Influence of cataract surgery for the first or second eye on vision-related quality of life (VR-QOL) and the predictive factors of VR-QOL improvement.

PURPOSE: To assess improvements in vision-related quality of life (VR-QOL) in patients undergoing their first or second eye cataract surgery, as well as clinical factors related to VR-QOL. STUDY DESIGN: Prospective case series. METHODS: We examined 282 patients undergoing their first (222) or second (60) eye cataract surgery. VR-QOL was evaluated before and after surgery using the 25-item National Eye Institute visual function questionnaire (VFQ-25), along with the best-corrected visual acuity (BCVA), uncorrected visual acuity, and the lens opacities classification system III (LOCSIII). The resulting VFQ-25 subscale scores were compared between patients undergoing their first or second eye cataract surgery, including multiple regression analysis. RESULTS: The mean VFQ-25 composite score (CS) was 71.5 ± 14.2 before and 84.0 ± 10.2 after the first eye cataract surgery and 73.5 ± 12.7 before and 85.4 ± 10.2 after the second eye cataract surgery. VFQ-25 scores improved significantly, with reduced disparity among patients after surgery in both groups. Preoperative CS was related to the preoperative sum of the BCVA (standardized partial regression coefficient (ß) = - 0.254, P < 0.001). Improvement in the CS was related to a preoperative poor BCVA (ß = 0.203, P < 0.001), low CS (ß = - 0.693, P < 0.001), and high general health score (ß = 0.118, P = 0.025). CONCLUSIONS: VR-QOL improved after the first and second eye surgery. Many VFQ-25 subscales were related to the BCVA or LOCSIII scores. Low preoperative VR-QOL and BCVA were related to an improved postoperative VR-QOL.

Anti-aquaporin-4 antibody-positive definite neuromyelitis optica in a patient with thymectomy for myasthenia gravis.

BACKGROUND: Since serum anti-aquaporin-4 (AQP4) antibody/neuromyelitis optica (NMO)-IgG has been detected as a specific biomarker for NMO), new diagnostic criteria have been proposed. Recently, a rare coexistence of NMO and myasthenia gravis (MG) has been documented. Here, we provide further evidence of the association of these 2 immunologic disorders and review the literature. CASE REPORT: A 60-year-old woman, who had a history of optic neuritis at age 43, developed longitudinally extended transverse myelitis (LETM). She had repeated optic neuritis and longitudinally extended transverse myelitis attacks, which have been treated by corticosteroid pulse therapy. She was diagnosed as having definite NMO, because serum anti-aquaporin-4 antibody was positive. She had also been suffering from generalized MG since age 30 and underwent thymectomy at age 40. There have been 14 patients with NMO associated with MG, most of whom developed NMO years after thymectomy. Our patient is the seventh patient with seropositive NMO who underwent thymectomy for MG. CONCLUSIONS: The coexistence of MG with thymectomy and seropositive NMO suggests an etiopathogenic link between these 2 disorders but remains underrecognized by neurologists. Neurologists should consider this possible association when encountering atypical presentations of either MG or NMO.

Reversible cerebral vasoconstriction syndrome presenting as subarachnoid hemorrhage, reversible posterior leukoencephalopathy, and cerebral infarction.

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by acute severe headache with or without additional neurological symptoms and reversible cerebral vasoconstriction. Unruptured aneurysm has been reported in some cases with RCVS. We report a severe case of a 53-year-old woman with RCVS having an unruptured cerebral aneurysm and presenting as cortical subarachnoid hemorrhage, reversible posterior leukoencephalopathy syndrome, and cerebral infarction. She was successfully treated with corticosteroids and a calcium channel blocker and the aneurysm was clipped. Her various complications are due to the responsible vasoconstriction that started distally and progressed towards proximal arteries. This case demonstrates the spectrum of presentations of RCVS, a clinically complicated condition.

[Cryptococcal meningitis successfully treated with liposomal amphotericin B and voriconazole in an elderly patient].

Abstract An 81-year-old woman who had microscopic polyangiitis that was being treated with corticosteroids for 2 months was admitted to our department because of fever and clouding of consciousness. Neurological examination showed disturbance of consciousness and nuchal stiffness. Analysis of cerebrospinal fluid (CSF) revealed pleocytosis, low glucose level, and elevated protein levels. On the basis of the presence of Cryptococcus neoformans in CSF, the patient was diagnosed with cryptococcal meningitis. On the basis of established practice guidelines, liposomal amphotericin B (L-AMB) was administered to avoid the possible nephrotoxicity of amphotericin B. After the treatment was started, the patient's condition gradually improved. The results of CSF analysis also showed a gradual recovery. Because the cryptococcal antigen in CSF did not disappear completely, voriconazole (VRCZ) was administered orally; subsequently, the CSF cryptococcal antigen titer gradually decreased. During the course of the treatment with L-AMB and VRCZ, there were no severe side effects that required a change in treatment. To the best of our knowledge, in Japan, the combination of L-AMB and VRCZ has rarely been reported to be effective for the treatment of cryptococcal meningitis. The recovery of our patient indicates that the administration of L-AMB and VRCZ to elderly patients with cryptococcal meningitis and renal insufficiency is safe and leads to a successful outcome.

Isolated proximal leg paresis due to a small cortical infarction.

We describe two patients with small cortical infarcts, who presented with isolated proximal weakness in one of their legs. These lesions were located in the contralateral precentral gyrus, more medial than the precentral knob, but more lateral than the topmost part of the motor cortex. These clinical findings are consistent with the physiological findings of Penfield and Boldrey, and those of recent activation studies by functional MRI. It is clinically important to pay close attention to the contralateral top of the motor cortex when examining pure motor monoparesis of a proximal part of a lower extremity.

Subacute longitudinal myelitis associated with Behcet's disease.

In neuro-Behcet's disease (NBD), myelitis is a rare clinical form. We report the case of a woman with NBD presenting with unsteady gait and generalized spasticity. Spinal magnetic resonance imaging (MRI) showed multiple and confluent hyperintensities extending throughout the entire spinal cord on a T2-weighted image with some contrast enhancement. High-dose steroid therapy was so effective that the myelitis was markedly improved, as shown in the follow-up MRI. Longitudinal myelitis involving the entire spinal cord, as seen in our patient, has never been described before. This case suggests that NBD could be one of the important differential diagnoses of longitudinal myelitis.

Blurred vision with acute hypertension indicating hypertensive brainstem encephalopathy-case report-.

A 49-year-old woman presented with hypertensive brainstem encephalopathy (HBE) manifesting as visual disturbance and papilledema but no other neurological abnormal findings. Magnetic resonance (MR) imaging showed extensive lesions in the brainstem and bilateral thalami but not in the occipital lobes. The patient also had renal failure and underwent hemodialysis. Her visual disturbance and MR lesions resolved rapidly after antihypertensive treatment. This case of HBE only caused visual disturbance despite the presence of massive brainstem edema. The presence of fetal-type posterior cerebral artery may have spared the occipital lobe. Clinicians should consider HBE in hypertensive patients with blurred vision. HBE is reversible if immediate antihypertensive treatment is initiated, but neurological sequelae may develop if treatment is delayed.

Successful treatment of relapsing neuro-Sweet's disease with corticosteroid and dapsone combination therapy.

Sweet's disease with central nervous system involvement, tentatively named neuro-Sweet's disease, has rarely been reported. Although systemic corticosteroid therapy is highly effective for neurologic symptoms in neuro-Sweet's disease, relapse is common. Here, we describe the case of a 38-year-old Japanese man who presented with relapsing neuro-Sweet's disease that was successfully treated with a combination of corticosteroid and dapsone. Dapsone should be considered as a therapeutic option for neuro-Sweet's disease patients showing relapse.

Segmental zoster paresis of limbs: report of three cases and review of literature.

OBJECTIVES: Segmental zoster paresis is a relatively rare complication characterized by focal motor weakness, which may occur in limbs affected by herpes zoster. We demonstrate the clinical characteristics of segmental zoster paresis by reviewing the cases of 138 patients, including 3 of our patients. CASE REPORT AND REVIEW SUMMARY: We report 3 patients with zoster paresis of the limbs. Patients 1 and 3 showed motor weakness in the left shoulder and arm after developing a herpetic rash in the left C5-C6 dermatomes. Patient 2 showed weakness in the right thigh and groin after a right L2-L3 herpetic eruption. The electromyograms of all 3 patients showed abnormal spontaneous activity in the affected muscles. Intravenous acyclovir and corticosteroid pulse therapy were added to oral antiviral drugs for patients 1 and 2. All 3 patients recovered favorably. Our review of the literature revealed that antiviral treatment may prevent the occurrence of zoster paresis; however, there is insufficient evidence to show what treatment hastens recovery from zoster paresis. CONCLUSIONS: Segmental zoster paresis is still underrecognized by neurologists. Awareness of this disorder is important because it may eliminate unnecessary invasive investigations and lead to appropriate treatment. Further studies on the treatment are necessary.

Isolated reversible splenial lesion in adult meningitis: a case report and review of the literature.

We report the rare case of a 38-year-old man who suffered from aseptic meningitis. Brain magnetic resonance imaging showed an ovoid lesion with a high signal intensity on T2- and diffusion-weighted images in the splenium of the corpus callosum that completely disappeared within a week. There were no symptoms or signs associated with the lesion, and the prognosis was good. Although rare in adult patients, awareness of a reversible splenial lesion in meningoencephalitis is important.

[A case of neuralgic amyotrophy in the lower extremity (lumbosacral radiculoplexus neuropathy)].

We report a 76-year-old man who suddenly had pain in the right buttock that radiated down the back of the leg. He underwent nerve block therapy, but subsequently noted weakness of the right leg. On examination, there was moderate weakness of the right gastrocnemius, flexor digitorum longus and extensor hallcis longus, and mild weakness of the tibialis anterior, quadriceps and hamstrings muscles. The right Achilles tendon reflex was absent. There was a slight diminution of touch sensation over the right L5-S1 area. A nerve conduction study revealed reduced motor and sensory action potentials in the right leg. An electromyography showed evidence of denervation in the right gastrocnemius and tibialis anterior muscles. Treatment with corticosteroids was effective for alleviating his pain, and muscle strength improved gradually. One year later, he showed no neurological deficits. Neuralgic amyotrophy in the lower extremities is rare, but awareness of this disorder helps us avoid unnecessary surgical investigations and leads to possible treatment with immunomodulative therapy.

Pseudoathetosis caused by migrated cervical disc.

Although pseudoathetosis has been described in various conditions, it has been rarely reported in patients with epidural lesions. We report the first case of a patient with pseudoathetosis caused by a migrated cervical disc. The patient was a 76-year-old woman who presented with progressive clumsiness of the hands and athetotic movements. Electrophysiological studies revealed normal peripheral nerve conduction and delayed and attenuated cortical SEPs. Spinal MRI revealed an epidural mass with gadolinium enhancement at the C3-4 level. An anterior cervical surgery disclosed a migrated disc, and its removal resulted in a marked improvement. Migrated cervical disc should be considered in patients with a profound sensory loss and pseudoathetosis in the hands, because this condition is treatable.

Cortical myoclonus due to hypocalcemia 12 years after thyroidectomy.

Although seizures have been described in hypocalcemia, myoclonus has been rarely reported. We report the first case of a patient with hypocalcemic cortical myoclonus due to hypoparathyroidism following a previous thyroidectomy. The patient was an 84-year-old woman who presented with multifocal myoclonus, which was predominant in the upper extremities, neck, jaw, and facial muscles. Electrophysiological studies revealed enlarged somatosensory evoked potentials, cortical reflexes evoked by peripheral nerve stimulation, and a cerebral potential preceding myoclonic jerks determined by jerk-locked averaging. All these findings were consistent with cortical myoclonus. The myoclonic state disappeared as serum calcium level became normal. Hypocalcemia should be considered in patients who had had a thyroidectomy, even if it was performed more than 10 years previously.

An extremely unusual presentation of varicella zoster viral infection of cranial nerves mimicking Garcin syndrome.

We report a patient with the varicella zoster viral (VZV) infection of multiple cranial nerves mimicking Garcin syndrome, who initially presented with Ramsay Hunt syndrome (herpes zoster oticus). A 78-year-old man showed left facial palsy with zosteric eruptions in his left auricle and dysphagia, followed by left total ophthalmoplegia. His serum anti-VZV antibody titer was elevated. Cerebrospinal fluid examination revealed pleocytosis with a slightly elevated protein level. He was treated with intravenous acyclovir and corticosteroids. His tongue weakness resolved, and then ocular movement improved. The improvement of facial palsy and swallowing difficulty was delayed. VZV infection should be considered even in patients who show unilateral multiple cranial neuropathy mimicking Garcin syndrome because it is treatable.

[Familial amyotrophic lateral sclerosis with rapid progression].

We report a family with autosomal dominant (AD) motor neuron disease. A 41-year-old man developed muscle weakness and fasciculation of the lower extremities. The weakness progressed to the upper extremities and bulbar muscles. The cerebrospinal fluid (CSF) protein level was slightly elevated. A nerve conduction study revealed reduced compound muscle action potentials, but conduction block was not observed. Electromyogram showed acute and chronic neurogenic changes. He was treated with intravenous immunoglobulin (IVIg) and methylprednisolone pulse therapy, but his condition rapidly deteriorated. He developed respiratory failure necessitating artificial ventilation within three months after the onset of the disease. His father developed muscle weakness and atrophy of the upper extremities at age 70, and his cousin developed muscle weakness of the legs at age 41. Their conditions rapidly progressed to quadriplegia. CSF and electrophysiological findings were similar to those of the proband. Treatments by steroid pulse therapy, IVIg, and plasmapheresis were not effective. The father and cousin also required artificial ventilation within 3-4 months from the onset of symptoms, and became locked-in state. Autosomal dominant amyotrophic lateral sclerosis (AD-ALS) was considered, but SOD1 gene mutation was not detected. The present pedigree may have familial ALS caused by a gene mutation other than SOD1.

Myoclonic tremulous movements in multiple system atrophy are a form of cortical myoclonus.

We prospectively studied the clinical and electrophysiological features of myoclonic tremulous movements in patients with multiple system atrophy (MSA). Among 42 consecutive patients, 12 MSA-p (parkinsonian type) and 3 MSA-c (cerebellar type) patients showed small-amplitude myoclonic movements. These movements occurred in the distal part of the arms and fingers, particularly in posture or during voluntary movements. We conducted detailed electrophysiological studies in 11 patients. Electromyographic recordings showed irregular bursts of brief duration often synchronously involving the antagonistic muscles. Somatosensory evoked responses were slightly enlarged in some patients. Long-latency reflexes were enhanced in 7 patients. A jerk-locked averaging technique revealed premyoclonic cortical potentials in 9 patients. These findings suggest that small-amplitude myoclonic movements in MSA are a form of cortical myoclonus.

Blepharospasm associated with multiple system atrophy: a case report and review of the literature.

Although blepharospasm has been occasionally associated with parkinsonism, it has rarely been reported in patients with multiple system atrophy (MSA). We report a 65-year-old woman with MSA who developed blepharospasm seven years after onset, rendering her functionally blind. Clinical course and the findings of magnetic resonance imaging indicated cerebellar type MSA. The blink reflex studies showed prolonged R2 response and enhanced recovery cycle, indicating an increased excitability of the brainstem interneurons. These results suggest that pathophysiology of blepharospasm in MSA is similar to that of essential blepharospasm. Recognition of blepharospasm in MSA patients is important, as blepharospasm is a treatable feature in this otherwise intractable disorder.