Pathological study of pseudohypertrophy of the inferior olivary nucleus

Ogawa, Kōichi; Mizutani, Tetsuya; Uehara, Kenji; Minami, Masayuki; Suzuki, Yoshio; Uchihara, Toshiki

doi:10.1111/j.1440-1789.2009.01033.x

Cited by 18 publications

(10 citation statements)

References 31 publications

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“…These pathological hallmarks have been thought to result from transynaptic degeneration secondary to a lesion of the ipsilateral central tegmental tract or the contralateral dentate nucleus. More recent immunohistochemical studies identified various changes in the neurons, their neurites, and presynaptic terminals confirming this hypothesis (74, 75). The main finding is a decreased synaptophysin immunoreactivity confirming the presynaptic abnormalities linked to deafferentation (75).…”

Section: Neuropathology Of the Degenerative Hypertrophic Inferior Olimentioning

confidence: 61%

“…More recent immunohistochemical studies identified various changes in the neurons, their neurites, and presynaptic terminals confirming this hypothesis (74, 75). The main finding is a decreased synaptophysin immunoreactivity confirming the presynaptic abnormalities linked to deafferentation (75). In 1981, Goto and Kaneko published a neuropathological study of eight cases of pontine hemorrhage involving unilaterally or bilaterally central tegmental tracts with different survival periods (76).…”

Section: Neuropathology Of the Degenerative Hypertrophic Inferior Olimentioning

confidence: 61%

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Hypertrophic Olivary Degeneration and Palatal or Oculopalatal Tremor

2017

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Hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor (PT) or oculopalatal tremor (OPT). This syndrome manifests as a synchronous tremor of the palate (PT) and/or eyes (OPT) that may also involve other muscles from the branchial arches. It is associated with hypertrophic inferior olivary degeneration that is characterized by enlarged and vacuolated neurons, increased number and size of astrocytes, severe fibrillary gliosis, and demyelination. It appears on MRI as an increased T2/FLAIR signal intensity and enlargement of the inferior olive. There are two main conditions in which hypertrophic degeneration of the inferior olive occurs. The most frequent, studied, and reported condition is the development of PT/OPT and hypertrophic degeneration of the inferior olive in the weeks or months following a structural brainstem or cerebellar lesion. This “symptomatic” condition requires a destructive lesion in the Guillain–Mollaret pathway, which spans from the contralateral dentate nucleus via the brachium conjunctivum and the ipsilateral central tegmental tract innervating the inferior olive. The most frequent etiologies of destructive lesion are stroke (hemorrhagic more often than ischemic), brain trauma, brainstem tumors, and surgical or gamma knife treatment of brainstem cavernoma. The most accepted explanation for this symptomatic PT/OPT is that denervated olivary neurons released from inhibitory inputs enlarge and develop sustained synchronized oscillations. The cerebellum then modulates/accentuates this signal resulting in abnormal motor output in the branchial arches. In a second condition, PT/OPT and progressive cerebellar ataxia occurs in patients without structural brainstem or cerebellar lesion, other than cerebellar atrophy. This syndrome of progressive ataxia and palatal tremor may be sporadic or familial. In the familial form, where hypertrophic degeneration of the inferior olive may not occur (or not reported), the main reported etiologies are Alexander disease, polymerase gamma mutation, and spinocerebellar ataxia type 20. Whether or not these are associated with specific degeneration of the dentato–olivary pathway remain to be determined. The most symptomatic consequence of OPT is eye oscillations. Therapeutic trials suggest gabapentin or memantine as valuable drugs to treat eye oscillations in OPT.

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Section: Neuropathology Of the Degenerative Hypertrophic Inferior Olimentioning

confidence: 61%

Section: Neuropathology Of the Degenerative Hypertrophic Inferior Olimentioning

confidence: 61%

Hypertrophic Olivary Degeneration and Palatal or Oculopalatal Tremor

2017

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“…12 Previous studies have reported neuropathological findings, including neuronal positivity for αB-crystallin (αBC), p-NFP (SMI31), microtubuleassociated protein 2 (MAP2), Lys-Asp-Glu-Leu (KDEL) receptors, and presence of synaptophysin-positive granules to be associated with inferior olivary hypertrophy. 13,14 However, the expression levels of these proteins among patients, including that of αBC, showed large variance, 14 suggesting the possible involvement of several cellular pathways in inferior olivary hypertrophy. The inferior olivary hypertrophy observed in our case was "isolated", possibly induced by a special mechanism.…”

Section: Discussionmentioning

confidence: 99%

A clinicopathological study of ALS with L126S mutation in the SOD1 gene presenting with isolated inferior olivary hypertrophy

et al. 2019

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We report an autopsy case of amyotrophic lateral sclerosis with L126S mutation in the superoxide dismutase 1 (SOD1) gene (SOD1). The patient was a 69‐year‐old Japanese man without relevant family history, who initially presented with slow progressive muscle weakness of the lower extremities without upper motor neuron signs, and died of respiratory failure 6 years after the onset. Neuropathological examination revealed a loss of lower motor neurons and degeneration of Clarke's column commensurate with that of the posterior spinocerebellar tract and the middle root zone of the posterior column. The primary motor area was minimally affected. Characteristic SOD1‐immunopositive neuronal intracytoplasmic inclusions, mixed with neurofilament accumulation, were present in the affected areas. Isolated inferior olivary hypertrophy was observed, but did not involve the contralateral dentate nucleus, or the ipsilateral red nucleus and central tegmental tract, where no neuronal inclusions were found. In combination with data from a previous autopsy case, this study suggests that the L126S mutation may cause focal neuronal degeneration in the brainstem.

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“…Second, central tegmental tract connects the red nucleus to ipsilateral ION at the medulla. Last, ION sends efferents through the inferior cerebellar peduncle, terminating at the contralateral dentate nucleus, which ends up forming the dentate-rubral-olivary circuitry [9][10][11][12][13][14]. A lesion in any part of this synaptic pathway may result in HOD and is accompanied, due to deafferentation, by atrophy of ipsilateral midbrain and contralateral cerebellar hemisphere, exactly as our patient.…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic Olivary Degeneration Secondary to Neuro-Behçet’s Disease

et al. 2015

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A 22-year-old man presented with an acute altered mental status, left third cranial nerve palsy, ataxia, and right side weakness, all of which suggestive of brainstem encephalitis. Six months earlier, he had persistent fever, myalgia, and weight loss. Soon after fever resolution, oral and scrotal ulcers, usually painless, appeared accompanied by acneiform skin lesions in the chest and arms. At the time, antibiotic treatment led to partial regression of the lesions.Brain magnetic resonance imaging (MRI) showed hyperintense lesions on left midbrain, thalamus, and subcortical regions (Fig. 1). Cerebrospinal fluid analysis showed only mild lymphocytic pleocytosis. Pulse therapy with methylprednisolone 1 g/day for 7 days followed outstanding clinical improvement, although residual right hemiparesis and kinetic tremor remained. At discharge, he was on prednisone 20 mg/day and azathioprine 150 mg/day. Four months after therapy onset, a control brain MRI demonstrated resolution of most lesions along with signal alteration and enlargement of the olivary nuclei in the left medulla (Fig. 2a). At the time, he presented only minor oculopalatal myoclonus. Clinical stability and imaging pattern of the lesion suggested a benign finding, and no further investigation was pursued.Six years later, brain MRI showed an atrophic left midbrain peduncle with less evident olivary degeneration. In addition, there was significant atrophy of right cerebellar hemisphere (Fig. 2b) and shrinkage of midbrain (Fig. 2c, d). At that point, the patient had only mild right hemiparesis and oculopalatal myoclonus. Azathioprine 150 mg/day was his only medication, and he remains stable until this moment.

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Pathological study of pseudohypertrophy of the inferior olivary nucleus

Cited by 18 publications

References 31 publications

Hypertrophic Olivary Degeneration and Palatal or Oculopalatal Tremor

Hypertrophic Olivary Degeneration and Palatal or Oculopalatal Tremor

A clinicopathological study of ALS with L126S mutation in the SOD1 gene presenting with isolated inferior olivary hypertrophy

Hypertrophic Olivary Degeneration Secondary to Neuro-Behçet’s Disease

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