the onset and progression were gradual . over the past year , dysarthria and liquid dysphagia developed and progressed . he had been a problem alcohol drinker for the past 7 years . over the past 3 years one year earlier , he was admitted to a local psychiatric hospital because of abnormal behavior , such as stealing alcohol from a store . he was told that the results of brain magnetic resonance imaging ( mri ) performed during that admission were abnormal , but this was not followed up . his elder brother had alopecia and a similar neurological condition , and died at the age of 46 years . he was alert and oriented . gaze - evoked nystagmus was observed in both directions and was worse when looking to the right . a review of the previous brain mri results showed mild brainstem and cerebellar atrophy , with a subtly increased signal intensity in the right middle cerebellar peduncle on fluid attenuated inversion - recovery ( flair ) imaging . the flair images showed symmetric high - intensity signals in the posterior limbs of the internal capsule , right middle cerebellar peduncle , and brachia of the inferior colliculus where it joins the medial geniculate body ( figure 1 ) . complete blood counts , erythrocyte sedimentation rate ( esr ) , urinalysis , plasma electrolytes , and kidney , liver , and thyroid function tests were normal . vitamin b12 was somewhat low at 196 pg / ml ( normal range , 2001,000 pg / ml ) , and the folate level was low at 1.3 ng / ml ( normal range , 315 ng / ml ) . gene tests for spinocerebellar ataxia types 13 , 6 , 7 , and 17 ; dentatorubropallidoluysian atrophy ; fragile x syndrome ; and friedreich s ataxia were all negative . the plasma levels of pyruvate , lactic acid , homocysteine , and tumor markers were all normal . the adrenocorticotropic hormone ( acth ) level at 8 a.m. was 90 pg / ml ( normal range , 060 pg / ml ) and the cortisol level was normal at 10.2 g / dl ( normal range , 525 g / dl ) . an adrenocorticotropic hormone ( acth ) a very long - chain fatty acid assay showed a c22 : 0 of 2.565 m / l ( normal , < 1.310 m / l ) , c24 : 0/c22 : 0 of 1.785 ( normal , < 1.390 ) , and c26 : 0/c22 : 0 of 0.114 ( normal , < 0.023 ) . a published classification divides the condition into child - onset cerebral , adolescent - onset cerebral , amn , adult - onset cerebral , addison s disease only , and non - symptomatic ald , based on the onset time and organs involved.1 the most common adult - onset ald is amn . however , rare cases presenting as progressive cerebellar dysfunction have been reported.210 one study analyzed the mri scans of 164 adult - onset ald patients who were classified into four subgroups based on their symptoms and patterns of brain involvement : amn with normal brain , amn with brain abnormalities limited to the long tract ( almn i ) , amn with diffuse lobar cerebral involvement ( almn ii ) , and adult - onset cerebral ald.11 our patient had progressive cerebellar ataxia . on mri , demyelination lesions were observed in the cerebellar white matter , including the dentate nuclei and right cerebellar peduncle , posterior limbs of both internal capsules , and auditory pathway . there is a report of a patient who had clinical symptoms similar to our case , and that patient was classified as almn i , or somewhere between almn i and ii.9 in another case of adult - onset ald showing slowly developing gait ataxia over 7 years , demyelination lesions were observed in the dentate nucleus , middle cerebellar peduncle , decussation of the superior cerebellar peduncles , and posterior limbs of both internal capsules on brain mri ; two years later , new white matter lesions were seen in the right frontal lobe , left occipital lobe , and both temporal lobes on brain mri.2 the brain mri findings in other cases of ald presenting as progressive cerebellar dysfunction are summarized in table 1 . our patient had a history of alcohol abuse , aggression , and abnormal behavior before the onset of cerebellar ataxia . preceding psychiatric symptoms have often been reported in adult - onset ald . in a review of 109 cases of ald , six cases presented after the age of 20 years , and four patients had psychiatric symptoms at the time of presentation.12 among 34 adult - onset ald cases , 19 ( 56% ) were reported to have psychiatric symptoms.13 in 13 of these cases , the psychiatric symptoms were assessed relatively thoroughly , and eight cases showed changes in behavior and character . these symptoms appeared between 2 months and 8 years before the onset of other neurological symptoms . of the 13 patients , 12 were reported to have bipolar disorder , including disinhibition and emotional lability , with two of them reported to abuse alcohol or other substances.13 when brain mri shows brainstem and cerebellar atrophy and demyelination lesions along the long tracts , the possibility of ald should be considered . accompanying adrenal insufficiency may be an important clue , but 30% of adult - onset ald cases have normal adrenal function.14 hyperpigmentation of the skin , alopecia , and prior psychotic symptoms are further clues to the diagnosis of ald .
x - linked adrenoleukodystrophy ( x - ald ) is a hereditary neurological disorder affecting the nervous system and adrenal cortex . 
 the phenotype of x - ald ranges from the rapidly progressive cerebral form to milder adrenomyeloneuropathy . however , cerebellar manifestations are rare . 
 we report a case of adrenoleukodystrophy presenting as progressive cerebellar dysfunction resembling olivopontocerebellar degeneration , with a review of the literature