Adie-Holmes syndrome associated with COVID-19 infection: A case report.

With increasing experience, it has been suggested that the SARS-CoV-2 virus has a neurotropic effect. Here, we present a case of a tonic pupil who developed after COVID-19 infection. A 36-year-old woman presented with progressive photophobia and blurred vision. On neurological examination, loss of deep tendon reflexes accompanying a tonic pupil was detected and brain MR imaging was normal. With this case, we aimed to describe a rare pattern of neurological involvement caused by the possible SARS-CoV-2 virus.

Ophthalmic and Neuro-ophthalmic Manifestations of Coronavirus Disease 2019 (COVID-19).

PURPOSE: To describe a case of inflammatory chorioretinopathy and Adie's syndrome possibly associated with COVID-19. METHODS: Observational case report. RESULTS: A 51-year-old woman developed fever, cough, and headache followed by retro-ocular pain and reading impairment. She tested positive for SARS-COV-2 infection by qualitative real-time reverse-transcriptase-polymerase-chain-reaction. The slit-lamp and funduscopic exam revealed abnormal pupillary response and yellowish creamy deep chorioretinal lesions, which were not present in previous examinations. Instillation of pilocarpine demonstrated denervation supersensitivity, and it was suggestive of bilateral Adie tonic pupil. A comprehensive work-up ruled out other systemic, autoimmune, or infectious diseases. CONCLUSIONS: This case illustrates the possible association between multifocal chorioretinitis and Adie's syndrome, and the SARS-COV-2 infection in humans. Further investigation of virus infectivity specifically within ocular tissues has to be conducted.

More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma.

Demirsoy U, Alparslan B, Sen MC, Anik Y, Akansel G, Görür G, Gürel B, Aksu G, Çorapçioglu F. More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma. Turk J Pediatr 2019; 61: 139-141. Paraneoplastic syndromes in Hodgkin lymphoma (HL) can be seen with different symptoms and organ findings within a significant time before definite diagnosis of the primary disease. Achalasia, Holmes-Adie pupil, and limbic encephalitis are rarely reported paraneoplastic components in pediatric HL. In this report, we present an 11-year-old girl who had all these three paraneoplastic components synchronously before HL was identified.

Two Uncommon Paraneoplastic Neurological Syndromes in a Child With Hodgkin Lymphoma.

Paraneoplastic neurological syndromes (PNS) are rare, remote effects of cancer that are usually caused by an altered immune response to the tumor and not due to the tumor mass, metastasis, infection, ischemia, or metabolic derangements. PNSs can affect any area of the central, the peripheral, and the autonomic nervous systems. These are rare in lymphomas compared with solid tumors attributed to their presentation even in late stages and the absence of onconeural antibodies. We present a child with stage IIB Hodgkin lymphoma who presented with dual PNS, achalasia cardia, and Holmes Adie pupil occurring synchronously with the cancer.

Unilateral Adie's Tonic Pupil and Viral Hepatitis - Report of Two Cases.

INTRODUCTION: Adie's (tonic) pupil is a neuro-ophthalmological disorder characterized by a tonically dilated pupil, which is unresponsive to light. It is caused by damage to postganglionic fibers of the parasympathetic innervation of the eye, usually by a viral.or bacterial infection. Adie's syndrome includes diminished deep tendon reflexes. OUTLINE OF CASES: We report data of a 59-year-old female with unequal pupil sizes. She complained of blurred vision and headache mainly while reading. She had a 35-year history of hepatitis B and liver cirrhosis. On exam, left pupil was mydriatic and there was no response to light and at slit lamp we saw segments of the sphincter constrict. We performed 0.125% pilocarpine test and there was a remarkable reduction of size in the left pupil. The second case is a 55-year-old female who was referred to the University Eye Clinic because of a headache and mydriatic left pupil. She had diabetes mellitus type 2, as well as hepatitis A virus 20 years earlier. On exam, the left pupil was mydriatic, with no response to light. Test with diluted pilocarpine was positive. Neurological examinations revealed no abnormality in either case so we excluded Adie's syndrome. CONCLUSION: Adie's tonic pupil is benign neuro-ophthalmological disorder of unknown etiology. Most patients commonly present no symptoms and anisocoria is noticed accidentally. Although the etiology is unknown, there are some conditions that cause tonic pupil. It may be a part of a syndrome in which tonic pupil is associated with absent deep tendon reflexes.

Reversal of pupillary atonia after removal of an encircling band.

: We describe a patient who developed an atonic pupil after placement of an encircling band during retinal detachment surgery. When the band was removed 18 months later, the pupil signs showed partial recovery demonstrating a degree of reversibility of the parasympathetic paresis. We speculate that in this case mechanical deformation of the sclera by the encircling band had produced a conduction block of the short posterior ciliary nerve fibers as they pass forward in the underlying suprachoroidal space.

The post illumination pupil response is reduced in seasonal affective disorder.

Individuals with seasonal affective disorder (SAD) may have a decreased retinal sensitivity in the non-image forming light-input pathway. We examined the post illumination pupil response (PIPR) among individuals with SAD and healthy controls to identify possible differences in the melanopsin signaling pathway. We also investigated whether melanopsin gene (OPN4) variations would predict variability in the PIPR. Fifteen SAD and 15 control participants (80% women, mean age 36.7 years, S.D.=14.5) were assessed in the fall/winter. Participants were diagnosed based on DSM-IV-TR criteria. Infrared pupillometry was used to measure pupil diameter prior to, during, and after red and blue stimuli. In response to blue light, the SAD group had a reduced PIPR and a lower PIPR percent change relative to controls. The PIPR after the blue stimulus also varied on the basis of OPN4 I394T genotype, but not OPN4 P10L genotype. These findings may indicate that individuals with SAD have a less sensitive light input pathway as measured by the PIPR, leading to differences in neurobiological and behavioral responses such as alertness, circadian photoentrainment, and melatonin release. In addition, this sensitivity may vary based on sequence variations in OPN4, although a larger sample and replication is needed.

Bilateral Holmes-Adie syndrome as an early manifestation of the HIV neuropathy.

A 38-year-old HIV-1 infected woman affected with bilateral tonic pupils. Ophthalmologic examination confirmed Holmes-Adie syndrome (HAS), and peripheral distal polyneuropathy, orthostatic hypotension and leg hyperhidrosis were detected on further workup. The HAS can be either idiopathic or associated with neuropathy of various etiology (autoimmune, paraneoplastic and infectious). In our patient, the pupillotonia was the first and early symptom of hitherto unrecognized HIV neuropathy. HAS has been previously observed in association with syphilis, Lyme borreliosis, herpes simplex and parvovirus B19 infection. Our case is the first report of HAS in a case of HIV infection.

Adie's tonic pupil secondary to migraine.

A 46-year-old woman observed transient unilateral mydriasis during a classic migraine attack. One week later she experienced a similar episode after which anisocoria was persistent. Subsequent examination showed the clinical and pharmacologic features of a postganglionic parasympathetic paresis (Adie's tonic pupil). This case confirms the hypothesis that transient mydriasis accompanying migraine is due to interruption of parasympathetic innervation rather than sympathetic overactivity.

Pure sensory neuropathy in patients with primary Sjögren's syndrome: clinical, immunological, and electromyographic findings.

A pure sensory neuropathy caused by lymphocytic infiltration of the dorsal root ganglia has been reported in a few patients with Sjögren's syndrome. The clinical, immunological, and electromyographic findings of five patients with this type of neuropathy and primary Sjögren's syndrome were reviewed. Typical clinical indications were the presence of a chronic asymmetrical sensory deficit, initial disease in the hands with a predominant loss of the vibratory and joint position senses, and an association with Adie's pupil syndrome or trigeminal sensory neuropathy. The simultaneous impairment of the central and peripheral evoked cortical potentials suggested that there was a lesion of the neuronal cell body. The neuropathy preceded the diagnosis of Sjögren's syndrome in four patients. Four patients were positive for Ro antibodies, but systemic vasculitis or malignancy was not found after a mean follow up of six years. These findings indicate that in patients with a sensory neuropathy the diagnosis of Sjögren's syndrome has to be considered, even if the patient denies the presence of sicca symptoms, and that appropriate tests must be carried out.

Adie's pupil following Le Fort I maxillary osteotomy. A complication or coincidence?

A case is reported of right-sided tonic pupil in isolation (Adie's pupil) following Le Fort I maxillary osteotomy. It is difficult to identify whether this rare finding immediately postoperatively is either a pure coincidence or a genuine complication of surgery.

[Myasthenia gravis associated with tonic pupil].

A 41 year-old female patient with acquired autoimmune myasthenia gravis (MG, IIa type) and tonic pupil (Adie's pupil) of the right side was reported. Adie's pupil was pointed out at the same time of the clinical diagnosis of MG in September, 1988. She had the solitary thymoma identified by pneumo-mediastinograph and mediastinal CT scan. Results of the positive anti-nicotinic AChR antibody titer, the positive edrophonium test and the electrophysiological examination gave unambiguous evidences of acquired autoimmune MG with thymoma. Instillations to the eyes of cocaine, pilocarpine and epinephrine, and the pupillary response to the intravenous injection of edrophonium revealed the postganglionic abnormality mainly in the parasympathetic system. There were no other overt symptoms due to dysautonomia. She refused thymectomy. Her myasthenic and pupillary signs became gradually improving without any immunosuppressive medications. It is concluded that two diseases of the patient may be probably caused by multiple organ- or tissue-specific autoantibodies, in addition to pathogenetic significance of Adie's pupil.