POEMS syndrome disclosed by ophthalmological manifestation

POEMS syndrome is characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes. Papilledema is a common finding but ophthalmological complaints are rare. We report a case of POEMS syndrome in a 42-year old man which was revealed by oscillopsia and blurred vision. An osteosclerotic myeloma of the fifth lef rib was found, but there was no improvement after removal of the rib. The patient died two months later from pulmonary embolism.     

The impact of blurred vision on cognitive assessment

The purpose of this study was to systematically assess the effect of blurred vision on several nonverbal neuropsychological measures commonly used as part of test batteries to assess the cognitive status of different patient populations. A total of 30 highly educated and healthy participants aged between 21 and 33 years were placed in one of three blurred vision groups, defined by their maximal visual acuity (20/20 or control group, 20/40, and 20/60). Blurred vision was simulated using positive diopters at a distance of 40 cm, the same distance as that at which tests were administered. Each participant was then assessed on a predetermined battery of nonverbal and verbal neuropsychological tests demanding different levels of acuity for optimal performance (i.e., tests whose items varied in terms of size and spatial frequency characteristics). In general, blurred vision significantly affected performance on nonverbal tests defined by small-sized/high-spatial-frequency items to a greater extent than on tests defined by larger sized/lower spatial-frequency items. As expected, blurred vision did not affect verbal test performance (Similarities, Information, and Arithmetic WAIS subtests).Our results are a clear indication of how even a "minimal" loss of visual acuity (20/40) can have a significant effect on the performance for certain nonverbal tests. In conclusion, such inferior performance is hypothetically interpretable as reflecting impaired cognitive functioning (i.e., attentional) targeted by a specific task (i.e., visual search) and suggests that the precision of the cognitive assessment and subsequent diagnosis are significantly biased when visuo-sensory abilities are not optimal, particularly for older patient populations where blurred vision resulting from correctable visual impairment is quite common.     

Water drinking as a potential treatment for idiopathic exercise-related syncope

We report a 20-year-old sportsman with frequent attacks of lightheadedness, chest pain, blurred vision and falls during and shortly after exercise. Cardiac and pulmonal evaluation and routine autonomic function tests were normal apart from a relatively high resting heart rate (70 bpm) compared to endurance-trained men. In view of the relation to exercise, the patient was asked to cycle with maximal effort on an ergometer with continuous blood pressure (BP), heart rate (HR) and electroencephalogram (EEG) registration. Immediately after cessation of exercise a brief hypotensive period (75/45 mmHg) occurred together with sinus tachycardia (180 bpm) during which the patient experienced his typical complaints. We hypothesized that our patient's symptoms were primary related to sympathetic failure. As water drinking has been demonstrated to raise sympathetic activity rapidly, we undertook a second cycling test after ingestion of 1000 mL tap water. Symptoms nor hypotension recurred. Because of the short lasting pressor effect and its minimal side effects, we suggest water drinking as simple and possible effective therapy for idiopathic exercise-related syncope. Received: 3 September 2002, Accepted: 16 January 2003 Correspondence to: R. D. Thijs, MD     

Intracerebral hematoma following lumboperitoneal shunt insertion: a rare case report

Lumboperitoneal shunting is widely used for the surgical management of pseudotumor cerebri and other pathologies such as communicating hydrocephalus. Although it is a safe method, it could be associated with complications including subarachnoid hemorrhage, subdural and rarely intracerebral hematoma. A 44-year-old female applied to our clinic with complaints of severe headache, retroorbital pain and blurred vision. Lumbar puncture demonstrated cerebrospinal fluid opening pressure of cmH2O. A non-programmable lumboperitoneal shunt with two distal slit valves was inserted due to pseudotumor cerebri. She deteriorated shortly after surgery. Immediate cranial computed tomography scan revealed a right parietal intracerebral hematoma. Development of intracerebral hematoma following lumboperitoneal shunt is a rare complication. We discuss this rare event accompanied by the literature.     

Recurrent sinus pericranii in a 14-year-old boy

A case of symptomatic and recurrent sinus pericranii in a 14-year-old boy is presented. A blood-filled cyst was diagnosed after head trauma. The sinus pericranii was surgically removed. One year later the patient presented with blurred vision, headaches, and recurrence at a different site in the skull.     

Cerebral Aspergillosis with Multiple Enhancing Nodules in the Right Cerebral Hemisphere in the Immune-Competent Patient

Aspergillosis in the central nervous system (CNS) is a very rare disease in immune-competent patients. There was a case of a healthy man without a history of immune-compromised disease who had invasive aspergillosis with unusual radiologic findings. A 48-year-old healthy man with diabetes mellitus, presented with complaints of blurred vision that persisted for one month. Brain magnetic resonance imaging (MRI) showed multiple nodular enhancing lesions on the right cerebral hemisphere. The diffusion image appeared in a high-signal intensity in these areas. Cerebrospinal fluid examination did not show any infection signs. An open biopsy was done and intraoperative findings showed grayish inflammatory and necrotic tissue without a definitive mass lesion. The pathologic result was a brain abscess caused by fungal infection, morphologically aspergillus. Antifungal agents (Amphotericin B, Ambisome and Voriconazole) were used for treatment for 3 months. The visual symptoms improved. There was no recurrence or abscess pocket, but the remaining focal enhanced lesions were visible in the right temporal and occipital area at a one year follow-up MRI. This immune-competent patient showed multiple enhancing CNS aspergillosis in the cerebral hemisphere, which had a good outcome with antifungal agents.     

Optic neuritis caused by varicella infection in an immunocompetent child

Chickenpox may lead to several neurologic complications, but optic neuritis has rarely been described. We report on a 6-year-old immunocompetent patient who presented with unilateral optic neuritis and severe visual loss because of varicella infection. A week after varicella eruption, the child experienced blurred vision. An examination revealed decreased visual acuity of his right eye, a right pupil poorly reactive to light, and almost no color vision in his right eye. A history of chickenpox, the fundus examination, and the measurement of visual-evoked potentials allowed us to make a diagnosis of optic neuritis caused by varicella infection. The patient received only symptomatic relief with antipyretics. Three months later, his visual acuity improved to 20/40 in the right eye.     

Bilateral visual loss complicating liposuction in a patient with idiopathic intracranial hypertension.

A 34-year-old obese woman developed blurred vision in both eyes soon after large-volume liposuction of the dorsum and gluteus region bilaterally associated with abdominal dermolipectomy. An ophthalmic examination revealed severe bilateral visual loss and pallid optic disc edema. The patient gave a history of transient obscurations of vision in the past. Neuroimaging studies were non-revealing, but a lumbar puncture disclosed a markedly elevated intracranial pressure. The patient was diagnosed as having had bilateral ischemic optic neuropathy superimposed on pre-existing idiopathic intracranial hypertension (IIH). Acetazolamide treatment was used. Some visual improvement occurred, and optic disc edema evolved into severe optic disc pallor. This case shows that visual loss from optic disc infarction may be a devastating complication of high-volume liposuction in patients with underlying IIH. Because liposuction is frequently performed on obese patients, physicians should screen for signs and symptoms of IIH before undertaking this procedure.     

The terminology of transient visual loss due to vascular insufficiency

Transient visual loss due to cerebro-ocular vascular disease is a common symptom. The purpose of this paper is to present a unified terminology of the monocular vs. binocular or homonymous types. Lack of proper identification may lead to mis-diagnosis and improper management of these entities. Monocular blurred vision must be investigated since its origin is so commonly due to atherosclerosis of the carotid system. Binocular blurred vision due to vertebro-basilar insufficiency is managed conservatively in almost all instances.     

Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum)

OBJECTIVE: Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a rare hamartomatous lesion of the cerebellar cortex. The pathogenesis of the disease is still poorly understood. Lhermitte-Duclos disease was recently considered to be part of a multiple hamartoma-neoplasia syndrome (Cowden disease). We add two further cases to this rare entity. PATIENTS: A 24-year old woman presented with occipital headaches, blurred vision, diplopia and ataxia of gait. Physical examination revealed turricephaly. The second patient was a 37-year old woman, who presented with progressive occipital headache with nausea and vomiting. Physical examination revealed congenital facial asymmetry. Computed tomography and NMR-imaging, respectively demonstrated a space occupying mass of a cerebellar hemisphere in both cases. RESULTS: Suboccipital craniotomy and complete removal of the infratentorial tumour were performed in both patients. Histopathological findings clinched the diagnosis of Lhermitte-Duclos disease. Postoperative course was uneventful in the first and complicated by progressive occlusive hydrocephalus in the second patient, necessitating permanent surgical shunt drainage. Both patients were discharged free of complaints. CONCLUSIONS: Dysplastic cerebellar gangliocytoma is commonly associated with progressive mass effects in the posterior fossa and typically presents with headaches, cerebellar dysfunction, occlusive hydrocephalus and cranial nerve palsies. The disease usually manifests in young adults, but the age at presentation ranges from birth to the sixth decade. There is no sex predilection. NMR-imaging became a useful clue to the diagnosis within the last decade. Therapy consists of decompression of the posterior fossa by total surgical removal of the tumour mass.     

Hyperactive vestibulo-ocular reflex in cerebellar degeneration: pathogenesis and treatment

We studied a patient with a cerebellar degeneration and hyperactive vestibulo-ocular reflex (VOR). He complained of oscillopsia and blurred vision with head movement. A twofold increase in VOR gain (peak eye velocity/peak head velocity) at high frequencies was associated with a VOR time constant of 6 seconds (low normal). Visual cancellation ("suppression") of the VOR and smooth pursuit were also abnormal. We hypothesized that his high VOR gain was due to dysfunction of olivocerebellar projections. Physostigmine reduced his VOR gain, consistent with the hypothesis that these projections are cholinergic.     

Occipital hypoperfusion in a patient with psychogenic visual disturbance

A patient with bilateral diminished visual acuity and blurred vision was given the diagnosis of psychogenic visual disturbance after extensive psychiatric and ophthalmological examinations. Single photon emission computed tomography with Tc-99m-ethylcysteinate dimer demonstrated remarkably reduced regional cerebral blood flow (rCBF) in the bilateral occipital lobes. More specifically, rCBF was reduced in the visual association areas, but not in the primary visual areas. These findings suggest that functional suppression of the visual association area is associated with the development of psychogenic visual disturbance.     

A case of a pituitary mass in association with sarcoidosis]

We report a case of a pituitary mass associated with sarcoidosis. Natural regression of the mass was demonstrated after the replacement of corticosteroid hormone. A seventy-one-year old man presented with blurred vision and general fatigability, and admitted to our hospital. He was diagnosed to have ophthalmic and pulmonary sarcoidosis. Two months later, he consulted our hospital again, with complaints of fatigability and vomiting. Laboratory data showed hyponatremia, and CT scan revealed a suprasellar mass. He had no neurological deficits. Diabetes insipidus was not observed. Magnetic resonance image showed a heterogeneously enhanced intrasellar and suprasellar dumbbell shaped mass and thickening of the pituitary stalk. Hormonal examination revealed corticosteroid insufficiency and hypothyroidism, possibly caused by the pituitary mass. Replacement of corticosteroid hormone significantly improved his fatigability. Two months later, follow-up MRI showed regression of the pituitary mass. Based on the clinical and radiological findings, pituitary mass was strongly suggested to be caused by sarcoidosis. We recommend prompt steroid treatment for such a pituitary mass and strict follow-up MRI study.     

Anticholinergic-induced chorea in the treatment of focal dystonia

The occurrence of chorea, induced by trihexyphenidyl (benzhexol hydrochloride) during the treatment of five adult patients who had focal or segmental dystonia, is described. The dose at which chorea appeared ranged from 15 to 60 mg/day (mean 31.7 mg/day). All but one patient had developed common adverse effects of this drug (dry mouth, blurred vision, and confusion) at lower doses (mean 21.8 mg per day). There was an inverse relationship between the age of the patient and the dose of trihexyphenidyl at which chorea developed.     

A Ruptured Dermoid Cyst of the Cavernous Sinus Extending into the Posterior Fossa

Supratentorial dermoid cysts are uncommon to develop in the cavernous sinus. We present a ruptured dermoid cyst of the cavernous sinus extending into the posterior fossa. The patient was a 32-year-old female who complained occipital headache, blurred vision, and tinnitus over 4 years. Brain magnetic resonance (MR) imaging revealed an enhanced tumor in the right cavernous sinus extending into the right temporal base and the posterior fossa with findings of ruptured cyst. Surgical resection was performed, and pathological findings were confirmed to be a dermoid cyst. We report a second case with ruptured dermoid cyst of the cavernous sinus extending into the posterior fossa.     

Treatment of empty sella syndrome with ventriculoperitoneal shunt.

A symptomatic empty sella developed in a female patient undergoing bromocriptine therapy for microprolactinoma. Placement of a ventriculoperitoneal shunt dramatically improved the symptoms of headache and blurred vision. The post-operative imaging showed resolution of the empty sella. She was able to resume bromocriptine therapy without recurrence of her previous symptoms and give birth to a baby 20 months later. An MRI 44 months after surgery and on bromocriptine therapy showed no recurrence of the empty sella. We conclude that ventriculoperitoneal shunt may be a simple, and durable treatment for drug induced empty sella and allows resumption of bromocriptine therapy for preexisting microprolactinoma.     

A brainstem variant of reversible posterior leukoencephalopathy syndrome (RPLS) presenting with visual disturbance]

A 40-year-old woman noticed blurred vision of the right eye. The optic disc edema of bilateral eyegrounds was noted, and brain MRI showed abnormal signals of the brainstem at a neurosurgical clinic. On her first visit, blood pressure was as remarkably high as 250/130 mmHg. Neurologically, only optic disc edema of bilateral eye-grounds was found. Both T2-weighted MRI and FLAIR showed hyperintense signal areas mainly from the ventral pons to medulla oblongata. These areas were not enhanced with gadolinium. About three weeks after the administration of an antihypertensive agent, brain MRI revealed no abnormal signal. About three months later, the blurred vision disappeared and ophthalmological abnormalities subsided. We diagnosed her with a brainstem variant of RPLS, presenting with visual disturbance caused by hypertensive retinopathy.     

Persistent visual problems thirty years after severe head trauma

INTRODUCTION: Visual disturbances are common after severe head trauma. Some authors report 50 percent of these patients having damage to the visual system, ocular motor nerve palsies, or central eye movement disturbances. Visual disturbances influence prognosis and outcome of neurological rehabilitation, and, in most cases, diagnosis and treatment require interdisciplinary care. CASE REPORT: We report here a 50-year-old patient who suffered from severe head trauma at age 20. After coming out of a 4-week coma, he suffered visual disturbances such as blurred vision and diplopia. Visual acuity was 1.0 and the visual fields were intact. The symptoms remained unexplained for a long time. One year after the trauma, right trochlear palsy was diagnosed and surgical treatment was performed. However, the symptoms persisted, and one year later, a second operation was performed without changing the symptoms. During many years, prisms or refractive corrections were used to improve the visual disturbances, and finally, a psychiatric treatment was started. At age 40, the patient became presbyopic and his visual problems increased, especially for reading. When he was examined for the first time in our consultation, corrected visual acuity was 1.0 with a small hyperphoria of the right eye. During careful examination of the eye downgaze, a conjugate pure torsional nystagmus was observed, which disappeared in primary gaze position. CONCLUSION: Pure torsional nystagmus is difficult to diagnose, especially when it is provoked only by one gaze direction such as in our patient. As a sequel of the severe head trauma with brainstem contusion, this type of nystagmus is a rare form of central vestibular nystagmus, and may by modified by head rotation or suppressed by convergence. The "treatment" of the patient's blurred vision when reading was easy by ordering goggles with a near part placed near the primary gaze position and a 6 base down prismatic correction. Reading was then possible without provoking torsional nystagmus.     

Giant pituitary adenoma invading the clivus

A 26-year-old man was admitted with complaints of worsening of vision for one month. Investigations revealed a large sellar and parasellar tumor mass invading into both the cavernous sinuses. The tumor invaded the clivus and both petrous apices. It was radically but partially resected through a transsphenoidal approach. Following surgery, the patient showed symptomatic improvement. The residual tumor was treated by radiation treatment. The case is discussed and the literature on the subject is briefly reviewed.     

Schwannoma of the spinal accessory nerve

The authors report a case of a schwannoma originating from the spinal root of the accessory nerve. The patient was a 40-year-old woman with chronic headache and blurred vision. A neurological examination failed to reveal any abnormal findings except for papilloedema. A computed tomographic (CT) scan showed hydrocephalus and a magnetic resonance image (MRI) on admission showed an enhancing extramedullary tumour located at the level of foramen magnum. The tumour was successfully removed and was verified to originate from the spinal root of the right accessory nerve. The hydrocephalus improved soon after surgery. The patient left hospital in a satisfactory condition. There are 15 cases of accessory nerve schwannomas reported in the literature; the authors report the 16th. Although accessory nerve schwannoma is very rare, one should consider it as one of the differential diagnoses for tumours at the foramen magnum.