Table 1. Summary of Clinical Information on Patients Studied and pooled Normal Subjects (first row).
Disturbances of eye movements are listed in order of prominence on examination. aVOR: angular vestibulo-ocular reflex. DBN: downbeat nystagmus. EA2: episodic ataxia type 2. GEN: gaze-evoked nystagmus. MRI: magnetic resonance imaging of head; MSO: macrosaccadic oscillations. SCA3-MJD: spinocerebellar ataxia type 3 – Machado-Joseph disease. SCA6: spinocerebellar ataxia type 6. SCASI: spinocerebellar ataxia with saccadic intrusions. UBN: upbeat nystagmus.
| Subject/Age/ Sex |
Diagnosis/ Duration |
Neurological & Neuro- imaging Findings |
Disturbance of Eye Movements |
9-Hole Peg Test |
Medicines |
|---|---|---|---|---|---|
| NS/52*/2F & 8M |
Controls | Normal | Normal | R: 18 s** L: 21 s** |
NA |
| CP1/58/M | SCASI/30 | Limb, trunk, & gait ataxia, dysarthria, neuropathy. MRI: diffuse cerebellar atrophy |
Saccadic hypermetria, mainly horizontal; MSO |
R: 44 s L: 44 s |
Memantine Valproate Pramipexole Gabapenti |
| CP2/62/M | SCASI/32 | Limb, trunk, & gait ataxia, dysarthria, neuropathy. MRI: diffuse cerebellar atrophy |
Saccadic hypermetria, mainly horizontal; MSO |
R: 45 s L: 48 s |
Memantine Valproate Clonazepam Fluoxetine |
| CP3/56/M | SCA3-MJD/35 | Limb & gait ataxia, dysarthria. MRI: cerebellar atrophy, most marked in vermis |
Saccades dysmetric & mildly slowed, vertical > horizontal; GEN; aVOR impaired |
R: 54 s L: 52 s |
Metoprolol |
| CP4/39/M | EA2/30 | Limb & gait ataxia during attacks, dysarthria. MRI: diffuse cerebellar atrophy |
GEN with downbeating component; saccadic dysmetria; impaired pursuit |
R: 25 s L: 20 s |
Acetazolamide Quetiapine Lamotrigine |
| CP5/59/F | SCA6/6 | Gait ataxia, photophobia. MRI: diffuse cerebellar atrophy |
GEN with downbeating component; impaired vertical aVOR and pursuit; saccadic dysmetria |
R: 25 s L: 22 s |
Acetazolamide Bupropion |
| CP6/78/M | Right vestibular schwannoma, 3 cm × 6 cm × 3 cm /1.5 resected 21 months previously |
Right-sided deafness, dysarthria, & limb ataxia; gait ataxia, vertical diplopia. MRI: Residual right-sided tumor with compression of middle cerebellar peduncle & hemisphere |
GEN on right gaze with upbeat component; impaired smooth pursuit and aVOR; left hypertropia; saccadic dysmetria; |
R: 34 s L: 27 s |
Prazosin |
| CP7/85/M | Sporadic cerebellar degeneration/7 |
lower limb & gait ataxia, mild diabetic neuropathy. MRI: normal for age |
DBN increasing on lateral gaze, with GEN; skew deviation with left hypertropia; mild saccadic hypometria; impaired vertical pursuit |
R: 29 s L: 27 s |
Metoprolol |
| CP8/67/M | Sporadic cerebellar degeneration/7 |
Gait ataxia, dysarthria, mild memory deficit with emotional swings. MRI: normal for age |
DBN increasing on lateral and down gaze and at near- viewing; abnormal “cross- axis” upward eye movements |
R: 31 s L: 26 s |
Buspirone |
| CP9/33/F | Wernicke- Korsakoff syndrome/1.5 |
Limb & gait dysmetria, mild short-term memory deficits. MRI: normal |
UBN switching to DBN at near and in right lateral gaze; aVOR impaired |
R: 23 s L: 18 s |
Lorazepam Gabapentin Topiramate Sertraline Clonazepam |
| CP10/60/M | Idiopathic downbeat nystagmus/2 |
Vertical oscillopsia, mild gait imbalance. MRI: normal |
Vertical diplopia due to DBN (worse during right gaze) |
R:19 s L: 19 s |
none |
Subject ages and disease duration are in years.
*
median age of normal subjects.
**
median of 9-Hole Peg Test results for normal subjects.