Case Reports in Neurology最新文献

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disease of the central nervous system that predominantly affects the brainstem. Apart from corticosteroids, there are few reported treatment options for CLIPPERS, and there is no standard therapy. A 77-year-old man presented with diplopia that had persisted for 5 months. Dysarthria and numbness of the distal right upper extremity and right lips were also observed. Brain magnetic resonance imaging (MRI) revealed a hyperintense area around the brainstem. Symptoms were relieved immediately following intravenous methylprednisolone (IVMP) administration. However, after gradual tapering of oral prednisolone to 5 mg/day, the symptoms relapsed, and brain imaging revealed that the condition had worsened. Intravenous immunoglobulins (IVIg) were administered for recurrence, with no clinical improvement. After each IVMP treatment, the patient recovered promptly. Based on the patient's symptoms and characteristic MRI findings, exclusion of other diseases, and the significant efficacy of corticosteroids, he was diagnosed with CLIPPERS. There was no recurrence at a maintenance prednisolone dose of 8 mg/day. IVIg had a poor effect on the acute phase of CLIPPERS symptoms. Compared with other immunosuppressants, IVIg is less effective in suppressing the relapse of CLIPPERS.

Anti-metabotropic glutamate receptor 1 (mGluR1) encephalitis is a rare autoimmune disorder manifesting with cerebellar syndrome. Patients with mGluR1 encephalitis have been treated with immunomodulatory therapies; however, little is known about the efficacy of this therapy. A 58-year-old Japanese woman presented with dizziness when walking and standing up. Symptoms persisted and the patient gradually deteriorated. The neurological examination revealed a broad-based gait, horizontal and slightly gaze-evoked nystagmus, noticeable head titubation, and truncal ataxia without limb ataxia. Magnetic resonance imaging was normal. The ¹²³I-isopropyl-iodoamphetamine single-photon emission-computed tomography scans showed normal cerebellar perfusion. Based on a positive antibody test for anti-mGluR1, the patient was diagnosed with anti-mGluR1 encephalitis. She was treated with intravenous methylprednisolone and intravenous immunoglobulin (IVIg). Symptoms gradually improved over 1 month and almost disappeared after additional IVIg therapy. Anti-mGluR1 encephalitis is a rare disease, and effective treatment is unclear. In this case, a favorable outcome was obtained with immunomodulatory therapy, even though the neurological disability of the disease course is worse. We emphasize the importance of early diagnosis and therapeutic intervention, suspecting the disease on the basis of its characteristic symptoms.

Hallucinations are common in neurodegenerative dementias, being present in a significant proportion of patients. Most of the available studies show that acetylcholinesterase inhibitors may be beneficial in preventing and treating hallucinations in patients with neurodegenerative and even psychiatric disorders, even though there are reports that they might also develop as an adverse effect of such therapy. However, a clear causal relationship for the latter association was not previously established. Here we describe 2 cases of patients treated with donepezil who developed complex multimodal hallucinations, which could be causally linked to the drug by means of a challenge-dechallenge (and rechallenge in one case) paradigm. We also provide a narrative review of the literature regarding donepezil and hallucinations and propose a hypothesis to explain the occurrence of this phenomenon.

Cerebral proliferative angiopathy (CPA) is a rare vascular abnormality characterized by transdural supply, stenoses of feeding arteries, and intermingled normal brain parenchyma in abnormal vessels. CPA is often regarded as a separate entity from "classical" brain arteriovenous malformations in angioarchitecture, natural history, clinical presentation, and treatment. Bleeding from CPA is uncommon, but once bleeding occurs, the risk of rebleeding is high. Herein, we describe a case of cerebral hemorrhage caused by CPA. We performed two different endovascular treatments: partial embolization with glue for a ruptured aneurysm and coil embolization for an unruptured growing aneurysm. To our knowledge, this is the first report of serial endovascular treatments for hemorrhagic CPA that included a ruptured aneurysm and a growing unruptured aneurysm.

We report 2 cases of reversible cerebral vasoconstriction syndrome (RCVS) associated with postpartum. In case 1, a 26-year-old woman developed sudden-onset headache, nausea, and vomiting 1 h after an uncomplicated vaginal delivery. In case 2, a 27-year-old woman developed generalized seizures 9 days after an uncomplicated vaginal delivery. In both cases, initial angiographic studies showed no significant vasoconstriction; however, repeat studies revealed reversible vasoconstriction. Serial magnetic resonance imaging (MRI) revealed transient brain lesions during 6 months. RCVS remains poorly characterized, misdiagnosed, and under-recognized. Serial MRI and magnetic resonance angiographic findings may contribute to diagnosis of RCVS.

Differentiating GCA from its many mimickers remains a challenge in the daily clinical practice, especially in patients presenting with unspecific manifestations. We present the case of an 82-year-old woman who presented with a 3-week history of left eye vision loss secondary to bilateral edema and hemorrhage of the optic discs. Despite negative bilateral temporal artery biopsies, the elevation of the inflammatory markers and brain MRA findings suggestive of temporal arteritis as well as stenosis of the basilar artery led us to initiate treatment with high-dose steroids. Inflammatory markers remained elevated despite high-dose steroids which prompted additional work leading to a diagnosis of varicella-zoster encephalitis. Steroid treatment was quickly tapered off and treatment with acyclovir resulted in the normalization of the acute phase reactants. The persistence of elevated inflammatory markers despite high-dose steroids should prompt additional work up for the search of an alternative diagnosis of GCA mimickers.

Vestibular schwannoma (VS) is commonly accompanied by hearing loss, tinnitus, and dizziness and tends to be chronically progressive in nature. We report a case of VS presenting as left vestibular neuritis (VN) in a previously healthy 57-year-old patient. Right-beating horizontal-torsional spontaneous nystagmus was observed, and the bedside head impulse test revealed a left catch-up saccade. The bithermal caloric test showed left canal paresis, and pure-tone audiometry revealed an average threshold of 22.5 dB bilaterally. Brain magnetic resonance imaging (MRI) demonstrated a 0.7-cm enhancing mass in the left internal auditory canal, consistent with VS. The patient was administered with high-dose systemic corticosteroids and vestibular suppressants with antiemetic, which relieved acute vertigo. Although dizziness in VS is chronically progressive in nature, VS may present as an acute vestibular syndrome that mimics VN. VS should be considered a potential cause of acute vestibular syndrome, and thorough neurological examination with MRI may be helpful for accurate diagnosis.

Mirror movements (MMs) are involuntary synchronous movements of one limb during voluntary movements of the contralateral limb. Generally, MMs after stroke are observed in the unaffected hand during voluntary movements of the affected hand; MMs in the affected hand are comparatively rare. In previous studies, evaluation of MMs in the affected hand was performed using simple unilateral movement tasks, such as tapping or forceful repeated hand closure. However, the impact of MMs of the affected hand on functional tasks, such as activities of daily living (ADLs), has not been reported. We report the rare case of a patient with MMs of the affected hand due to atherothrombotic cerebral infarction of the right postcentral and precentral gyri. An 85-year-old Japanese man presented with left-sided hemiplegia and sensory impairment. MMs were observed in the left (affected) hand during many ADLs and could not be suppressed by the patient's will even when the examiner verbally instructed the patient to move only the unaffected hand. The patient was aware that his hand moved on its own, but he could not control it. The patient was trained on various types of bilateral coordinated motor exercises for 114 days after the MMs were first identified. However, this did not affect MM occurrence, and the MMs remained at the time of discharge. Future research is necessary to plan long-term interventions for MMs of the affected hand.

Chronic daily headaches are often refractory to prescribed or non-prescribed medications. Transcranial direct current stimulation (tDCS) is a new technological-based intervention with various modes of applicability. Therefore, we aimed to study the effectiveness of tDCS in an individual with symptoms of chronic daily headaches and the associated comorbidities like depression, anxiety, stress, and RLS or sleepiness, numbness, a sensation of fullness, or ringing in the ears. Based on DASS-21 and Epworth questionnaires, headache diary, and semi-structured interviews, we used repeated measures for assessing the symptoms such as frequency, duration, intensity, or severity of chronic daily headaches and associated comorbidities at baseline, after tDCS-intervention, and at a 12-month follow-up. The results showed that tDCS-intervention reduced the frequency, duration, intensity, or severity of chronic daily headaches and associated symptoms after tDCS-intervention and at a 12-month follow-up.