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Approach to the meningioma patient

Introduction Meningiomas are slow-growing extraaxial tumors that account for about 25% of all intracranial tumors. The World Health Organization (WHO) classifies meningiomas as grade I, II, or III based upon morphologic criteria with higher grades correlating with more aggressive behavior such as local invasion, recurrence, and shortened survival. The main options for the management of meningiomas include surgery, radiation, and observation. The management of these patients…

Evaluation of peripheral nerve lesions

Disclosures Dr. Ly has no disclosures. Dr. Jordan has research funding from the Department of Defense, the National Institutes of Health, and the Burke Foundation. He receives royalties from Elsevier and has received honoraria from the American Academy of Neurology. He performs paid consultation for CereXis Pharmaceuticals, Navio Theragnostics, Health2047 Inc., and the Neurofibromatosis Network. Introduction Peripheral nerve lesions encompass a broad differential diagnosis and may…

Imaging of spinal lesions

Introduction Spinal cord tumors are uncommon causes of back pain, radicular pain, and sensorimotor deficits in adults and pediatric patients. Primary spinal tumors are less common than intracranial tumors and represent approximately 2–4% of primary central nervous system (CNS) tumors. Classification of spinal cord tumors Spinal cord tumors are classified as either (1) intradural intramedullary, (2) intradural extramedullary, or (3) extradural depending on the relationship to…

Evaluation of an infratentorial lesion

Introduction The posterior fossa is the infratentorial compartment of the cranial vault, which houses the cerebellum and the bulk of the brainstem. Tumors that occur in this region can arise from, or spread to, any of the adjacent structures. The brainstem, which is composed of the midbrain, pons, and medulla, may develop or seed tumors that are intrinsic and contained within the brainstem, intrinsic with exophytic…

Evaluation of a supratentorial parenchymal lesion

Introduction Central nervous system (CNS) tumors encompass histologically diverse entities with widely varying prognostic and therapeutic implications. Within the brain, tumors can be divided into two main categories: primary and secondary brain tumors. Primary brain tumors arise from the brain parenchyma, meninges, nerves, or glands within the cranial cavity. In order to uniformly classify these tumors, the World Health Organization (WHO) has established an internationally accepted…

Evaluation of a dural-based lesion

Introduction There is significant overlap in the imaging appearance and presentation of dural-based mass lesions. The differential diagnosis includes both benign lesions (such as meningioma—WHO grade I) and more aggressive, malignant lesions (such as dural-based metastasis, atypical or malignant meningioma—WHO grade II and III, and lymphoma). Also included are inflammatory pathologies, such as IgG4-related disease (RD) and granulomatous disease (e.g., sarcoid). This chapter reviews several patient…

Evidence-based approaches to chemotherapy for gliomas

Introduction Chemotherapy has a crucial role in the management of gliomas. Traditionally, the management is characterized by an extensive surgery followed by a combination of radiotherapy (RT) and chemotherapy. In general, chemotherapy has been used as a neoadjuvant, adjuvant, or concurrent treatment, and our understanding of optimal use has continued to evolve over time. Neoadjuvant chemotherapy is administered prior to the main or definitive treatment, which…

Introduction to radiation therapy

Background: radiation biology and physics overview Radiation therapy treats cancer by causing DNA damage, most importantly double-strand breaks (DSBs). Although normal cells can efficiently detect and repair DSBs through homologous recombination or non-homologous end joining, malignant cells lack these mechanisms and DSBs are often lethal. As a result, malignant cells are more susceptible to radiation-induced DNA damage than normal cells. Importantly, normal cells can safely tolerate…

Surgical considerations for brain and spine tumors

Introduction to brain and spine tumor surgery For tumors affecting the brain and spinal cord, surgical resection is the fastest and most definitive treatment. In some cases, it is also the safest, such as when mass effect from the tumor compresses vital structures or when cerebral edema causes a life-threatening rise in intracranial pressure (ICP). Before making the decision to operate, the surgeon must weigh the…

Fundamentals of neuropathology: Introduction to neuropathology and molecular diagnostics

Introduction In typical cases, the clinical workflow for brain tumors begins with imaging studies; however, despite the increased sensitivity and capabilities of these methodologies, diagnosing brain tumors requires histopathologic evaluation of tissue. It is imperative for clinicians to understand how brain tumors are classified so that they can better counsel patients at the time of initial presentation, accurately describe prognosis, and prioritize management of medical or…

Treatment and Management of Hereditary Metabolic Myopathies

Hereditary metabolic myopathies are a group of muscle disorders that result from a shortage of energy production (i.e., deficiency of adenosine triphosphate [ATP]). In muscle, ATP is produced by aerobic glycogenesis or glycogenolysis and glycolysis, using the respiratory chain, or by anaerobic glycolysis, resulting in lactate production. For sustained work, muscle switches its energy source to fatty acid oxidation. Important pathways in muscle metabolism are shown…

Treatment and Management of Autoimmune Myopathies

The autoimmune inflammatory myopathies comprise five major and distinct subsets: polymyositis (PM), dermatomyositis (DM), necrotizing autoimmune myositis (NAM), overlap myositis and antisynthetase syndromes (OM-AS), and inclusion body myositis (IBM) ( ). Although moderate to severe muscle weakness, endomysial inflammation, and variable creatine kinase (CK) elevation are common features in all these conditions, unique clinical, immunopathologic, and histologic criteria characterize each subset ( ; , , ,…

Neuromuscular Manifestations of Acquired Metabolic, Endocrine, and Nutritional Disorders

Endocrine Disorders Table 21.1 summarizes the neuromuscular manifestation of these disorders. Table 21.1 Neuromuscular Manifestation of Endocrine Disorders Modified with permission from Bertorini T. E. (2008). Neuromuscular case studies . Philadelphia: Butterworth-Heinemann. Diabetes Symptoms Laboratory Test Results EMG Biopsy Treatment Acromegaly Proximal weakness, CTS, neuropathy, muscle atrophy Normal CK (may be elevated), elevated growth hormone, IGF-1, TSH Normal or myopathic Nonspecific; some fiber necrosis may be…

Treatment and Management of Muscular Dystrophies

Muscular dystrophies have long been recognized as heterogeneous inherited disorders, characterized by progressive skeletal muscle degeneration, weakness, and dystrophic changes in muscle biopsy. These diseases are known to have autosomal dominant, recessive, or X-linked inheritance. Clinical observations initially led to classification into six groups: Duchenne like, Emery-Dreifuss type, limb-girdle type, facioscapulohumeral peroneal type, distal myopathies, and oculopharyngeal type ( ). The expanding genetic and molecular understanding…

Treatment and Management of Disorders of the Neuromuscular Junction

Disorders of the neuromuscular junction include myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), botulism, congenital myasthenic syndrome (CMS), snakebite myasthenia, organophosphate poisoning (OPP), and hypermagnesium-induced paralysis. 1 1 Tick paralysis is excluded from this chapter because it is due to conduction block at the node of Ranvier. For this chapter, I reviewed all existing major literature on the subjects of management and treatment of these diseases…

Treatment and Management of Disorders of Neuromuscular Hyperexcitability and Periodic Paralysis

Disorders of Neuromuscular Hyperexcitability Muscle pain, stiffness, and cramps are symptoms frequently seen in neuromuscular practice. The differential diagnosis of these symptoms is wide and includes systemic disorders, neurologic disorders involving the pyramidal or extrapyramidal systems, and neuromuscular diseases ( Box 18.1 ). The neuromuscular diseases that manifest with muscle stiffness, cramps, and muscle pain as the predominant symptoms are referred to as the “syndromes of…

Treatment and Management of Segmental Neuromuscular Disorders

This chapter is devoted to focal disorders of the peripheral nervous system, including radiculopathies, plexopathies, and mononeuropathies. These conditions span a broad range of disorders, from radiculopathy due to Lyme disease and obturator neuropathy at the extremes of rarity to very common disorders such as cervical radiculopathy and carpal tunnel syndrome. This group of disorders makes up a large portion of neuromuscular medicine practice and only…

Treatment and Management of Infectious, Granulomatous, and Toxic Neuromuscular Disorders

The disorders that fall under infectious, granulomatous, and toxic neuromuscular disorders are too numerous and broad to be covered comprehensively in a single chapter. To achieve the objectives of this book, we focus on conditions that are seen more frequently and concentrate on the most important and relevant aspects of their management and treatment. Infections of Nerve and Muscle Human Immunodeficiency Virus Neuropathies and Myopathies The…

Treatment and Management of Autoimmune Neuropathies

Introduction Autoimmune peripheral neuropathies (APNs) develop when immunologic tolerance to key antigenic sites on myelin, axon, nodes of Ranvier, or ganglionic neurons is lost ( , ). These conditions may manifest either acutely or in a more chronic manner; symptoms may include motor, sensory, and autonomic in isolation or in concert. The classic prototypical syndromes are the Guillain-Barré syndrome (GBS)—an acute postinfectious sensorimotor neuropathy—and chronic inflammatory…

Treatment and Management of Hereditary Neuropathies

The authors thank Gerald Raymond for helpful feedback on this chapter. Overview of Inherited Neuropathies Hereditary neuropathy remains a major cause of neuropathy in patients referred to tertiary centers, estimated to represent 25% to 40% of all causes of neuropathy. With a prevalence of 1 per 2500, Charcot-Marie-Tooth (CMT) is the most common inherited neurologic disorder, affecting approximately 150,000 Americans. The advent of next-generation sequencing technologies…