**7. IVIg in therapy of Stiff-Person Syndrome (SPS)**

Stiff-person syndrome (SPS) is characterized by muscle stiffness and episodic spasms. A significant decline of the stiffness scores was found in a randomized trial of 16 SPS patients treated with IVIg . Based on this study IVIg may be considered as a safe and effective second-line therapy for patients with SP incompletely responding to diazepam and or baclofen and who have significant disability requiring a cane or a walker due to truncal stiffness and frequent falls. The recommendation is to use IVIg (2 g kg in 2–5 days) (EFNS task force, 2008).

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348 Autoimmune Disorders – Current Concepts and Advances from Bedside to Mechanistic Insights

tumours, and Hodgkin's lymphoma. Neurological deficits are sometimes preceded by prodromal symptoms, such as a viral-like illness, dizziness, nausea, or vomiting that might be attributed to a peripheral vestibular process. These symptoms are followed by gait unsteadiness that rapidly develops into ataxia, diplopia, dysarthria, and dysphagia. Some patients have blurry vision, oscillopsia, and transient opsoclonus.Initial MRI is normal in most patients, although over time, MRI shows cerebellar atrophy and PET demonstrates

Autoimmune limbic encephalitis (LE) can arise both by paraneoplastic and nonparaneoplastic mechanisms. Patients with LE usually have a subacute onset of memory impairment, disorientation and agitation, but can also develop seizures, hallucinations and sleep disturbance. The following investigations may aid the diagnosis: analysis of cerebrospinal fluid (CSF), electroencephalography, magnetic resonance imaging, fluorodeoxyglucose positron emission tomography and neuronal antibodies in the serum and CSF. Neuronal antibodies are sometimes, but not always, pathogenic. Autoimmune LE may respond to corticosteroids, intravenous IgG (IVIG) or plasma exchange. The cornerstone of paraneoplastic LE therapy is resection of the tumour and/or oncological treatment. Several differential diagnoses must be excluded, among them herpes simplex

Paraneoplastic sensory neuronopathy (SSN) is characterized by primary damage of the sensory nerve cell body of the dorsal root ganglia. A paraneoplastic origin is only one of the causes of SSN The most common low associated tumor is small cell lung carcinoma. The main clinical complains at onset are pain and paresthesias with asymmetric distribution that involves the arms rather than the legs. Later, pain is replaced by numbness, limb ataxia, and pseudoathetotic movements of the hands. The neurologic examination shows abolition of the deep tendon reflexes and involvement of all modalities of sensation with clear predominance of the joint position. Electrophysiologic studies show marked, but not

Evidence for the effect of IVIg in paraneoplastic cerebellar degeneration, limbic encephalitis and sensory neuropathy is scarce. In previously published reports, patients were treated with a combination of immunosupressive or immunomodulatory drugs, including IVIG,


Stiff-person syndrome (SPS) is characterized by muscle stiffness and episodic spasms. A significant decline of the stiffness scores was found in a randomized trial of 16 SPS patients treated with IVIg . Based on this study IVIg may be considered as a safe and effective second-line therapy for patients with SP incompletely responding to diazepam and or baclofen and who have significant disability requiring a cane or a walker due to truncal

restricted, involvement of the sensory fibres (Dalmau & Rosenfeld, 2008).

with a poor response (class IV evidence) (EFNS task force, 2008).

**7. IVIg in therapy of Stiff-Person Syndrome (SPS)** 

to lack of data (EFNS task force, 2008).

hypometabolism (Dalmau & Rosenfeld, 2008).

encephalitis (Vedeler & Storstein, 2009)

**6.6 Paraneoplastic sensory neuronopathy (SSN)** 

**6.5 Limbic encephalitis** 

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