-
The Turkish Journal of Pediatrics 2019Demirsoy U, Alparslan B, Şen MC, Anık Y, Akansel G, Görür G, Gürel B, Aksu G, Çorapçıoğlu F. More than Ophelia syndrome: Multiple paraneoplastic syndromes in...
Demirsoy U, Alparslan B, Şen MC, Anık Y, Akansel G, Görür G, Gürel B, Aksu G, Çorapçıoğlu 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.
Topics: Adie Syndrome; Child; Esophageal Achalasia; Female; Hodgkin Disease; Humans; Limbic Encephalitis; Paraneoplastic Syndromes
PubMed: 31559737
DOI: 10.24953/turkjped.2019.01.024 -
Journal of Family Medicine and Primary... Apr 2019Ross syndrome is diagnosed by the presence of the characteristic triad of segmental anhidrosis, depressed deep tendon reflex, and tonic pupils. It is a rare,...
Ross syndrome is diagnosed by the presence of the characteristic triad of segmental anhidrosis, depressed deep tendon reflex, and tonic pupils. It is a rare, misdiagnosed autonomic disorder with less than 80 cases reported in the world literature. Two representative cases of Ross syndrome are presented with their laboratory correlates and relevant review of literature. Both cases (aged 35 and 58) presented with complaint of decreased sweating over one half of the face and ipsilateral upper limb and trunk and contralateral lower limb. There was compensatory increased sweating and hyperpigmentation over the remaining parts of the body. The duration of symptoms was 2 years and 15 days. The patients had variegated skin color as per the above distribution and hyporeflexia in lower limbs. One patient also had Holmes-Adie pupil. Iodine test showed hypohidrosis in the described areas, which was confirmed by skin biopsy in both cases. The patients were treated symptomatically with incomplete relief. The authors aim to highlight this rare disorder that can be one of the causes of pathological sweating encountered in general practice and the challenges in its management.
PubMed: 31143750
DOI: 10.4103/jfmpc.jfmpc_151_19 -
Neurological Sciences : Official... Jan 2018Sir Gordon Morgan Holmes (1876-1965) was one of the most important founders of modern neurology and a great teacher and scientist. He was the first scientist to...
Sir Gordon Morgan Holmes (1876-1965) was one of the most important founders of modern neurology and a great teacher and scientist. He was the first scientist to challenge the theory of the unitary function of the cerebellum and described cerebellar disorders. Holmes together with Thomas Grainger Stewart (1877-1957) described 40 cases of the rebound phenomenon in cerebellar disease (Stewart-Holmes maneuver or Stewart-Holmes test). He also described the symptoms of inherited neurodegenerative spinocerebellar ataxia involving the olivary nucleus (Gordon-Holmes syndrome). Independently from the Australian neurologist William John Adie (1886-1935), he described the partial iridoplegia (Holmes-Adie pupil or Holmes-Adie syndrome). His teaching skills became clearly visible in Goulstonian and Croonian lectures dedicated to spinal cord injuries.
Topics: History, 19th Century; History, 20th Century; Neurology
PubMed: 29116546
DOI: 10.1007/s10072-017-3180-6 -
Molecular and Clinical Oncology Jun 2017Adie's syndrome (AS) and paraneoplastic sensorimotor neuropathy with cerebellar ataxia (PSN CA) are extremely rare, rapidly progressive, autoimmune diseases associated...
Synchronous Adie's syndrome and type 1 antineuronal nuclear antibody (anti-Hu)-related paraneoplastic neurological syndromes as predictors of complete response in limited-stage small-cell lung cancer: A case report.
Adie's syndrome (AS) and paraneoplastic sensorimotor neuropathy with cerebellar ataxia (PSN CA) are extremely rare, rapidly progressive, autoimmune diseases associated with the development of antibodies against neuronal-specific Hu proteins that are abnormally expressed in small-cell lung cancer (SCLC). We herein present the unique case of a 55-year-old obese woman, previous heavy smoker, who, during treatment with standard cisplatin-etoposide chemotherapy for limited-stage SCLC, developed simultaneous AS and worsening symptoms consistent with PSN CA that led to significant neurological disability and severe axonal electrophysiological pattern on nerve conduction studies. Serology confirmed the presence of low-titre type 1 antineuronal nuclear antibodies (ANNA-1), previously referred to as anti-Hu antibodies. Following plasmapheresis, immunosuppressive therapy and physical rehabilitation, the neurological symptoms progressively improved. The tumour completely regressed, with no recurrence detected on subsequent radiological examinations. The aim of this case was to highlight the importance of a multidisciplinary team approach for early recognition and rapid treatment of paraneoplastic neurological syndromes (PNS) as key to achieving significant recovery and marked improvement of the neurological deficit. This report extends the literature by confirming earlier studies showing that the presence of serum ANNA-1 in SCLC, an aggressive type of pulmonary carcinoma that is challenging to treat, may portend a more favourable prognosis and response to chemotherapy. Thus, patients with SCLC and new-onset neurological symptoms should be tested for ANNA-1. The role of a multimodality approach to treating PNS is also emphasized.
PubMed: 28588791
DOI: 10.3892/mco.2017.1248 -
Case Reports in Neurological Medicine 2017A 40-year-old woman was found to have bilateral Adie's pupils and generalized muscle stretch areflexia. She did not have orthostatic hypotension but, in an ECG strip in...
A 40-year-old woman was found to have bilateral Adie's pupils and generalized muscle stretch areflexia. She did not have orthostatic hypotension but, in an ECG strip in the office, she appeared to have an almost fixed heart rate. We thus studied the heart rate variability (HRV) and the systolic blood pressure variability (SBPV) in supine and standing position and also during rhythmic breathing. We found a decreased HRV in the time domain with very low standard deviation in supine and standing position and during rhythmic breathing. HRV in the frequency domain was low with a decrease in the absolute power of HF and LF and a decrease in the sympathovagal balance in supine and standing positions. SBPV in the time and frequency domains was found to be normal. This patient with Holmes-Adie syndrome had an asymptomatic severe loss of HRV and a preserved SBPV. The global decrease in the HRV in the time and frequency domains indicated that she had both vagal and sympathetic cardiac denervation, whereas the preserved SBPV suggested normal innervation of the blood vessels.
PubMed: 28428900
DOI: 10.1155/2017/4919758 -
Indian Journal of Dermatology 2016Ross syndrome is a rare dysautonomia characterized by a clinical complex of segmental anhidrosis or hypohidrosis, areflexia, and tonic pupils. A very few cases (≃50)...
Ross syndrome is a rare dysautonomia characterized by a clinical complex of segmental anhidrosis or hypohidrosis, areflexia, and tonic pupils. A very few cases (≃50) have been reported in literature since its original description in 1958. Here, we report the case of a middle-aged homemaker from Odisha, India, who presented with complaints of segmental hypohidrosis for the past 7 years.
PubMed: 27293279
DOI: 10.4103/0019-5154.182472 -
Anais Brasileiros de Dermatologia 2016Ross syndrome is a rare disease characterized by peripheral nervous system dysautonomia with selective degeneration of cholinergic fibers. It is composed by the triad of...
Ross syndrome is a rare disease characterized by peripheral nervous system dysautonomia with selective degeneration of cholinergic fibers. It is composed by the triad of unilateral or bilateral segmental anhidrosis, deep hyporeflexia and Holmes-Adie's tonic pupil. The presence of compensatory sweating is frequent, usually the symptom that most afflicts patients. The aspects of the syndrome are put to discussion due to the case of a male patient, caucasian, 47 years old, with clinical onset of 25 years.
Topics: Cholinergic Fibers; Humans; Hyperhidrosis; Hypohidrosis; Male; Middle Aged; Nerve Degeneration; Peripheral Nervous System Diseases; Primary Dysautonomias; Syndrome
PubMed: 26982793
DOI: 10.1590/abd1806-4841.20163918 -
Srpski Arhiv Za Celokupno Lekarstvo 2015Adie'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...
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.
Topics: Accommodation, Ocular; Adie Syndrome; Diabetes Complications; Female; Hepatitis B; Humans; Iris; Middle Aged; Pilocarpine; Pupil; Reflex, Pupillary; Tonic Pupil
PubMed: 26506756
DOI: 10.2298/sarh1508451k -
Neuro-ophthalmology (Aeolus Press) Oct 2015Adie's pupil is characterised by pupil dilatation, segmental iris palsy, light-near dissociation, and slow re-dilatation. Most commonly, tonic pupils are unilateral and...
Adie's pupil is characterised by pupil dilatation, segmental iris palsy, light-near dissociation, and slow re-dilatation. Most commonly, tonic pupils are unilateral and idiopathic, but can be caused by orbital disorders and autonomic neuropathies. There are only a few case reports of tonic pupils in patients with Sjögren's syndrome, caused by an autoimmune ciliary ganglionitis. The authors report on two cases with bilateral tonic pupils as the initial manifestation of primary Sjögren's syndrome. Both patients presented with blurred vision, bilateral tonic pupils, and sicca symptoms. The findings suggest that Sjögren's syndrome should be considered in patients presenting with bilateral Adie's tonic pupils.
PubMed: 27928364
DOI: 10.3109/01658107.2015.1067231 -
The Pan African Medical Journal 2015
Topics: Adie Syndrome; Adolescent; Diagnosis, Differential; Female; Humans
PubMed: 26213604
DOI: 10.11604/pamj.2015.20.330.4437