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| Year : 2006 | Volume
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| Issue : 4 | Page : 701-704 |
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| Hypertrophic olivary degeneration following trauma - a case report |
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V Arora, IS Nijjar, PS Sandhu, J Singh, R Abrol, Roopa
Nijjar Scan & Diagnostic Centre, Amritsar, India
Click here for correspondence address and email
| Date of Submission | 28-Oct-2006 |
| Date of Acceptance | 20-Nov-2006 |
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 Abstract | | |
Hypertrophic Olivary degeneration (HOD) is a rare type of neuronal degeneration involving the dentatorubro-olivary pathway. It is a secondary change caused by the impairment of Guillain Mollaret triangle. We present a case of a thirty-five years old
man who had head injury with MR evidence of haemorrhagic contusion at the ponto-mesencephalic junction extending into the superior cerebellar peduncle on left side. Five months after the injury he developed left upper extremity rhythmic tremor. Repeat MRI permitted visualization of hypertrophic olivary degeneration, which appeared as a characteristic high signal intensity in the right inferior olivary nucleus on T2 W and FLAIR images, with enlargement of the nucleus. The literature and imaging findings of this uncommon condition are reviewed. Keywords: Hypertrophic Olivary, degeneration; magnetic resonance imaging
How to cite this article:
Arora V, Nijjar I S, Sandhu P S, Singh J, Abrol R, Roopa. Hypertrophic olivary degeneration following trauma - a case report. Indian J Radiol Imaging 2006;16:701-4 |
How to cite this URL:
Arora V, Nijjar I S, Sandhu P S, Singh J, Abrol R, Roopa. Hypertrophic olivary degeneration following trauma - a case report. Indian J Radiol Imaging [serial online] 2006 [cited 2020 Dec 3];16:701-4. Available from: https://www.ijri.org/text.asp?2006/16/4/701/32327 |
| Introduction | |  |
Hypertrophic olivary degeneration occurs in lesions involving the dentatorubro-olivary system [1]. A unique finding in this form of transneuronal degeneration is enlargement rather than atrophy of the affected structure. Typically, haemorrhages following a hypertensive crisis are the cause of this pathological entity [2],[3],[4]. We describe a patient in whom haemorrhagic contusion in the region of superior cerebellar peduncle and midbrain resulted in hypertrophic olivary degeneration.
| Case report | | |
A thirty-five years old man was unconscious after severe head injury. His MRI revealed evidence of diffuse axonal injury with a large haemorrhagic contusion at the ponto-mesencephalic junction and left superior cerebellar peduncle, appearing hyperintense on T2 W and FLAIR images with areas of hypointensity in it [Figure - 1]. There was also evidence of subarachnoid haemorrhage seen as hyperintensity in the sulcal spaces on FLAIR images. The patient was kept on conservative management.
After five months following trauma he developed tremor of the left upper limb. The tremor was present at rest and further amplified during intentional movements. It disappeared during sleep. There was no evidence of palatal tremor.
He was referred for a repeat MRI that showed gliottic changes and haemorrhagic residue in the left superior cerebellar peduncle-sequelae of old head injury [Figure - 2]. In addition, there was hyperintense signal in the right inferior Olivary nucleus on T2 W and FLAIR images with enlargement of the nucleus [Figure - 3]. The lesion was isointense on T1 W images (not shown). Diffusion weighted images did not reveal the lesion [Figure - 4].
| Discussion | | |
Hypertrophic olivary degeneration is a form of transsynaptic degeneration [3]. It represents the end result of a lesion that damages the neural connections between the dentate nucleus of the cerebellum, the red nucleus, and the inferior olivary nucleus: the dentatorubral - olivary pathway [5],[6]. It is considered a unique type of degeneration because it is associated with enlargement, rather than atrophy, of the affected structure - the inferior olivary neurons [7].
The dentatorubral - olivary connections were described by Guillain and Mollaret [6] in 1931, and this pathway is thus referred to as the Guillain Mollaret triangle. The Guillain Mollaret triangle is a triangular circuit connecting the dentate nucleus of the cerebellum of one side with the red nucleus and the inferior olivary nucleus of the other side [Figure 5]. The dentate nucleus and the contralateral red nucleus are connected by the superior cerebellar peduncle (dentatorubral tract), with fibres crossing in the decussation of the peduncle at the lower midbrain. This tract is part of the reflex arc that controls fine voluntary movements. The red nucleus and the ipsilateral inferior olivary nucleus are connected by the central tegmental tract.
[Figure 5] - Guillain Mollaret triangle.
There are three possibilities of presentation of hypertrophic olivary degeneration, ipsilateral and contralateral to the lesion, but rarely it can involve the olives bilaterally [8]. If the lesion is located in the tegmentum of the brainstem and involves the central tegmental tract, the degeneration occurs in the ipsilateral olive. If the lesion is located in a cerebellar hemisphere and involves the dentate nucleus, the olivary degeneration will be contralateral, due to the dicussation of the dentato-rubral fibres. If the lesion involves the superior cerebellar peduncle, the degeneration will occur contra-laterally, as was seen in our case. Bilateral hypertrophic ovary degeneration has also been reported [9],[10]. A paramedian lesion, if located near the peduncle, may result in bilateral hypertrophic olivary degeneration, if there is involvement of both the dentato-rubral fibres, and the central tegmental tract.
Focal brainstem insults that may lead to pathway interruption include ischaemic infarction, demyelination and haemorrhage. The latter often related to hypertensive disease or diffuse axonal injury following severe head trauma [2],[11]. Olivary hypertrophy is not seen immediately after the brainstem insult but typically appears in a delayed fashion, usually within 4-6 months. The pathologic process persists and is frequently visible after 10 months. Although olivary hypertrophy typically resolves in 10-16 months, olivary hyperintensity on T2 W images may persist for years after resolution of the hypertrophy [12],[13]. Clinical symptoms such as abnormal movement rarely improve. Olivary enlargement corresponds pathologically to an unusual vacuolar degeneration of cytoplasm that results in enlargement related in part to an increased number of astrocytes [13].
Clinical findings associated with olivary hypertrophy include the dentatorubral or Holmes' tremor, palatal myoclonus and occular myoclonus. Holmes' tremor includes an unusual combination of 2-5 Hz rest, postural and kinetic tremor of an upper extremity. It usually begins weeks to months after brainstem stroke, so compensatory or secondary changes in nervous system function must contribute to tremor genesis. Palatal tremor is a rhythmic involuntary movement that appears mainly in the soft palate. There have been few cases describing the development of palatal tremor some time after the appearance of Holmes' tremor [9],[14]. At the time of repeat MRI, our patient had Holmes' tremor but no evidence of palatal tremor.
The differential diagnosis of signal hyperintensity in the antero-lateral part of the medulla includes infarction, demyelination, tumour and inflammatory processes (tuberculosis, sarcoidosis or encephalitis). The lack of contrast enhancement, however is against many tumours or infectious entities, while the additional enlargement of the olivary nucleus is against chronic stages of infarction or multiple sclerosis [15]. Diffusion weighted study helps to rule out acute infarction. The most important clue to the diagnosis is the association of a remote lesion within the Guillain - Mollaret triangle [3].
We conclude that hypertrophic olivary degeneration is caused by various etiologies, and may present various aspects. When inferior olivary lesions, especially, hyperintensity on T2 W images and / or swelling, are recognised, it is important to investigate if the Guillain Mollaret triangle is impaired. Familiarity with the characteristic MR features and exact knowledge of this degeneration would avoid misinterpretation of hypertrophic olivary degeneration as other independent lesions such as infarction, tumour or demyelinating disease.
| References | | |
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Correspondence Address:
V Arora
Nijjar Scan and Diagnostic Centre, 51/1, Court Road, Amritsar-143001
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0971-3026.32327
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4] |
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