Heimann- Bielschowsky Phenomenon

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Heimann-Bielschowsky Phenomenon

For a video description of this entity, you can access the American Academy Neurology [1]. [1]

https://cdn-links.lww.com/permalink/wnl/a/wnl_2017_12_20_nguyen_1_sdc1.mp4

Disease Entity

Disease

The Heimann- Bielschowsky phenomenon (HBP) is a monocular vertical nystagmus characterized by slow, coarse, pendular, variable amplitude movement found in an eye with profound visual loss[2] .The new onset vertical pendular nystagmus of HBP could be mistaken for efferent (rather than afferent disease) and might raise suspicion for a posterior fossa or brainstem lesion.[3] [4]

Etiology

The mechanism for HBP is currently unknown. Leigh et al. propose the pathogenesis could be related to disturbances in the fusional vergence mechanism or the monocular visual stabilization system. [5] The cause of the HBP can be secondary to a congenital (e.g. anterior segment dysgenesia, optic nerve hypoplasia, infantile cataract, microphthalmia, or sclerocornea) or an acquired cause (e.g. amblyopia, absolute glaucoma,optic neuropathy or a traumatic cataract).[2]

Diagnosis

History

The HBP was first described by Ernst Heimann in 1902. He described this nystagmus occurring in amblyopia secondary to strabismus and other neurological diseases such as multiple sclerosis, neurosyphilis, and epilepsy. HBP was then distinguished as different from Dissociated Vertical Deviation (DVD) in 1931 by Alfred Bielschowsky due to DVD having better best corrected visual acuity and a slow downward drift with no nystagmoid or rhythmic movements. [6][2]

Presentation

The HBP presents as a strictly monocular, coarse, slow, and pendular vertical oscillation occurring mainly in eyes with profound visual loss. The oscillations seen may be easily missed because of how slow and low frequency they present.

Visual Loss: The HBP predominantly occurs in eyes with a large amount of visual loss. When looking at 3 studies totaling 28 patients, 24 patients had visual acuity of 20/200 or worse. [2][3]

Strabismus: There is a strong association between the HBP and strabismus as it is present in greater than 70% of patients with the HBP. [2][3]

Wave form characteristics: The oscillations in the HBP are slow and pendular.

  • Frequency: 1-5 Hz
  • Amplitude: Frequently 2-5 degrees but may be as high as 20-30 degrees (40-60 prism diopters)

Diseases That Can Present Similar to the HBP:

  • Spasmus nutans: A pediatric disorder consisting of a triad of ocular oscillations, compensatory head nodding, and head turning (torticollis). The nystagmus seen is small frequency. Unlike the HBP, it has a high amplitude and is usually horizontal, although it can also be vertical, rotary, and vary with different gaze positions. The nystagmus can be unilateral or bilateral and is often intermittent. Visual acuity is typically normal. [7]
  • Ocular Neuromyotonia: A rare ocular motor disorder characterized by intermittent, tonic spasms of one or more of the extraocular muscles, resulting in strabismus and paroxysmal diplopia occurring spontaneously or after eccentric gaze lasting a few seconds up to several minutes. Most cases are unilateral and occur in adults with normal visual acuity. [8]
  • Acquired Pendular Nystagmus: Most commonly due to multiple sclerosis but may be a sign of a stroke or lesion in the cerebellum or brainstem. Often entails slow-phase eye movements in horizontal, vertical, and torsional planes with resultant elliptical or circular nystagmus. The movements may be monocular, or if bilateral, conjugate or disconjugate, and may also be dissociated. Cerebellar signs or brainstem signs (e.g. internuclear ophthalmoplegia) are often also present and help to differentiate from the HBP. [9]
  • Oculopalatal myoclonus: Pendular nystagmus with concomitant palatal myoclonus (oscillations of the palate) usually developing months to years after a brainstem infarction. The eye movements are continuous with both torsional and vertical components with a frequency of 1-3 Hz. Additional muscles (pharynx, face, vocal cords, respiratory muscles, and even trunk and extremities) may be involved in this involuntary rhythmic movement that persists during sleep. [10]
  • See-Saw nystagmus: A subtype of torsional nystagmus, is descriptively named for the pendular, vertical disconjugate movement of eyes in which one elevates/intorts while the fellow eye depresses/extorts. The frequency is usually 2-5 Hz. [10] [11]

Symptoms

Most patients are aware that there is an intermittent oscillation but symptoms such as diplopia and oscillopsia are infrequent due to the profound visual loss usually seen in the affected eye. Although no other study reports symptoms of diplopia or oscillopsia, Davey et. al. reported 3 cases, each of which had visual acuity better than 20/120. [3]

Clinical diagnosis

The HBP is a clinical diagnosis in which slow, pendular, vertical oscillations are noted in a low vision eye. It is recommended that the affected eye be observed for 1 minute. [2]

Differential diagnosis

  • Spasmus nutans
  • Acquired Pendular nystagmus
  • Ocular Neuromyotonia
  • Oculopalatal myoclonus
  • See-saw nystagmus
  • Chiasmal tumor [3]

Management

Medical therapy

There is no medical therapy shown to be effective in improving the nystagmus of the HBP.

Surgery

There have been mixed results when strabismus surgery is performed to try and reduce oscillations. Smith et. al. noted dampened oscillations after a Faden procedure and Sebastian et. al. noted 4 cases with large amplitude vertical oscillations that had decreased oscillations following superior rectus muscle recession with or without inferior rectus muscle recession. [4][12] Davey et. al. observed an improvement in alignment and cosmesis in 7 patients undergoing strabismus surgery, however none had reductions in oscillations. [3]

Vision improving surgery has not consistently shown to improve the nystagmus.

References

  1. Teaching Video NeuroImages: Heimann-Bielschowsky phenomenon A harmless monocular nystagmus. Audrey Nguyen, François-Xavier Borruat Neurology Feb 2018, 90 (8) e731; DOI: 10.1212/WNL.0000000000005004
  2. 2.0 2.1 2.2 2.3 2.4 2.5 Surachatkumtonekul, T., & Pamonvaechavan, P. (2009). Unilateral vertical nystagmus: the Heimann-Bielschowsky phenomenon. Journal of the Medical Association of Thailand = Chotmaihet thangphaet, 92 3, 373-6 .
  3. 3.0 3.1 3.2 3.3 3.4 3.5 Davey, K. , Kowal, L. , Friling, R. , Georgievski, Z. and Sandbach, J. (1998), The Heimann‐Bielscholwsky phenomenon: Dissociated vertical nystagmus. Australian and New Zealand Journal of Ophthalmology, 26: 237-240. doi:10.1111/j.1442-9071.1998.tb01318.
  4. 4.0 4.1 Smith JL, Flynn JT, and Spiro HJ; Monocular vertical oscillations of amblyopia: The Heimann-Bielschowsky phenomenon. J Clin Neuro Ophthalmol. 2:85, 1982.
  5. Leigh, R.J., Thurston, S.E., Tomsak, R.L., Grossman, G., & Lanska, D. (1989). Effect of monocular visual loss upon stability of gaze. Investigative ophthalmology & visual science, 30 2, 288-92 .
  6. Rajavi Z, Feizi M, Haftabadi N, Sheibani K. Hypotropic dissociated vertical deviation; a case report. J Ophthalmic Vis Res. 2013;8(3):271–273.
  7. Weissman BM, Dell'Osso LF, Abel LA, Leigh RJ. Spasmus Nutans: A Quantitative Prospective Study. Arch Ophthalmol. 1987;105(4):525–528. doi:10.1001/archopht.1987.01060040095041
  8. Stockman AC, Dieltiëns M, Janssens H, Van Lammeren M, Beelen L, Van Bellinghen V, Cassiman C. Ocular Neuromyotonia: Case Reports and Literature Review. Strabismus. 2018 Sep;26(3):133-141.
  9. Acquired Pendular Nystagmus. In: Basic and clinical science course (BCSC) Section 5: Neuro-Ophthalmology. Academy of Ophthalmology; 2015:237.
  10. 10.0 10.1 Fong AMF. A Practical Approach to Nystagmus and Saccadic Oscillations. Focal Points by American Academy of Ophthalmology. 2014.
  11. Druckman R, Ellis P, Kleinfield J, Waldman M. Seesaw Nystagmus. Arch Ophthalmol. 1966;76(5):668–675. doi:10.1001/archopht.1966.03850010670008
  12. Sebastian, R.T., Marsh, I.B.; Heimann–Bielscholwsky Phenomenon: Vertical Recti Recession Gives Good Cosmetic Results in Patients With Large Amplitude Vertical Nystagmoid Movements . Invest. Ophthalmol. Vis. Sci. 2005;46(13):2942.