Monoclonal Protein–Associated Neuropathies: Amyloid Neuropathy
IgM MGUS NEUROPATHY Although most patients who present with chronic distal sensory or sensorimotor symptoms and signs have an axonal polyneuropathy unrelated to a paraprotein, rarely, patients with distal clinical…
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
The diagnosis of CIDP can be challenging. Patients generally have both proximal and distal weakness on examination. Deep tendon reflexes are markedly reduced or absent. Distal sensory loss is often…
Diabetic Neuropathies
CLINICAL MANIFESTATIONS Diabetic polyneuropathy (DPN) is one of the most common neuropathies seen in clinical practices (see Plate 6-21). It is generally length dependent, with prominent sensory symptoms, and can…
Guillain-Barré Syndrome (Continued)
The differential diagnosis for AIDP needs to be carefully considered because the classic electrophysiologic features are often not present early in the disease process. Acute spinal cord lesions may be…
Guillain-Barré Syndrome
Mild cases of AIDP may never come to medical attention, but the typical presentation in those that seek medical assistance is one of acute ascending paralysis, which reaches its peak…
Hereditary Sensory and Autonomic Neuropathy
A careful history and neurologic examination is crucial in these patients. Family history is very important, particularly a history of painful feet and/or amputations. Neurologic examination should include testing of…
Hereditary Motor and Sensory Neuropathies (HMSN, i.e., Charcot-Marie-Tooth Disease)
The classifications of the different types of hereditary polyneuropathy were initially based on clinical and electrophysiologic features. More recently, however, advances in genetics have led to identification of many specific…
Hereditary Motor and Sensory Neuropathy Types I and II
Nerve conduction studies (NCS) distinguish predominantly demyelinating diseases, such as CMT1, from axonal neuropathies. In demyelinating neuropathies (e.g., CMT1a), conduction velocities are generally less than 60% of normal and may…
Other Hereditary Motor and Sensory Neuropathies (Types III, IV, and X)
The cerebrospinal fluid (CSF) protein is often significantly increased. Nerve conduction studies may show absent sensory potentials and marked slowing of motor conduction velocity, with values in the range of…