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Background: The intranasal (IN) route for rapid drug administration in patients with brain disorders, including status epilepticus, has been investigated. Status epilepticus is an emergency, and the IN route offers a valuable alternative to other routes, especially when these fail.Objectives: To compare IN versus IV midazolam (MDZ) at the same dosage (0.2 mg/kg) for controlling status epilepticus in dogs.Abbreviations: BBB, blood-brain barrier; IN, intranasal; MAD, mucosal atomization device; MDZ, midazolam.

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A six-month-old West Highland white terrier with progressive, multifocal neurological disease was diagnosed with canine globoid cell leucodystrophy (GCL). Magnetic resonance imaging (MRI) of the brain was performed, as well as electrophysiological testing (including brainstem auditory evoked response, peripheral nerve conduction velocity, repetitive stimulation, F wave analysis and electromyography). MRI findings were consistent with diffuse, symmetrical white matter disease. Electrodiagnostic testing revealed evidence of peripheral neuropathy and an abnormal brainstem auditory evoked response. These observations were consistent with the pathological changes in central and peripheral white matter described for canine GCL, and resembled what has been described in human patients. It is believed that the tests may raise the suspicion of GCL in dogs and may aid in monitoring disease progression.