ABSTRACT
Paroxysmal tonic spasms [PTS] are common in patients with neuromyelitis optica spectrum disorder (NMOSD).1 2 In patients with demyelinating disease, PTS can significantly reduce the quality of life, limit activities of daily living and the rehabilitative process following an acute relapse 3. As in patients with multiple sclerosis (MS), paroxysmal tonic spasms in NMOSD usually respond well to treatment with carbamazepine.2 However, the optimal treatment in patients where carbamazepine is contraindicated or poorly tolerated is unclear. We describe a patient with NMOSD with severe paroxysmal tonic spasms who did not tolerate carbamazepine but was successfully treated with lacosamide (Vimpat).
Subject(s)
Acetamides/therapeutic use , Anticonvulsants/therapeutic use , Neuromyelitis Optica/complications , Spasm/drug therapy , Adult , Carbamazepine/adverse effects , Female , Humans , Lacosamide , Spasm/etiology , Treatment OutcomeABSTRACT
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterised by psychiatric symptoms, movement disorder and seizures often evolving into a severe encephalopathy. An overlap has recently been recognised between anti-NMDAR encephalitis and inflammatory demyelinating disorders, particularly neuromyelitis optical spectrum disorder (NMOSD). In this case report, we describe two patients with an initial presentation consistent with anti-NMDAR encephalitis who have subsequently developed relapsing-remitting multiple sclerosis (MS) and discuss the literature pertaining to potential overlap between NMDAR encephalitis and inflammatory demyelinating disorders.
Subject(s)
Anti-N-Methyl-D-Aspartate Receptor Encephalitis/complications , Multiple Sclerosis, Relapsing-Remitting/etiology , Adult , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/diagnostic imaging , Anti-N-Methyl-D-Aspartate Receptor Encephalitis/physiopathology , Brain/diagnostic imaging , Female , Humans , Male , Multiple Sclerosis, Relapsing-Remitting/diagnostic imaging , Multiple Sclerosis, Relapsing-Remitting/physiopathology , Spinal Cord/diagnostic imagingABSTRACT
BACKGROUND: Spinal cord pathology is an important substrate for long-term disability in multiple sclerosis (MS). OBJECTIVE: To investigate longitudinal changes in spinal cord lesions and atrophy in patients with a non-spinal clinically isolated syndrome (CIS), and how they relate to the development of disability. METHODS: In all, 131 patients with a non-spinal CIS had brain and spinal cord imaging at the time of CIS and approximately 5 years later (median: 5.2 years, range: 3.0-7.9 years). Brain magnetic resonance imaging (MRI) measures consisted of T2-hyperintense and T1-hypointense lesion loads plus brain atrophy. Spinal cord MRI measures consisted of lesion number and the upper cervical cord cross-sectional area (UCCA). Disability was measured using the Expanded Disability Status Scale (EDSS). Multiple linear regression was used to identify independent predictors of disability after 5 years. RESULTS: During follow-up, 93 (71%) patients were diagnosed with MS. Baseline spinal cord lesion number, change in cord lesion number and change in UCCA were independently associated with EDSS ( R2 = 0.53) at follow-up. Including brain T2 lesion load and brain atrophy only modestly increased the predictive power of the model ( R2 = 0.64). CONCLUSION: Asymptomatic spinal cord lesions and spinal cord atrophy contribute to the development of MS-related disability over the first 5 years after a non-spinal CIS.
Subject(s)
Multiple Sclerosis/pathology , Spinal Cord Diseases/pathology , Adolescent , Adult , Atrophy/diagnosis , Brain/pathology , Demyelinating Diseases/pathology , Disability Evaluation , Female , Humans , Magnetic Resonance Imaging/methods , Male , Middle Aged , Young AdultABSTRACT
Acute multifocal placoid pigment epitheliopathy (AMPPE) is an autoimmune chorioretinal disease that can be complicated by neurological involvement. There is limited information on this potentially treatable condition in the neurological literature. The objective of this patient series is to describe the neurological complications of AMPPE. We retrospectively identified patients with neurological complications of AMPPE seen at Auckland Hospital between 2008 and 2013 and summarised cases in the literature between 1976 and 2013. We identified five patients with neurological complications of AMPPE at Auckland Hospital and 47 reported patients. These patients demonstrated a spectrum of neurological involvement including isolated headache, stroke or transient ischaemic attack, seizures, venous sinus thrombosis, optic neuritis, sensorineural hearing loss and peripheral vestibular disorder. We propose criteria to define AMPPE with neurological complications. A cerebrospinal fluid (CSF) lymphocytosis in a patient with isolated headache may predict the development of cerebrovascular complications of AMPPE. Patients with cerebrovascular complications of AMPPE have a poor prognosis with high rates of death and neurological disability among survivors. Predictors of poor outcome in those who develop neurological complications of AMPPE are a relapsing course, generalised seizures and multifocal infarction on MRI. All patients with neurological complications of AMPPE, including headache alone, should be investigated with an MRI brain and CSF examination. Patients with focal neurological symptoms should receive intravenous (IV) methylprednisolone followed by a tapering course of oral steroids for at least 3months. Patients with AMPPE and an isolated headache with a CSF pleocytosis should be treated with oral steroids.
Subject(s)
Autoimmune Diseases/complications , Choroiditis/complications , Nervous System Diseases/etiology , Retinal Diseases/complications , Adult , Female , Humans , Magnetic Resonance Imaging/adverse effects , Male , Multifocal ChoroiditisABSTRACT
Embolic stroke is the most common neurological complication of infective endocarditis and a major source of morbidity and mortality. Septic embolism is considered a contraindication to intravenous thrombolysis in patients with ischaemic stroke because of concerns over an increased risk of intracranial haemorrhage. We describe a patient with occult endocarditis who was treated with thrombolysis for acute stroke and review other cases reported in the literature.
Subject(s)
Endocarditis , Intracranial Embolism/etiology , Streptococcal Infections , Streptococcus sanguis/isolation & purification , Stroke , Thrombolytic Therapy , Administration, Intravenous , Adult , Anti-Bacterial Agents/administration & dosage , Contraindications , Echocardiography , Endocarditis/complications , Endocarditis/diagnosis , Endocarditis/drug therapy , Endocarditis/physiopathology , Female , Fibrinolytic Agents/administration & dosage , Fibrinolytic Agents/adverse effects , Gentamicins/administration & dosage , Humans , Magnetic Resonance Imaging , Penicillins/administration & dosage , Streptococcal Infections/complications , Streptococcal Infections/diagnosis , Streptococcal Infections/drug therapy , Streptococcal Infections/physiopathology , Stroke/diagnosis , Stroke/etiology , Stroke/therapy , Thrombolytic Therapy/adverse effects , Tissue Plasminogen Activator/administration & dosage , Tissue Plasminogen Activator/adverse effects , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
This report describes a man who died after a gunshot wound that entered the right atrium and exited from the right ventricle without entering the cardiac septa or the left side of the heart. At necropsy, the left atrial appendage was found to be inverted and invaginated into the mitral orifice. The invagination of the left atrial appendage is viewed as anatomic evidence that a negative left ventricular pressure was created as the left ventricular volume rapidly decreased as a result of right-sided cardiac exsanguination. Previously reported experiments in animals demonstrating the sucking (negative pressure) action of the left ventricle during ventricular diastole are summarized. The prerequisite for creation of a negative pressure in the ventricles during diastole is an extreme diminution in left ventricular volume, in this case as a result of right-sided cardiac bleeding. Only a vacuum effect of the left ventricle during diastole can explain the inversion and invagination of the left atrial appendage in this patient.