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ILAE type 3 hippocampal sclerosis in patients with anti-GAD-related epilepsy.

Glover RL, DeNiro LV, Lasala PA, Weidenheim KM, Graber JJ, Boro A - Neurol Neuroimmunol Neuroinflamm (2015)

Bottom Line: Following surgery, both developed signs and symptoms of stiff person syndrome and later cerebellar ataxia.Laboratory studies demonstrated high concentrations of anti-GAD antibodies in both patients.These cases suggest that ILAE type 3 hippocampal sclerosis may be immunologically related to and may exist as part of a broader anti-GAD-related neurologic syndrome in some instances.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology (R.L.G., L.V.D., K.M.W., J.J.G., A.B.), Department of Neurological Surgery (P.A.L., K.M.W.), and Department of Pathology (K.M.W.), Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, NY.

ABSTRACT

Objective: To describe the neuropathologic findings and clinical course of 2 patients who underwent temporal lobectomy for medically refractive epilepsy and were later found to have high anti-glutamic acid decarboxylase (GAD) concentrations.

Methods: Small case series.

Results: Neuropathologic examination of both patients revealed International League Against Epilepsy (ILAE) type 3 hippocampal sclerosis. Following surgery, both developed signs and symptoms of stiff person syndrome and later cerebellar ataxia. Laboratory studies demonstrated high concentrations of anti-GAD antibodies in both patients.

Conclusions: These cases suggest that ILAE type 3 hippocampal sclerosis may be immunologically related to and may exist as part of a broader anti-GAD-related neurologic syndrome in some instances.

No MeSH data available.


Related in: MedlinePlus

Case 2(A) Section stained with Luxol fast blue for orientation; CA4 is seen in the center between the blades of the fascia dentata (original magnification ×20). (B) Synaptophysin staining is well preserved. (C) Gliosis was noted in CA4 corresponding to the neuronal loss. This is the pattern of end folium sclerosis. (D) High-power (original magnification ×200) view of the fascia dentata showing dispersion and migration of neurons stained with NeuN.
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Figure 2: Case 2(A) Section stained with Luxol fast blue for orientation; CA4 is seen in the center between the blades of the fascia dentata (original magnification ×20). (B) Synaptophysin staining is well preserved. (C) Gliosis was noted in CA4 corresponding to the neuronal loss. This is the pattern of end folium sclerosis. (D) High-power (original magnification ×200) view of the fascia dentata showing dispersion and migration of neurons stained with NeuN.

Mentions: A 31-year-old man developed complex partial seizures at the age of 22. Serial MRI studies showed progressive left hippocampal atrophy and signal abnormality. His seizures rapidly became intractable to medical therapy. Memory performances on Wada testing were nonlateralizing. He underwent a left temporal lobectomy at the age of 29. Invasive monitoring was done because of concern that the seizure onset zone was neocortical. The initial ictal EEG changes were detected in perihippocampal depth contacts. He underwent left amygdalohippocampectomy. He has been completely free of seizures since. Surgical pathology demonstrated ILAE type 3 HS (figure 2). Immunohistochemical studies using antibodies for T cells (CD3), T cell subsets (CD4 and CD8), B lymphocytes (CD20), plasma cells (CD138), and granzyme were performed and did not reveal lymphocytes or plasma cells in the parenchyma or subarachnoid space.


ILAE type 3 hippocampal sclerosis in patients with anti-GAD-related epilepsy.

Glover RL, DeNiro LV, Lasala PA, Weidenheim KM, Graber JJ, Boro A - Neurol Neuroimmunol Neuroinflamm (2015)

Case 2(A) Section stained with Luxol fast blue for orientation; CA4 is seen in the center between the blades of the fascia dentata (original magnification ×20). (B) Synaptophysin staining is well preserved. (C) Gliosis was noted in CA4 corresponding to the neuronal loss. This is the pattern of end folium sclerosis. (D) High-power (original magnification ×200) view of the fascia dentata showing dispersion and migration of neurons stained with NeuN.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4484895&req=5

Figure 2: Case 2(A) Section stained with Luxol fast blue for orientation; CA4 is seen in the center between the blades of the fascia dentata (original magnification ×20). (B) Synaptophysin staining is well preserved. (C) Gliosis was noted in CA4 corresponding to the neuronal loss. This is the pattern of end folium sclerosis. (D) High-power (original magnification ×200) view of the fascia dentata showing dispersion and migration of neurons stained with NeuN.
Mentions: A 31-year-old man developed complex partial seizures at the age of 22. Serial MRI studies showed progressive left hippocampal atrophy and signal abnormality. His seizures rapidly became intractable to medical therapy. Memory performances on Wada testing were nonlateralizing. He underwent a left temporal lobectomy at the age of 29. Invasive monitoring was done because of concern that the seizure onset zone was neocortical. The initial ictal EEG changes were detected in perihippocampal depth contacts. He underwent left amygdalohippocampectomy. He has been completely free of seizures since. Surgical pathology demonstrated ILAE type 3 HS (figure 2). Immunohistochemical studies using antibodies for T cells (CD3), T cell subsets (CD4 and CD8), B lymphocytes (CD20), plasma cells (CD138), and granzyme were performed and did not reveal lymphocytes or plasma cells in the parenchyma or subarachnoid space.

Bottom Line: Following surgery, both developed signs and symptoms of stiff person syndrome and later cerebellar ataxia.Laboratory studies demonstrated high concentrations of anti-GAD antibodies in both patients.These cases suggest that ILAE type 3 hippocampal sclerosis may be immunologically related to and may exist as part of a broader anti-GAD-related neurologic syndrome in some instances.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology (R.L.G., L.V.D., K.M.W., J.J.G., A.B.), Department of Neurological Surgery (P.A.L., K.M.W.), and Department of Pathology (K.M.W.), Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, NY.

ABSTRACT

Objective: To describe the neuropathologic findings and clinical course of 2 patients who underwent temporal lobectomy for medically refractive epilepsy and were later found to have high anti-glutamic acid decarboxylase (GAD) concentrations.

Methods: Small case series.

Results: Neuropathologic examination of both patients revealed International League Against Epilepsy (ILAE) type 3 hippocampal sclerosis. Following surgery, both developed signs and symptoms of stiff person syndrome and later cerebellar ataxia. Laboratory studies demonstrated high concentrations of anti-GAD antibodies in both patients.

Conclusions: These cases suggest that ILAE type 3 hippocampal sclerosis may be immunologically related to and may exist as part of a broader anti-GAD-related neurologic syndrome in some instances.

No MeSH data available.


Related in: MedlinePlus