Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report.

Background and Objectives: Sensory ganglionopathy is a rare neurological disorder caused by degeneration of the neurons composing the dorsal root ganglia. It manifests as various sensory disturbances in the trunk, proximal limbs, face, or mouth in a patchy and asymmetrical pattern. Harlequin syndrome is characterized by unilateral flushing and sweating of the face, neck, and upper chest, concurrent with contralateral anhidrosis. Here, we present and discuss a clinical case of sarcoidosis-associated ganglionopathy and Harlequin syndrome. Case presentation: A 31-year-old woman complained of burning pain in the right side of the upper chest and the feet. She also experienced episodes of intense flushing and sweating on the right side of her face, neck, and upper chest. Three years before these symptoms began, the patient was diagnosed with pulmonary sarcoidosis. On neurological examination, sensory disturbances were present. In the trunk, the patient reported pronounced hyperalgesia and allodynia in the upper part of the right chest and some patches on the right side of the upper back. In the extremities, hypoalgesia in the tips of the fingers and hyperalgesia in the feet were noted. An extensive diagnostic workup was performed to eliminate other possible causes of these disorders. A broad range of possible metabolic, immunological, and structural causes were ruled out. Thus, the final clinical diagnosis of sarcoidosis-induced sensory ganglionopathy, small-fiber neuropathy, and Harlequin syndrome was made. Initially, the patient was treated with pregabalin and amitriptyline, but the effect was inadequate for the ganglionopathy-induced pain. Therefore, therapeutic plasma exchange as an immune-modulating treatment was selected, leading to partial pain relief. Conclusions: This case report demonstrates the possible autoimmune origin of both sensory ganglionopathy and Harlequin syndrome. It suggests that an autoimmune etiology for these disorders should be considered and the diagnostic workup should include screening for the most common autoimmune conditions.

Anatomical evidence of non-parasympathetic cardiac nitrergic nerve fibres in rat.

Neuronal nitric oxide synthase (nNOS)-derived nitric oxide (NO) plays a major role in the neural control of circulation and in many cardiovascular diseases. However, the exact mechanism of how NO regulates these processes is still not fully understood. This study was designed to determine the possible sources of nitrergic nerve fibres supplying the heart attempting to imply their role in the cardiac neural control. Sections of medulla oblongata, vagal nerve, its rootlets and nodose ganglia, vagal cardiac branches, Th1 -Th5 spinal cord segments, dorsal root ganglia of C8 -Th5 spinal nerves, and stellate ganglia from 28 Wistar rats were examined applying double immunohistochemical staining for nNOS combined with choline acetyltransferase (ChAT), peripherin, substance P, calcitonin gene-related peptide, tyrosine hydroxylase or myelin basic protein. Our findings show that the most abundant population of purely nNOS-immunoreactive (IR) neuronal somata (NS) was observed in the nodose ganglia (37.4 ± 1.3%). A high number of nitrergic NFs spread along the vagal nerve and entered its cardiac branches. All nitrergic neuronal somata (NS) in the nucleus ambiguus were simultaneously immunoreactive (IR) to ChAT and composed only a small subset of neurons (6%). In the dorsal nucleus of vagal nerve, biphenotypic nNOS-IR/ChAT-IR neurons composed 7.0 ± 1.0%, while small purely nNOS-IR neurons were scarce. Nitrergic NS were plentifully distributed within the nuclei of solitary tract. In the examined dorsal root and stellate ganglia, a few nitrergic NS were sporadically present. The majority of sympathetic NS in the intermediolateral nucleus were simultaneously immunoreactive for nNOS and ChAT. In conclusion, an abundant population of nitrergic NS in the nodose ganglion implies that neuronal NO is involved in afferent cardiac innervation. Nevertheless, nNOS-IR neurons identified within vagal nuclei may play a role in the transmission of preganglionic parasympathetic nerve impulses.

Incidence and clinical significance of postoperative delirium after brain tumor surgery.

BACKGROUND: Delirium is an acute and reversible deterioration of mental state. Postoperative delirium (POD) can develop after surgical procedures and is associated with impaired health status and worse recovery. So far, there is little data about postoperative delirium after brain surgery. The aim of this study was to evaluate frequency, risk factors, and prognostic value of POD in predicting short-term postoperative outcomes after brain tumor surgery. METHODS: Five-hundred and twenty-two patients who underwent elective brain tumor surgery in 2010-2017 were included in this prospective study. Patients were monitored for POD using the Confusion Assessment Method for the ICU (CAM-ICU) for 2 to 7 days after the surgery. At hospital discharge, outcomes were evaluated using the Glasgow Outcome Scale (GOS). RESULTS: POD was diagnosed in 22 (4.2%) patients. Risk factors of POD were low level of hemoglobin, poor functional status at time of admission, low education level and older age (65 years and older). POD incidence was not associated with brain tumor laterality, location, extent of resection, histological diagnosis, or affected brain lobe. POD was associated with greater risk for unfavorable outcomes at hospital discharge (OR = 5.3; 95% CI [2.1-13.4], p = 0.001). CONCLUSIONS: POD is not a common complication after elective brain tumor surgery. Older age, poor functional status, low education level and anemia are associated with greater POD risk. Extent of surgical intervention and brain tumor location are not associated with POD risk. POD is associated with worse outcome at hospital discharge.