Genes and Inherited Neuropathies
Figure 78-1 The relationships of inherited neuropathies to each other and other syndromes. These Venn diagrams depict that dominantly inherited neuropathies can be separated into demyelinating and axonal forms (A);…
Schwann Cell Investment of Unmyelinated Nerve Fiber
Figure 73-1 A teased unmyelinated fiber cluster (UFC) stained with cresyl violet. The preparation allows linear measurement of internuclear lengths (upper panel). Transverse sections of a teased UFC under light…
Corneal Lattice Dystrophy, Facial Weakness and Sensorimotor Polyneuropathy—Gelsolin Amyloidosis
Figure 34-1 Symmetric facial weakness and skin laxity. Her family history was significant for multiple similarly affected family members shown in the pedigree (Fig. 34-2). Her position in the pedigree…
A Distinct Neuropathy with Hypotonia, Kinky Hair, and Axonal Spheroids—Giant Axonal Neuropathy
Figure 28-1 The patient showing the kinky hair described in text. Nerve conduction studies demonstrated absent upper extremity sensory responses. The sural response was low normal amplitude (8 μV) with…
Role of High-Resolution Imaging in Patients with Painful Lumbosacral Plexopathy
Figure 79-1 Pelvic magnetic resonance imaging scan with and without gadolinium. A, Oblique coronal T1-weighted image (T1WI) demonstrates isointense enlargement of the right S1 and S2 nerve roots. B, Fat-saturated…
Amyloidosis Typing Based on Laser Microdissection and Mass Spectrometry of Paraffin-Embedded Tissue Biopsies
Figure 80-1 Illustration of the process of amyloid specific microdissection. A, Peripheral nerve biopsy stained with Congo red indicating amyloid positive regions (arrows). B, Amyloid-positive areas are selected and outlined…
Hemorrhagic Quadriplegic Polyneuropathy
Figure 53-1 iThe extensive bleeding into sciatic nerve (A) and its multifocal distribution at different proximal to distal levels of a freshly isolated fascicle of sciatic nerve before fixation (B)…
“Small-Fiber” Neuropathy, Angiokeratoma, and Heat Intolerance—Fabry’s Disease
Figure 27-1 A, Skin angiokeratoma appear as raised red lesions over the lateral flank (arrows). These are typical lesions in Fabry’s disease. B, Angiokeratomas are typically seen over pressure points,…
Bilateral Phrenic Nerve Injury Following Radiofrequency Catheter Ablation for Atrial Fibrillation
Figure 69-1 Chest x-ray (posteroanterior view) showing elevation of the diaphragm, right greater than left. Bilateral phrenic nerve conduction studies3 were similar at 2, 8, and 12 months. On the…
Evaluation of Diabetic Polyneuropathy: Design of the Neurologic Examination Versus Clinical Neurophysiology Tests Trial
Figure 75-1 The report sheet that was completed by investigators. Participating physicians had been asked before and at the study not to discuss the approaches and criteria they planned on…
