The Bruns-Garland Syndrome (Diabetic Amyotrophy)

Diabetic amyotrophy progressing to severe quadriparesis

“…Sural nerve biopsies showed primary changes of axon loss with predominant loss of large myelinated fibers; perivascular inflammatory changes were prominent in one case but absent in the other. These findings are consistent with those that have been previously reported in diabetes mellitus–associated neuropathies 1–3, 6, 11, 18, 21…”

“…The presence of distal sensory peripheral neuropathy in both cases supports the view that the various forms of diabetic peripheral nervous system disease are not necessarily mutually exclusive conditions 1, 2, 6, 7, 11, 13. While a case can be argued for distinct diabetic neuropathies, alternatively there may be only a few pathologically distinct processes (metabolic or ischemic; inflammatory or noninflammatory), with shifts in emphasis determining the clinical expression 3, 18, 21.…”

“…The two cases we present indicate the potential for patients presenting with diabetic amyotrophy to progress to severe quadriparesis, a finding not documented in previously published large series 1, 2, 6, 11. In both cases, other nondiabetic causes of neuropathy were carefully excluded by clinical assessment and laboratory investigations.…”

“…Sural nerve biopsy on light microscopy showed prominent axonal loss mainly involving large myelinated (10–12 μm) fibers, patchy perivascular mononuclear cell infiltrates without involvement of the vessel wall, and no demyelination, consistent with diabetic neuropathy 1, 3, 8–10, 18, 21. Electron microscopy showed loss of myelinated fibers.…”

Diabetic amyotrophy progressing to severe quadriparesis

“…Sural nerve biopsies showed primary changes of axon loss with predominant loss of large myelinated fibers; perivascular inflammatory changes were prominent in one case but absent in the other. These findings are consistent with those that have been previously reported in diabetes mellitus–associated neuropathies 1–3, 6, 11, 18, 21…”

“…The presence of distal sensory peripheral neuropathy in both cases supports the view that the various forms of diabetic peripheral nervous system disease are not necessarily mutually exclusive conditions 1, 2, 6, 7, 11, 13. While a case can be argued for distinct diabetic neuropathies, alternatively there may be only a few pathologically distinct processes (metabolic or ischemic; inflammatory or noninflammatory), with shifts in emphasis determining the clinical expression 3, 18, 21.…”

“…The two cases we present indicate the potential for patients presenting with diabetic amyotrophy to progress to severe quadriparesis, a finding not documented in previously published large series 1, 2, 6, 11. In both cases, other nondiabetic causes of neuropathy were carefully excluded by clinical assessment and laboratory investigations.…”

“…Sural nerve biopsy on light microscopy showed prominent axonal loss mainly involving large myelinated (10–12 μm) fibers, patchy perivascular mononuclear cell infiltrates without involvement of the vessel wall, and no demyelination, consistent with diabetic neuropathy 1, 3, 8–10, 18, 21. Electron microscopy showed loss of myelinated fibers.…”

Intravenous immunoglobulin therapy in proximal diabetic neuropathy

“…Two courses of IVIg significantly reduced severe pain and muscle weakness. The clinical course and laboratory findings observed in this patient were concordant with those of typical PDN 1 , 4. PDN is often accompanied by distal symmetrical sensory polyneuropathy as well as diabetic thoracoabdominal neuropathy, as in this patient 5 , 9…”

Diabetic amyotrophy progressing to severe quadriparesis

“…Sural nerve biopsies showed primary changes of axon loss with predominant loss of large myelinated fibers; perivascular inflammatory changes were prominent in one case but absent in the other. These findings are consistent with those that have been previously reported in diabetes mellitus–associated neuropathies 1–3, 6, 11, 18, 21…”

“…The presence of distal sensory peripheral neuropathy in both cases supports the view that the various forms of diabetic peripheral nervous system disease are not necessarily mutually exclusive conditions 1, 2, 6, 7, 11, 13. While a case can be argued for distinct diabetic neuropathies, alternatively there may be only a few pathologically distinct processes (metabolic or ischemic; inflammatory or noninflammatory), with shifts in emphasis determining the clinical expression 3, 18, 21.…”

“…The two cases we present indicate the potential for patients presenting with diabetic amyotrophy to progress to severe quadriparesis, a finding not documented in previously published large series 1, 2, 6, 11. In both cases, other nondiabetic causes of neuropathy were carefully excluded by clinical assessment and laboratory investigations.…”

“…Sural nerve biopsy on light microscopy showed prominent axonal loss mainly involving large myelinated (10–12 μm) fibers, patchy perivascular mononuclear cell infiltrates without involvement of the vessel wall, and no demyelination, consistent with diabetic neuropathy 1, 3, 8–10, 18, 21. Electron microscopy showed loss of myelinated fibers.…”

Intravenous immunoglobulin therapy in proximal diabetic neuropathy

“…Two courses of IVIg significantly reduced severe pain and muscle weakness. The clinical course and laboratory findings observed in this patient were concordant with those of typical PDN 1 , 4. PDN is often accompanied by distal symmetrical sensory polyneuropathy as well as diabetic thoracoabdominal neuropathy, as in this patient 5 , 9…”

Diabetic amyotrophy progressing to severe quadriparesis

“…Sural nerve biopsies showed primary changes of axon loss with predominant loss of large myelinated fibers; perivascular inflammatory changes were prominent in one case but absent in the other. These findings are consistent with those that have been previously reported in diabetes mellitus–associated neuropathies 1–3, 6, 11, 18, 21…”

“…The presence of distal sensory peripheral neuropathy in both cases supports the view that the various forms of diabetic peripheral nervous system disease are not necessarily mutually exclusive conditions 1, 2, 6, 7, 11, 13. While a case can be argued for distinct diabetic neuropathies, alternatively there may be only a few pathologically distinct processes (metabolic or ischemic; inflammatory or noninflammatory), with shifts in emphasis determining the clinical expression 3, 18, 21.…”

“…The two cases we present indicate the potential for patients presenting with diabetic amyotrophy to progress to severe quadriparesis, a finding not documented in previously published large series 1, 2, 6, 11. In both cases, other nondiabetic causes of neuropathy were carefully excluded by clinical assessment and laboratory investigations.…”

“…Sural nerve biopsy on light microscopy showed prominent axonal loss mainly involving large myelinated (10–12 μm) fibers, patchy perivascular mononuclear cell infiltrates without involvement of the vessel wall, and no demyelination, consistent with diabetic neuropathy 1, 3, 8–10, 18, 21. Electron microscopy showed loss of myelinated fibers.…”

Intravenous immunoglobulin therapy in proximal diabetic neuropathy

“…Two courses of IVIg significantly reduced severe pain and muscle weakness. The clinical course and laboratory findings observed in this patient were concordant with those of typical PDN 1 , 4. PDN is often accompanied by distal symmetrical sensory polyneuropathy as well as diabetic thoracoabdominal neuropathy, as in this patient 5 , 9…”