in CIDP, this enlargement emerges in peripheral nerves, even on a fascicular level, in the pure sensory nerves and in the roots and the vagus nerve, in GBS, this enlargement is found particularly in the roots and the vagus nerve.
Thus, in GBS, the finding of multiple A-waves, the electrophysiological sural sparing next to ultrasonic sensory sparing pattern uSSP , and swelling of the roots and vagus nerve are diagnostic hallmarks. After 6 months, nerve enlargement in GBS often disappears or at least decreases significantly, whereas in CIDP, overall nerve enlargement remains—with heterogeneous development—almost unchanged. The longitudinal clinical process is well resembled by the ultrasound behavior in both groups.
Nerve enlargement is a commonly described finding in immune-mediated neuropathies with a heterogeneous and regionally predominant pattern, particularly in arm nerves and roots in CIDP and variants and marked nerve root and vagus nerve enlargement in GBS [ 5 , 6 , 7 , 16 , 17 , 18 , 19 ]. By defining scores, i. For CIDP, a significant correlation of ultrasonic and clinical course over 12 or more months has been described recently, whereas in GBS, decrease of nerve enlargement over less than 6 months has been shown [ 8 , 9 , 10 , 20 , 21 ].
This, to the best of our knowledge, is the first study describing ultrasound, electrophysiological, and clinical data comparatively over a predefined period of 6 months. MRI of the lumbar spine or ganglioside antibody data showed no significant differences and thus could not contribute for distinction.
The amount of electrophysiological nerve pathology as measured by the NCSS did not differ significantly. This finding is in line with previous results [ 5 , 6 ]. Nevertheless, we must consider that hypoechoic nerve enlargement as a possible sign of edema [ 8 ] predominates in early stages of disease. As pathognomonic for GBS patients, we could therefore highlight sensory nerve enlargement sparing in ultrasound uSSP in most of our patients in contrast to CIDP; herein, pure sensory nerves were mostly regionally enlarged.
Cross-sectional comparison of clinical, ultrasound, and electrophysiological data has no suitable correlation; this is in line with previous data [ 6 , 8 , 10 , 16 ]. After an observational period of 6 months, both groups showed clinical improvement in many patients; however, the median amount of improvement—as measured by MRCSS and INCAT overall disability score—and the intraindividual relative improvement were significantly higher in GBS patients.
In contrast to the cross-sectional data, the longitudinal development of ultrasound, clinical disability, and NCS evaluated as delta correlates significantly with each other, which means the better the clinical recovery is, the larger the decrease of nerve enlargement is and vice versa. Therefore, ultrasound, NCS, and clinical score might serve as follow-up tool over a longer disease course in CIDP, whereas in GBS, early decrease of nerve enlargement is a common finding [ 9 ].
As a limitation we must consider, altogether 12 patients However, significant differences have only been described for few sites of the UPSS [ 14 ]. This difference might be a consequence of the more acute nerve pathology in GBS edema, inflammation, and thus conduction block compared with the more chronic damage in CIDP next to edema and inflammation, pronounced fibrosis and onion bulb formation as well as long distance and multifocal demyelination and axonal damage , which might already arise in early CIDP.
The pathognomonic occurrence of class 2 pattern in CIDP might be a further proof of this assumption as increased echosignal might be a consequence of chronic nerve damage [ 8 ]. These findings might help to discontinue or to continue therapy in cases, in which differentiation might be difficult, if nerve morphology has overall normalized after 6 months or significant enlargement might still arise.
Ultrasound might herald the start of a new era of immunotherapy in neuropathies [ 22 ]. Ultrasound in combination with electrophysiology facilitates the early differentiation between GBS and acute-onset CIDP and serves as an observational tool, whereas CSF analysis and antibody testing do not contribute to differentiation.
As GBS patients show nerve root and vagus enlargement at the beginning in combination with A-waves, and ultrasonic sensory sparing as well as electrophysiological sural sparing pattern, in CIDP, multifocal peripheral nerve enlargement and enlargement of pure sensory nerves seem to be a hallmark; increased echointensity is only found in CIDP.
Further, ultrasound and NCS might be a resource, which could influence ongoing therapeutic steps. These tools therefore might facilitate therapeutic release or continuity.
Latov N. Diagnosis and treatment of chronic acquired demyelinating polyneuropathies.
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