Cerebellar ataxia, neuropathy, and vestibular areflexia
syndrome is rare, idiopathic, slowly progressive neurodegenerative disorder.
The patient progressively improved, and the paresthesia areas retreated to hands and feet and dysarthria disappeared; however, the areflexia
Results of the vestibular tests using vectoelectronystagmography in relation to the type of alteration in the caloric reflex test in each age group Age Group/ Caloric reflex test Range Normal Abnormal n/% n/% G1(n=9) 7/78.00 2/32.00 20-39 years G2 (n=8) 7/87.50 1/1.50 40-49 years G3 (n=10) 7/70.00 3/30.00 50-59 years G4 (n=11) 6/54.50 5/45.50 60-69 years G5 (n=6) 4/67.00 2/33.00 70-99 years Total (n=44) 31/70.45 13/29.55 Age Group/ Type of abnormal in the caloric reflex test Range Hyperreflexia Hyporeflexia Areflexia
n/% n/% n/% G1(n=9) 1/50 1/50.00 0/0.00 20-39 years G2 (n=8) 0/0.00 1/100.00 0/0.00 40-49 years G3 (n=10) 0/0.00 2/66.50 1/33.50 50-59 years G4 (n=11) 0/0.00 5/100.00 0/0.00 60-69 years G5 (n=6) 0/0.00 2/100.00 0/0.00 70-99 years Total (n=44) 1/2.27 11/25.00 1/2.27 Table 2.
Miller-Fisher syndrome typically presents with areflexia
, ataxia, and ophthalmoplegia .
Guillain-Barre syndrome (GBS) is an inflammatory demyelinating peripheral neuropathy, characterized by acute weakness of the extremities and areflexia
. Acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) are the common subtypes of GBS [1, 2].
In 20% of patients, the BVP is associated with gangliopathy, described in 2011 as CANVAS (cerebellar ataxia, nonlength-dependent sensory neuropathy, vestibular areflexia
) , while 50% of the idiopathic BVH patients present with migraine according the International Headache Society criteria .
Miller Fisher (MF), a rare variant of GBS, was first described in 1956 due to its unique clinical symptoms, including ophthalmoplegia, areflexia
, and ataxia .
Peripheral neuropathies consist of injury or loss of peripheral nerves function, observed by conscious proprioceptive deficits, hypoalgesia or analgesia and hyporeflexia or areflexia
of the affected limb (VAN METRE et al., 2001; DIVERS, 2004).
A 6-year-old girl presented with fever, headache, and flaccid paralysis of the right arm with areflexia
. With little prompting, the students generated a short differential diagnosis.
Considering the age of complaint onset, absence of any known cardiac involvement, absence of similar complaints in family members, ENMG findings compliant with sensory-motor type neuropathy and accompanying areflexia
, the presence of generalized tonicclonic type seizures, our case was determined to indicate chorea-acanthocytosis, which is a neurodegenerative neuroacanthocytosis subgroup.
A case whose bladder's pathology changed from areflexia
to instability with impaired contractility was reported in a study by Porru and colleagues (7).
In addition to AHC, heterozygous mutations of the ATP1A3 gene have been reported in association with rapid-onset dystonia parkinsonism (RDP) and cerebellar ataxia, areflexia
, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) (Figure 2).