Stiff person syndrome

Dalakas, Marinos C.; Li, Mian; Fujii, Mavis; Jacobowitz, David M.

doi:10.1212/wnl.57.5.780

Cited by 185 publications

(217 citation statements)

References 25 publications

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“…Since anti-GAD antibodies are unable to cross the blood-brain barrier (18), the intrathecal-produced anti-GAD antibodies not from systemic circulation, are essential for the impairment of CNS. In the present case, anti-GAD-specific IgG index (1.62), an assessment index for intrathecal production of anti-GAD antibody (8), was greater than 1.0, thus lending a strong support for its intrathecal production (8,19). In addition, it has been shown that the characteristics of anti-GAD antibodies are different between type 1 diabetes and SPS: anti-GAD65 antibodies in type 1 diabetes may recognize conformational epitopes in the middle and C-terminus of the protein, whereas in SPS they recognize both the linear and conformational epitopes at Nterminus and C-terminus of GAD65 molecules (9,18,20, and 21).…”

Section: Discussionmentioning

confidence: 76%

Type 1 Diabetes Mellitus and Drug-resistant Epilepsy: Presence of High Titer of Anti-Glutamic Acid Decarboxylase Autoantibodies in Serum and Cerebrospinal Fluid

Yoshimoto¹,

Doi²,

Fukai³

et al. 2005

Intern. Med.

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A 55-year-old man who was diagnosed as having type 1 diabetes mellitus (DM) at the age of 50 years was started on insulin therapy. At 54 years old of age, he suddenly developed complex partial seizures, which frequently occurred despite intensive anti-epileptic drug therapy. Neurological examination on admission revealed hyporeflexia in bilateral upper and lower extremities without any muscle rigidity, painful spasm or cerebellar ataxia. Laboratory examination showed poor glycemic control with increased glycated hemoglobin levels. Positive anti-thyroglobulin antibodies and anti-thyroid peroxidase (TPO) antibodies and slight elevation of TSH levels are consistent with subclinical hypothyroidism due to Hashimoto's thyroiditis. A high titer of anti-glutamic acid decarboxylase (GAD) antibodies was detected in the patient's serum and cerebrospinal fluid (CSF). Electroencephalography showed temporal spikes, consistent with complex partial seizure. This is a very rare case presenting with concomitant type 1 diabetes and drugresistant epilepsy associated with high titers of circulating and CSF anti-GAD antibodies.

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Section: Discussionmentioning

confidence: 76%

Type 1 Diabetes Mellitus and Drug-resistant Epilepsy: Presence of High Titer of Anti-Glutamic Acid Decarboxylase Autoantibodies in Serum and Cerebrospinal Fluid

Yoshimoto¹,

Doi²,

Fukai³

et al. 2005

Intern. Med.

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“…CSF index for LGI1‐IgG and subclasses as compared to serum was calculated according to the Link formula,12 modified for specific antibodies13, 14:

\frac{LGI1 IgG titer in CSF/ LGI1 lgG titer in serum}{CSF Albumin (ng/mL)/ serum Albumin (ng/mL)}

…”

Section: Methodsmentioning

confidence: 99%

Elevated LGI1‐IgG CSF index predicts worse neurological outcome

Gadoth

Ζεκερίδου

Klein

et al. 2018

Ann Clin Transl Neurol

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To determine whether CSF leucine‐rich glioma‐inactivated 1(LGI1)‐IgG titer, index or IgG subclass has prognostic significance, we tested serum and CSF specimens collected concomitantly from 39 seropositive patients. LGI1‐IgG index was elevated (>1) in 21 patients (54%), suggesting intrathecal synthesis. Patients with worse outcome at last follow‐up (modified Rankin Scale >2) had significantly higher index (median 6.57 vs. 0.5, P = 0.048) compared to those with better outcome. Higher CSF LGI1‐IgG4 subclass‐specific titer and index correlated with worse outcome (P < 0.005 for both). These data suggest that evidence of intrathecal LGI1‐IgG synthesis may correlate with neuronal injury and warrant consideration of aggressive immunotherapy.

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“…Also, detectable levels of Immunoglobulin belonging to the subtype IgG were found in the CSF confirming the infiltration of B-cells (Dalakas et al, 2001;Ishii, 2010). The recent epidemiological studies report the existence of anti-gephyrin, anti-amphiphysin and anti-gamma-aminobutyric acid A receptor-associated protein (GABARAP) antibodies in the disease pathology.…”

Section: Stiff-man Syndrome (Sms)mentioning

confidence: 84%