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SENSORY PREDOMINANT
NEUROPATHY
Ade Wijaya – July 2019
Botez SA, Herrmann DN. Sensory neuropathies, from symptoms to treatment. Current opinion in neurology. 2010 Oct 1;23(5):502-8.
INHERITED NEUROPATHIES WITH SENSORY
INVOLVEMENT
• Hereditary sensory and autonomic neuropathies
• Familial amyloid polyneuropathies (FAPs)
• Neuropathies associated with mitochondrial disorders
• Charcot–Marie–Tooth (CMT) disease
Burns TM, Phillips LH 2nd, Dimberg EL, et al. Novel myelin protein zero mutation (Arg36Trp) in a patient with acute onset painful neuropathy. Neuromuscul Disord 2006; 16:308–310.
Sowden JE, Logigian EL, Malik K, Herrmann DN. Genotype-phenotype correlation in a family with late onset CMT and an MPZ lys236del mutation. J Neurol Neurosurg Psychiatry 2005; 76:442–444.
MANAGEMENT
IMMUNE-MEDIATED SENSORY NEUROPATHIES AND SENSORY
NEURONOPATHIES
• IVIG for ataxic SNN or progressive refractory painful sensory
neuropathies or SFSN in Sjogren’s syndrome
• TNF-a inhibitor (etanercept) considered in patients with progressive,
disabling SNN in the setting of connective tissue disorders, who are
refractory to IVIG and in whom there is no evidence of central or
peripheral nervous system demyelination (a potential complication of
TNF-a blockade)
Figueroa JJ, Tabechian D, Herrmann DN. Severe long-standing dysimmune sensory neuronopathy responsive to etanercept. J Clin Neuromuscul Dis 2008; 9:415–420
Caroyer JM, Manto MU, Steinfeld SD. Severe sensory neuronopathy responsive to infliximab in primary Sjo¨ gren’s syndrome. Neurology 2002; 59:1113–1114.
Morozumi S, Kawagashira Y, Iijima M, et al. Intravenous immunoglobulin treatment for painful sensory neuropathy associated with Sjo¨ gren’s syndrome.J Neurol Sci 2009; 279:57–61.
HIV - NEUROPATHY
• DSP is the most common neurological complication of HIV infection
• Symptoms are usually symmetric, mainly distal and sensory with only
minimal motor involvement
• Pathogenesis: indirect neurotoxicity of the HIV virus via immune
activation and direct neurotoxicity of HIV proteins
• Acute toxic neuropathy (ATN) due to dideoxynucleoside reverse
transcriptase inhibitor drugs
• Management: gabapentin, high concentrated capsaicin patch
Botez SA, Herrmann DN. Sensory neuropathies, from symptoms to treatment. Current opinion in neurology. 2010 Oct 1;23(5):502-8.
DIABETIC NEUROPATHY -
MANAGEMENT
• FDA approved: pregabalin dan duloxetine
• TENS as adjunctive therapy
• Botox injections
Botez SA, Herrmann DN. Sensory neuropathies, from symptoms to treatment. Current opinion in neurology. 2010 Oct 1;23(5):502-8.
SUMMARY
• Sensory neuropathies are challenging because of their diversity
• Diagnostic criteria for DSP, SFSN and sensory ganglionopathies have
improved with the ever widening array of neuropathy assessment
tools such as skin biopsy, which permit more precise characterization
of sensory neuropathies
• Genetic testing allows the diagnosis of sensory predominant
hereditary neuropathies, providing insight into disease mechanisms
and potential treatments
• IVIG are demonstrating promise for a widening range of immune-
mediated sensory neuropathies
Sensory Predominant Neuropathy

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Sensory Predominant Neuropathy

  • 2.
  • 3. Botez SA, Herrmann DN. Sensory neuropathies, from symptoms to treatment. Current opinion in neurology. 2010 Oct 1;23(5):502-8.
  • 4. INHERITED NEUROPATHIES WITH SENSORY INVOLVEMENT • Hereditary sensory and autonomic neuropathies • Familial amyloid polyneuropathies (FAPs) • Neuropathies associated with mitochondrial disorders • Charcot–Marie–Tooth (CMT) disease Burns TM, Phillips LH 2nd, Dimberg EL, et al. Novel myelin protein zero mutation (Arg36Trp) in a patient with acute onset painful neuropathy. Neuromuscul Disord 2006; 16:308–310. Sowden JE, Logigian EL, Malik K, Herrmann DN. Genotype-phenotype correlation in a family with late onset CMT and an MPZ lys236del mutation. J Neurol Neurosurg Psychiatry 2005; 76:442–444.
  • 5. MANAGEMENT IMMUNE-MEDIATED SENSORY NEUROPATHIES AND SENSORY NEURONOPATHIES • IVIG for ataxic SNN or progressive refractory painful sensory neuropathies or SFSN in Sjogren’s syndrome • TNF-a inhibitor (etanercept) considered in patients with progressive, disabling SNN in the setting of connective tissue disorders, who are refractory to IVIG and in whom there is no evidence of central or peripheral nervous system demyelination (a potential complication of TNF-a blockade) Figueroa JJ, Tabechian D, Herrmann DN. Severe long-standing dysimmune sensory neuronopathy responsive to etanercept. J Clin Neuromuscul Dis 2008; 9:415–420 Caroyer JM, Manto MU, Steinfeld SD. Severe sensory neuronopathy responsive to infliximab in primary Sjo¨ gren’s syndrome. Neurology 2002; 59:1113–1114. Morozumi S, Kawagashira Y, Iijima M, et al. Intravenous immunoglobulin treatment for painful sensory neuropathy associated with Sjo¨ gren’s syndrome.J Neurol Sci 2009; 279:57–61.
  • 6. HIV - NEUROPATHY • DSP is the most common neurological complication of HIV infection • Symptoms are usually symmetric, mainly distal and sensory with only minimal motor involvement • Pathogenesis: indirect neurotoxicity of the HIV virus via immune activation and direct neurotoxicity of HIV proteins • Acute toxic neuropathy (ATN) due to dideoxynucleoside reverse transcriptase inhibitor drugs • Management: gabapentin, high concentrated capsaicin patch Botez SA, Herrmann DN. Sensory neuropathies, from symptoms to treatment. Current opinion in neurology. 2010 Oct 1;23(5):502-8.
  • 7. DIABETIC NEUROPATHY - MANAGEMENT • FDA approved: pregabalin dan duloxetine • TENS as adjunctive therapy • Botox injections Botez SA, Herrmann DN. Sensory neuropathies, from symptoms to treatment. Current opinion in neurology. 2010 Oct 1;23(5):502-8.
  • 8. SUMMARY • Sensory neuropathies are challenging because of their diversity • Diagnostic criteria for DSP, SFSN and sensory ganglionopathies have improved with the ever widening array of neuropathy assessment tools such as skin biopsy, which permit more precise characterization of sensory neuropathies • Genetic testing allows the diagnosis of sensory predominant hereditary neuropathies, providing insight into disease mechanisms and potential treatments • IVIG are demonstrating promise for a widening range of immune- mediated sensory neuropathies