Other tumors of the neural crest

Neuroblastoma

Ganglioneuroblastoma

Ganglioneuroma

Paraganglioma

Pheochromocytoma

Peripheral nerve Cranial nerves, nerve roots, the nerve plexus and single nerves can be affected involvement in cancer in cancer patients. The table gives an overview over the most frequently patients affected nerves (Table 12).

Table 12. Involvement of peripheral nerves in cancer patients

Nerve

Neoplastic

Therapy-related

Other causes

CN

Base of skull metastasis Leptomeningeal carcinomatosis Head and neck tumors

Toxicity of chemo- and radiotherapy

Axillary nerve

Surgery, mastectomy, neck dissection

Long thoracic nerve

Mastectomy Radiotherapy

Inflammatory neuropathy

Phrenic nerve

Lung cancer, lymphoma, thymoma

Thoracic surgery thymectomy

Critical illness neuropathy in intesive care patients and sepsis

Pectoral nerves

Neck dissection

Musculocutaneus nerve

Local metastasis

Perioperative position

Nerve

Neoplastic

Therapy-related

Other causes

Cutaneous antebrachii medialis nerve

Median nerve

Ulnar nerve Radial nerve Truncal nerves

Iliohypogastric nerve

Ilioinguinal nerve

Genitofemoral nerve

Cutaneus femoris lateral nerve

Femoral nerve

Obturator nerve Gluteus medius Sciatic nerve

Tibial nerve

Peroneal nerve

Neurolymphomatosis

C8 lesion, Pancoast Tumor

Metastasis, local metastasis into vertebral column, collapse of vertebral column

Local pelvic tumor, inguinal tumor or lymph nodes

Metastasis, obturator foramen

Recurrence of local tumor

Metastasis, Foramen piriforme

Local destruction of vertebral column, meningeal carcinomatosis

Compression of cauda equina Osteolysis of capitulum fibulae

All local mononeuropathies

Paravenous injection

Radiotherapy Malpostioning

Malpositioning, chemotherapy (vincristine)

Operations

Longterm steroid treatment with osteoporotic bone lesions

Renal operations

Abdominal surgery

Renal surgery

Surgery radiotherapy

Surgery, anticoagulation, radiotherapy

Surgery pelvis

Intraarterial cytostatic perfusion, radiotherapy

Malpositioning, cytostatic drugs (vincristine)

Intravenous Intraarterial perfusions

Amyloid deposition Paraproteinemia

Herpes Zoster

Injections, malpositioning

Rarely affected: cauda equina, sacral plexus lesion

Paraneoplastic Cachexia

Peroneal lesion may be part of sciatic nerve lesion

Basheer H, Rabia F, el-Hewl K (1997) Neurofibromas of digital nerves. J Hand Surg (Br) 22: References 61-63

Birch B (1993) Peripheral nerve tumors. In: Dyck PJ, Thomas PK, Griffin JP, Low PA, Poduslo JF (eds) Peripheral neuropathy. Saunders WB, Philadelphia, pp 1623-1640 Ferner RE, Lucas JD, O'Doherty MJO, et al (2000) Evaluation of 18 fluorodeoxyglucose positron emission tomography (18 FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry 68: 353-357

Foley KM, Woodruff M, Ellis FT (1980) Radiation induced malignant and atypical peripheral nerve sheath tumors. Ann Neurol 7: 311-318

Gabet JY (1989) Amyloid pseudotumor of the sciatic nerve. Rev Neurol 145: 872-876

Gijtenbeek JMM, Gabreels-Festen AAWM, Lammens M, et al (2001) Mononeuropathy multiplex as the initial manifestation of neurofibromatosis type 2. Neurology 56: 17661768

Krücke W (1955) Erkrankungen der peripheren Nerven. In: Lubarsch O, Henke F, Rössle R (Hrsg) Handbuch der speziellen pathologischen Anatomie und Histologie. Springer, Berlin, S1-248

Mitsumoto H (1992) Perineural cell hypertrophic mononeuropathy manifesting as CTS. Muscle Nerve 15: 1364-1368

Roncaroli F, Poppi M, Riccioni L, et al (1997) Primary non Hodgkin's lymphoma of the sciatic nerve folowed by localization in the central nervous system. Neurosurgery 40: 618621

Tang JB, Ishii S, Usui M, et al (1990) Multifocal neurilemomas in different nerves of the same upper extermity. J Hand Surg (Am) 15: 788-792

Thomas PK, King RHMT, Chiang TR, et al (1990) Neurofibromatous neuropathy. Muscle Nerve 13: 93-101

Yassini PR, Sauter K, Schochet SS, et al (2000) Localized hypertrophic mononeuropathy involving spinal roots and associated with sacral meningocele. Case report. J Neurosurg 79:774-778

Polyneuropathies

Fig. 1. Common stocking and glove distribution in polyneur-opathies

The peripheral nervous system (PNS) is defined as cell bodies or axons supported by Schwann cells. The PNS includes the cranial nerves (except the second cranial nerve), the dorsal root ganglia, the spinal nerve roots, the peripheral nerve trunks, and peripheral nerves. The peripheral autonomic system also lies within the PNS.

Peripheral neuropathy in its broadest definition encompasses any injury to the PNS. More precise terminology describes the specific site of PNS injury. Neuronopathies are direct injury to the cell bodies with a secondary axonal loss. Axonopathies represent a primary insult to axons; axonopathies, particularly when severe, can result in a secondary loss of cell bodies. A radiculopathy

Introduction is injury to spinal nerve roots while a plexopathy denotes injury in peripheral nerves as they course through a plexus. Polyneuropathy, the main focus of this chapter, refers to bilateral symmetrical injury to the peripheral nerves.

Polyneuropathy is commonly secondary to more generalized disease processes including systemic, metabolic or rheumatological disorders, cancer, vitamin deficiency states, exposure and/or ingestion of toxins and drugs, infections, immune reactions and inherited disorders of Schwann cell function. Table 13 provides a more complete list of disorders that lead to polyneuropathy. Multiple isolated peripheral nerve injuries, known as multiple mononeuropa-thies or mononeuropathy multiplex, are also usually due to systemic disease. It can be difficulty to distinguish near confluent mononeuropathy multiplex from generalized polyneuropathy. In contrast, isolated peripheral nerve injury is usually due to focal injury and is termed mononeuropathy. The mononeuropa-thies are discussed in chapter mononeuropathy.

The most common polyneuropathy has a distal distribution with loss of Anatomical sensory function beginning in the toes. As the sensory loss progresses to mid distribution calf, the patient experiences sensation loss in the fingertips, resulting in the classic stocking-glove distribution of distal symmetric polyneuropathy (Fig. 1). Reflex changes parallel sensory disturbances with ankle reflexes being first decreased then absent. Symptomatic distal motor nerve involvement is less common and, when present, suggests specific underlying systemic disease processes, particularly immune mediated and toxic neuropathies. Motor weakness can occur in a proximal distribution, leading to a proximal symmetric polyneuropathy. This pattern is also most commonly present in immune or toxic neuropathies. A pure sensory proximal symmetric polyneuropathy is very rare but can occur in acute intermittent porphyria. Another less common distribution of symmetric polyneuropathies is with initial motor or sensory loss in the arms. This can occur in immune mediated neuropathies, porphyria and inherited disorders of the PNS. Clinical syndrome Patients with polyneuropathy generally fall into two major classes: patients with negative symptoms and patients with positive symptoms. This distinction can be helpful to the clinician in both the diagnosis and care of the patient. As the term suggests, patients with negative symptoms have painless loss of sensory function or motor loss that does not perturb the patient's functional ability. Loss of sensation most commonly reflects loss of both large and small nerve fibers. Patients with negative symptoms develop the insensate foot with loss of vibratory perception and proprioception (large fiber) and light touch, temperature and pain sensation (small fiber). Eighty five percent of patients with diabetic polyneuropathy have no symptomatic complaints (i.e. negative sensory symptoms). This group of patients however is at high risk for ulcer formation because of their lack of pain sensation. In parallel negative motor symptoms, particularly atrophy of distal foot musculature, can lead to foot deformities and can also increase the risk of ulcers. Positive sensory symptoms can occur in patients with polyneuropathy in the absence or presence of external stimuli. At rest patients can experience painful parasthesias and/or frank pain. In response to normal stimuli such as light touch, patients may develop symptoms of hyperalgesia, dysesthesias or allodynia. Positive motor symptoms include cramps, fasciculations and functional weakness.

In summary, this chapter discusses the main polyneuropathies encountered by a physician in daily practice. It is not intended to be inclusive of all polyneuropathies but the disorders discussed should provide the clinician with the knowledge required to diagnose and treat nearly all patients seen in an outpatient clinic. The neuropathies will be discussed in the order outlined in Table 13. Some key abbreviations used in this discussion include CMAP (compound muscle action potential), SNAP (sensory nerve action potential), and CSF (cerebrospinal fluid).

Table 13. Differential diagnosis of polyneuropathy

Metabolic Disease

Diabetic distal symmetric polyneuropathy Diabetic autonomic neuropathy Diabetic mononeuritis multiplex Diabetic polyradiculopathy

Renal Disease

Systemic Disease Systemic vasculitis Non-systemic vasculitis Paraproteinemia Amyloidosis Cancer

Neoplastic disease Paraneoplastic disease Motor neuron disease syndrome Critical Illness

Infectious

Human Immunodeficiency Virus (HIV)

Hepatitis B

Lyme

Diphtheria Leprosy Syphylis Parasites

Inflammatory

Acute motor axonal neuropathy

Acute motor and sensory axonal neuropathy

Acute inflammatory demyelinating polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy

Chronic demyelinating polyradiculoneuropathy with anti-MAG antibodies

Miller-Fisher Syndrome

Multifocal Motor Neuropathy

Nutritional Cobalamin Post-gastroplasty Pyridoxine Strachan's syndrome Thiamine Tocopheral

Industrial Agents, Metals and Drugs Industrial Agents Acrylamide Carbon Disulfide Hexacarbons

Organophosphorous Agents

Table 13. Continued

Drugs

Alcohol Amiodarone Chloramphenicol Colchicine Dapsone Disulfiram Vinka alkaloids Platinum Taxol Metals

Arsenic

Mercury

Thallium

Hereditary

Hereditary Autonomic and Sensory Neuropathy

Hereditary Motor Sensory Neuropathy (Charcot-Marie-Tooth Disease) Types 1, 2

Hereditary Neuropathy with Pressure Palsies

Porphyria

Diabetes is the most common cause of neuropathy in the Western World. Metabolic diseases The 4 main peripheral nervous system complications of diabetes will be discussed: distal symmetric polyneuropathy, autonomic neuropathy, mononeuritis multiplex and the syndrome of plexopathy/polyradiculopathy that is frequently termed amyotrophy.

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