Severe deficits in vibration

and loss of CH5424802 proprioception were present up to the right elbow and right hip. Tendon stretch reflexes were symmetric. Cognitive evaluation showed impairments in immediate memory, delayed recall, and calculation. Speech and language examinations were normal. Because of her ongoing cognitive and subjective receptive language impairment and slowed information processing, she has been unable to resume her university studies. The final diagnosis was SHM with persistent neurologic deficits. Treatment with acetazolamide was initiated. SHM is a subtype of HM characterized by episodes of gradual progression of hemiparesis and at least 1 other neurological symptom/sign, in the absence of a first-degree relative with similar attacks. Although according to formal diagnostic criteria neurologic symptoms are fully reversible within 24 hours, many cases have been reported in which neurological deficits last up to several weeks.[1, 2, 4, 5] A few reports have illustrated irreversible neurological deficits.[3, 6] The main 2 types of permanent deficit described include cerebellar signs and cognitive deficits.[7] In this case, the patient had persistent cognitive deficits, but no cerebellar signs such as nystagmus, ataxia, and dysarthria. This patient also had severe and persistent hemisensory

HSP phosphorylation deficits in all domains, including proprioception. Although sensory symptoms are common during attacks of HM, such deficits are typically reversible.[7, 8] The pathophysiological mechanisms of HM are unclear. Cortical spreading depression is considered to play an important role in the aura symptoms of HM.[9, 10] Mutations in the ion transportation genes CACNA1A, ATP1A2, and SCN1A can cause familial hemiplegic migraine (FHM).[7, 11, 12] As a result of these mutations, there is altered membrane polarity, a lowered threshold for depolarization, and neuronal and/or glial excitability.[13] These ionic channelopathies result in a large and sustained depolarization-induced rise in extracellular synaptic glutamate through increased release and/or reduced removal

learn more or reuptake. A sustained and excessive rise in synaptic glutamate may result in neuronal death as a result of excitotoxicity.[7, 14] After 7 years of recurrent HM attacks, our patient developed persistent cognitive and sensory impairments following her most severe HM attack. There was no evidence of cerebral infarction and no evidence of cerebellar atrophy, the latter of which has been previously reported in patients with HM.[15] Glucose hypometabolism and cerebral hypoperfusion have been reported in patients with HM.[3, 6, 9, 10] However, fluorodeoxyglucose positron emission tomography, single photon emission computed tomography, and perfusion studies were not performed in the evaluation of this patient. Nonetheless, the patient’s persistent symptoms and continued abnormalities on neurologic examination suggest that irreversible neuronal damage accounts for the persistent symptoms.