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Severe cranial nerve involvement in longstanding demyelinating polyneuropathy: a clinicopathologic correlation
Erin L. McCann1, T. W. Smith, David A. Chad, Jane Sargent2
Onion bulb formations involving cranial nerves are an unusual pathologic feature. We report the postmortem neuropathologic findings in a 69-year-old man with a longstanding neuropathy characterized by progressive muscle weakness, sensory ataxia and multiple cranial nerve abnormalities. Electrodiagnostic testing disclosed features of an acquired demyelinating polyneuropathy. Treatment with corticosteroids and plasmapheresis resulted in no change in his neurologic status, and the patient died after repeated episodes of pneumonia and sepsis. Autopsy showed widespread onion bulb formation in cranial nerves III, IV, V, VI, X, XI and XII, anterior and posterior spinal nerve roots, dorsal root ganglia and multiple peripheral nerves, some of which also had foci of epineurial perivascular inflammation. Muscle sections revealed severe neurogenic atrophy. This case demonstrates that, in longstanding acquired demyelinating neuropathy, the cranial nerves also undergo repetitive cycles of demyelination and remyelination resulting in severe weakness of the bulbar musculature and histologic features of hypertrophic neuropathy.
Key words Cranial nerves · Chronic inflammatory · demyelinating polyneuropathy · Onion bulb formations · Clinicopathologic study
Facial and trigeminal neuropathies as the initial manifestation of chronic inflammatory demyelinatingpolyradiculopathy.
Varela H, Rubin DI.
Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA.
To report the clinical and electrophysiological features of a patient who presented with facial weakness and numbness as the initial manifestation of Chronic inflammatory demyelinating polyradiculopathy (CIDP).
An 83-year-old woman presented with facial numbness and weakness. Examination demonstrated mild facial weakness with no other cranial abnormalities. Blink and jaw-jerk reflex latencies were prolonged bilaterally; myokymic discharges were seen in the right frontalis. Cerebrospinal fluid demonstrated an elevated protein. After 3 weeks of intravenous immunoglobulin treatment, the symptoms and electrophysiological findings improved but the symptoms recurred and became more generalized after cessation of intravenous immunoglobulin.
This case represents a patient with an unusual initial presentation of cranial nerve involvement in CIDP. Blink reflexes may be a useful electrophysiological technique to assess for peripheral nerve demyelination in patients with suspected CIDP.
Intern Med. 2009;48(22):1985-7. Epub 2009 Nov 16.
Transient oculomotor palsy correlated with nerve enhancement on MRI in chronic inflammatory demyelinatingpolyneuropathy.
Tsuda E, Imai T,.
Department of Neurology, Sapporo Medical University School of Medicine, Sapporo.
A 40-year-old woman was admitted to our hospital because of double vision combined with left ptosis. Although at 25 years of age she had already been diagnosed with limb weakness associated with chronic inflammatory demyelinating polyneuropathy (CIDP), she had never experienced double vision until her latest condition. Neurological examination revealed left oculomotor palsy without other cranial nerve involvement. Serial magnetic resonance imaging (MRI) studies demonstrated a temporal correlation between clinical severity of oculomotor palsy and segmental enhancement of the oculomotor nerve. Gadolinium enhancement on MRI may be a significant finding indicating relapse of oculomotor involvement of CIDP