Introduction of Horner's Syndrome with an Unusual Presentation of Hodgkin's Disease: A Case Report

Horner's syndrome is a rare disease with three clinical signs of ptosis, miosis and unilateral anhydrosis of the face and neck. In this syndrome, Hodgkin's lymphoma should be considered as one of the first life-threatening symptoms. Horner's syndrome, or oculosympathetic paralysis, is a rare disease first reported by Mr. Horner (1883-1886) with three clinical signs including unilateral ptosis, miosis, and Unilateral and unilateral anhidrosis of the face and neck were described, all due to involvement of the oculosympathetic pathway (1). Horner syndrome results from an interruption of the oculosympathetic pathway. This condition does not usually cause vision problems or other significant symptoms, but is important as a warning sign that the oculosympathetic pathway has been interrupted, potentially with serious and even life-threatening processes. The oculosympathetic pathway has a long and circuitous course, beginning in the brain and traveling down the spinal cord to exit in the chest, then up the neck and into the orbit. The sympathetic nervous supply is responsible for the dilation of the pupil (mydriasis). When disrupted, parasympathetic supply is uninhibited, and constriction of the pupil (miosis) ensues. The reaction of the pupils to light and accommodation is normal as those systems do not depend on sympathetic nerve supply. Therefore, this syndrome with unimpressive clinical findings and insignificant symptoms may be a sign of serious pathology in the head, chest, or neck. Patients with Horner syndrome present with a slightly droopy upper lid and a smaller pupil on the affected side; less commonly, there is a deficiency of sweating over the brow or face on the affected side (2). Horner syndrome is almost always diagnosed clinically, though pharmacological testing can be used to confirm the diagnosis. Imaging modalities such as PET, CT and MRI are important components of work‐up for patients presenting with acquired Horner syndrome (3). Factors that cause this disease include trauma, brain tumors, migraines, cluster headaches, hyperthyroidism, and lung cancer(4). Because Horner syndrome sometimes occurs as the first sign of a life-threatening condition, a thorough post-diagnosis systemic examination is necessary to follow up on patients for signs and symptoms (5). This is a clonal malignancy (dependent on asexual reproduction) of the lymphatic system with different clinical manifestations that can be treated in the early and advanced stages (6). Horner's syndrome is an unusual initial manifestation of Hodgkin's disease, and, in this case, it was due to oculosympathetic damage from mediastinal compression (7, 8). The aim of this study was to report and introduce a patient referred to Imam Khomeini Hospital in Kermanshah with a diagnosis of Horner's syndrome and Hodgkin's lymphoma.

The patient is a 30-year-old man with left ptosis about 50 days before seeing an ophthalmologist. The patient also complains of sweating on the right side of the body and lack of sweating on the left side of the upper torso and face. No previous history of specific illness or family history of the symptoms were reported. In ocular examinations, the corrected vision of both eyes is equal to 10/10, normal eye movements, mild ptosis in the range of 2 mm in the left eye, meiosis of the left eye and the development of Anisocoria (an ocular complication in which the pupil of one eye from Size differs from other pupils.) Was reported more clearly in low light. According to the mentioned symptoms, Horner syndrome was presented to the patient. A complete and appropriate examination is sufficient for the initial diagnosis of this syndrome, but in order to confirm the diagnosis and determine the location of the injury, naphazoline drops were used. In this way, this drop was poured in both eyes of the patient and after a few minutes, the ptosis disappeared and the amount of anisocoria decreased. At present, after starting treatment, in addition to improving anisocoria, the amount of ptosis has decreased and the patient is satisfied with continuing treatment. On systemic examination, several large lymph nodes on the left side of the neck were touched in the area of the sternocleidomastoid muscle (SCM). Ultrasound of this area also showed a lymphadenopathy measuring 15 x 25 mm deep in the SCM muscle in the subclavian space (Subclavian triangle) and a lymph node measuring 10 x 15 mm in the upper region of the supraclavicular space with involvement of the upper mediastinum. MRI (Magnetic resonance imaging) of the brain and spinal cord was also reported to be normal. An X-ray of the chest showed umbilical cord lymphadenopathy. Finally, after biopsy of Hodgkin's lymph node, the diagnosis of cervical lymph node was made.

In the present report, after examining the patient, Horner syndrome was clinically confirmed and after Hodgkin's lymph node biopsy, the diagnosis of Hodgkin's lymphoma was made. Involvement was also seen in the SCM muscle area and the upper mediastinum. In line with the results of the present study in a study by Simon et al. Conducted in 1985 on a 22-year-old woman with Horner syndrome, a mass in the mediastinum was observed on a patient's chest X-ray and evaluated. Further leads to the diagnosis of Hodgkin. This study suggests that Horner's syndrome is an unusual early manifestation of Hodgkin's disease caused by damage to the oculosympathetic pathway due to pressure on the mediastinum(9). A 1980 study by Maloney et al. On 450 patients with Horner's syndrome reported less than 3 percent of Hodgkin's lymphoma(7). Another study by Giles et al. In 1958 on 216 patients with Horner syndrome identified three cases of Hodgkin's lymphoma(6). Also, a 1940 study by Goldman et al., Conducted on 212 patients with Hodgkin's disease, reported no cases of Horner's syndrome(10). Kaplan also stated in his 1980 study that no cases of the syndrome were observed in patients with Hodgkin's disease(11). However, in the present report, MRI of the patient's brain and spinal cord was normal and no complication was observed. However, Williams et al., In their 1959 study of the complications of leukemia and lymphoma, found that neurological complications occur in 15% of patients with Hodgkin's lymphoma(12). Jackson et al., Who studied Hodgkin's disease in 1945, also noted complications such as brain and spinal cord injuries, polyneuropathy, mononorrhea, and myopathy(13).According to the results of this study, with the confirmation of Hodgkin's lymphoma in the patient, it is necessary that in addition to the entire oculosympathetic pathway, the patient be examined for neoplasms, including Hodgkin's lymphoma, due to its high potential. Also, due to the involvement of the upper mediastinum, it can be stated that Horner's syndrome is an unusual initial manifestation of Hodgkin's disease, which is caused by damage to the oculosympathetic pathway due to pressure on the mediastinum. In general, according to the results of the present study and similar studies among the early life-threatening symptoms, although Hodgkin's disease has been reported as uncommon, but due to the high potential for its treatment, it should be Patients with this syndrome should be considered. Also, with the publication of the present study, we can point to the sensitivity among ophthalmologists in identifying certain cases of malignancies associated with Horner's syndrome, which can lead to their early detection and better prognosis. Unfortunately, the lack of similar Iranian and foreign studies and the unavailability of the full text of some other articles for comparison prevented a comprehensive decision on the results.