Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Associated With Area Postrema Syndrome: A Case Report

Glial fibrillary acidic protein astrocytopathy is an immunotherapy-responsive autoimmune disease of the central nervous system with various clinical manifestations; among these, there are few reports about area postrema syndrome (APS). The authors present the case of a female patient admitted to the hospital with intractable nausea and vomiting as the predominant symptom. The patient's cerebrospinal fluid was tested by cell-based assays (CBA) and found positive for the presence of anti-glial fibrillary acidic protein (GFAP) antibody, in addition, serological testing showed elevated levels of thyroglobulin and thyroperoxidase-specific antibodies. Brain and cervical MRI showed abnormally high signal on the T2 sequence in the dorsal medulla oblongata and right pontine arm. Therefore, the patient was diagnosed with autoimmune GFAP astrocytopathy. The symptoms improved rapidly after treatment with corticosteroids, and no recurrence has been observed thus far. APS may be a relatively rare clinical manifestation of GFAP astrocytopathy. Importantly, such presentation is challenging to correctly diagnose without typical MRI imaging findings. However, the detection of antibodies in the cerebrospinal fluid or serum may be valuable. Systemic and neurological autoimmunity often coexist, comprehensive antibody screening should be conducted.

Resection of a Medulla Oblongata Hemangioblastoma: 2-Dimensional Operative Video

Hemangioblastomas are benign vascular tumors that can be sporadic or multiple, as part of Von Hippel-Lindau disease. They develop at any level of the central nervous system, with a predilection for the dorsal medulla among brainstem locations. Radical resection of the solid portion of the tumor is the best treatment option.1,2 The resection should be en bloc to avoid uncontrollable intraoperative hemorrhage hindering safe dissection. Preservation of the venous drainage during the progressive dissection of the tumor of the surrounding structures and interruption of numerous small arterial feeders is a tenet for safe surgical resection.3 Once the tumor is completely disconnected, the large draining veins can be coagulated, and the tumor removed. We demonstrate these technical principles in the surgery of a 30-yr-old female with an exophytic hemangioblastoma from the dorsal medulla obstructing the fourth ventricle outflow. We demonstrate the resection of this lesion through a suboccipital craniotomy in a sitting position.4 The patient consented to the surgery and publication of images. Image at 1:26 from Kadri and Al-Mefty,4 by permission from the Congress of Neurological Surgeons.

Chronic stimulation of group II metabotropic glutamate receptors in the medulla oblongata attenuates hypertension development in spontaneously hypertensive rats

Baroreflex dysfunction is partly implicated in hypertension and one responsible region is the dorsal medulla oblongata including the nucleus tractus solitarius (NTS). NTS neurons receive and project glutamatergic inputs to subsequently regulate blood pressure, while G-protein-coupled metabotropic glutamate receptors (mGluRs) play a modulatory role for glutamatergic transmission in baroreflex pathways. Stimulating group II mGluR subtype 2 and 3 (mGluR2/3) in the brainstem can decrease blood pressure and sympathetic nervous activity. Here, we hypothesized that the chronic stimulation of mGluR2/3 in the dorsal medulla oblongata can alleviate hypertensive development via the modulation of autonomic nervous activity in young, spontaneously hypertensive rats (SHRs). Compared with that in the sham control group, chronic LY379268 application (mGluR2/3 agonist; 0.40 μg/day) to the dorsal medulla oblongata for 6 weeks reduced the progression of hypertension in 6-week-old SHRs as indicated by the 40 mmHg reduction in systolic blood pressure and promoted their parasympathetic nervous activity as evidenced by the heart rate variability. No differences in blood catecholamine levels or any echocardiographic indices were found between the two groups. The improvement of reflex bradycardia, a baroreflex function, appeared after chronic LY379268 application. The mRNA expression level of mGluR2, but not mGluR3, in the dorsal medulla oblongata was substantially reduced in SHRs compared to that of the control strain. In conclusion, mGluR2/3 signaling might be responsible for hypertension development in SHRs, and modulating mGluR2/3 expression/stimulation in the dorsal brainstem could be a novel therapeutic strategy for hypertension via increasing the parasympathetic activity.

Evidence of intermediate reticular formation involvement in swallow pattern generation, recorded optically in the neonate rat sagittally sectioned hindbrain

For the first time, medullary networks that control breathing and swallow are recorded optically. Episodic swallows are induced via electrical stimulation along the dorsal medulla, in and near the NTS, during spontaneously occurring fictive respiration. These findings establish that networks regulating both orofacial behaviors and breathing are accessible for optical recording at the surface of the sagittally sectioned rodent hindbrain preparation.

Wernicke’s Encephalopathy: Typical Disease with an Atypical Clinicoradiological Manifestation

Wernicke’s encephalopathy is an acute neurological disorder caused due to thiamine deficiency. It is characterized by a triad of confusion, ataxia, and ophthalmoplegia. Wernicke’s encephalopathy can present with atypical clinical manifestations, particularly in setting of nonalcoholic background. Our patient manifested with clinical complaints of acute drowsiness, speech difficulty, and ataxia. Magnetic resonance imaging of the brain revealed T2 hyperintensity in bilateral medial thalami, globus pallidus, periaqueductal gray matter, dorsal pons, and dorsal medulla. The diagnosis of Wernicke’s encephalopathy was entertained and he well responded to parental thiamine therapy. The clinician should be aware about atypical presentation of Wernicke’s encephalopathy.

An atypical manifestation of lateral medullary syndrome

ABSTRACT Lateral medullary syndrome is a common brainstem stroke associated with a classical triad of Horner’s Syndrome, ipsilateral ataxia and hypalgesia and thermoanasthesia of ipsilateral face. We report a case of a 49-year-old diabetic, non-hypertensive, postmenopausal female who presented with symptoms involving the left dorsal medulla along with right sided hemiparesis and left UMN-type facial palsy. Contralateral hemiparesis was explained by caudal extension of infarct involving the pyramids before decussation at the medulla, known as Babinski–Nageotte Syndrome. UMN-type facial palsy was attributed to involvement of hypothetical supranuclear aberrant corticobulbar fibres of facial nerve which descend down in the contralateral ventromedial medulla, decussate at level of upper medulla and then ascend in the dorsolateral medulla to reach the facial nerve nucleus. Association of these two entities with Wallenberg’s Syndrome have been reported separately in literature, but not together as in this case.