Hyaline Protoplasmic Astrocytopathy in the Setting of Epilepsy.

OBJECTIVES Cerebral hyaline protoplasmic astrocytopathy (HPA) is a clinicopathologic entity characterized by eosinophilic cytoplasmic inclusions within astrocytes. It has been observed in a subset of patients with early-onset epilepsy, brain malformations, and developmental delay. The exact association of this entity with epilepsy is still unknown. This report, with its review of the literature, aims to summarize HPA features to raise awareness regarding this entity. METHODS We report on 2 HPA cases and critically review the literature. RESULTS Approximately 42 cases of HPA have been reported, including the 2 cases presented here, consisting of 23 female and 19 male patients. Patient age ranged from 3 to 39 years. All patients had early-onset seizures (3-20 months of age), ranging from partial to generalized, that were refractory despite treatment with antiepileptic drugs. Postoperative follow-up intervals ranged from 2 to 93 months, and the clinical outcome was graded according to the Engel classification, showing variable results. CONCLUSIONS Clinicians should consider HPA in differential diagnosis in patients with intractable seizures, especially when they are associated with developmental delay and brain malformations. Increasing awareness of this entity among pathologists may promote better understanding of this condition as well as better diagnosis and treatment for these patients.

[1]  M. Bhattacharjee,et al.  Filamin A-negative hyaline astrocytic inclusions in pediatric patients with intractable epilepsy: report of 2 cases. , 2020, Journal of neurosurgery. Pediatrics.

[2]  D. Spencer,et al.  Association of Seizure Spread With Surgical Failure in Epilepsy , 2019, JAMA neurology.

[3]  R. Prayson Hyaline Protoplasmic Astrocytopathy:  A Clinicopathologic Study. , 2016, American journal of clinical pathology.

[4]  R. Prayson Hyaline protoplasmic astrocytopathy with associated focal cortical dysplasia and hippocampal sclerosis. , 2016, Clinical neuropathology.

[5]  L. Hazrati,et al.  Child Neurology: Pediatric seizures with hyaline astrocytic inclusions , 2013, Neurology.

[6]  L. Hazrati,et al.  A proteomic analysis of pediatric seizure cases associated with astrocytic inclusions , 2012, Epilepsia.

[7]  C. Godfraind,et al.  Hyaline astrocytic inclusions in pediatric epilepsy: report of two cases. , 2010, Clinical neuropathology.

[8]  E. Benarroch Astrocyte-neuron interactions , 2009, Neurology.

[9]  R. Schmidt,et al.  Hyaline Protoplasmic Astrocytopathy of Neocortex , 2009, Journal of neuropathology and experimental neurology.

[10]  L. Hazrati,et al.  Astrocytic Inclusions in Epilepsy: Expanding the Spectrum of Filaminopathies , 2008, Journal of neuropathology and experimental neurology.

[11]  G. Heit,et al.  Astrocytic cytoplasmic inclusions within an epileptic focus in an otherwise neurologically intact patient. , 2003, Human pathology.

[12]  H. Lüders,et al.  Proposal for a New Classification of Outcome with Respect to Epileptic Seizures Following Epilepsy Surgery , 2001 .

[13]  K. Maekawa,et al.  Peculiar eosinophilic inclusions within astrocytes in a patient with malformed brain , 1994, Brain and Development.

[14]  J. Engel Update on surgical treatment of the epilepsies , 1993, Neurology.

[15]  A. Sabbagh,et al.  Hyaline eosinophilic cortical astrocytic inclusions in a child with intractable epilepsy , 2016 .

[16]  K. Shioda,et al.  Inclusion bodies in cerebral cortical astrocytes: a new change of astrocytes , 2004, Acta Neuropathologica.

[17]  J. Hunter,et al.  Presence of filamin in the astrocytic inclusions of Aicardi syndrome. , 2004, Pediatric neurology.

[18]  S. Hamano,et al.  Novel eosinophilic inclusion in astrocytes , 2004, Acta Neuropathologica.

[19]  T. Umahara,et al.  Immunohistochemical studies on the new type of astrocytic inclusions identified in a patient with brain malformation , 2004, Acta Neuropathologica.