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Clinical assessment of the sensory
ataxias; diagnostic algorithm with
illustrative cases
S K Chhetri,1,2 D Gow,3 S Shaunak,1 A Varma3

1
Neurology Department, Royal ABSTRACT
Preston Hospital, Lancashire Box 1 Proposed criteria for diagnos-
Ataxia is a common neurological syndrome
Teaching Hospitals NHS
Foundation Trust, Preston, UK resulting from cerebellar, vestibular or sensory ing clinically probable sensory ataxia
2
The University of Manchester, disorders. The recognition and characterisation of
Manchester Academic Health sensory ataxia remains a challenge. Cerebellar 1. Ataxia confirmed by clinical examin-
Science Centre, Manchester, UK
3 ataxia is the more common and easier to ation: finger–nose incoordination and/
Neurology Department, Greater
Manchester Neurosciences identify; sensory ataxia is often mistaken for or heel–toe ataxia and/or broad-based
Centre, University of Manchester, cerebellar ataxia, leading to diagnostic errors and ataxic gait
Salford Royal Hospital, delays. A coherent aetiological work-up is only
Manchester, UK
2. Two or more of:
possible if clinicians initially recognise sensory A. Romberg’s sign or ataxia signifi-
Correspondence to ataxia. We discuss ways to separate sensory from cantly worse with eyes closed, or
Dr Anoop Varma, Consultant cerebellar ataxia, the causes of sensory ataxia history of ‘wash basin sign’3
Neurologist, Neurology and the clinico-neurophysiological syndromes
Department, Greater Manchester B. Pseudoathetosis and/or impaired
Neurosciences Centre, University causing the sensory ataxia syndromes. We joint position and/or vibration
of Manchester, Salford Royal summarise a logical tiered approach as a sense/s.
Hospital, Stott Lane, Salford, diagnostic algorithm. C. Absence of nystagmus and/or cere-
M6 8HD, UK;
dranoopvarma@gmail.com bellar dysarthria.
INTRODUCTION
Published Online First
25 February 2014 Ataxia (Greek ‘taxis’ = order1) refers to
incoordination without significant weak- Finding Romberg’s sign, pseudoathetosis,
ness. It may result from cerebellar, ves- impaired joint position or vibration sense
tibular or sensory ( proprioceptive) argues strongly for sensory ataxia.
dysfunction. The presenting features Table 1 lists the characteristics which
include impaired balance and/or direc- help to distinguish pure sensory from
tional limb movements and/or dysarthria, cerebellar ataxia.1 2 The degree of
depending upon the underlying locus of impaired joint position or vibration sense
pathology. There is often clinical confu- loss may vary; patients need a detailed
sion between sensory ataxia and cerebel- and experienced sensory evaluation to
lar ataxia1 2; patients with chronic record large fibre sensory deficit.
sensory ataxia may carry the wrong diag- Additionally, distal deep tendon reflexes
nostic label for decades. There are many may be retained in posterior column or
possible causes which need a careful non-length-dependent dorsal root gan-
history, examination and appropriately glion pathologies. Varying degrees of wor-
targeted investigations to delineate. We sened limb or gait ataxia on eye closure is
present a clinical algorithm to facilitate not totally specific for sensory ataxia.
this. Similarly, Romberg’s sign may be difficult
to perform or interpret in a grossly ataxic
CLINICAL APPROACH patient with a cerebellar disorder. A sig-
The first step is to distinguish cerebellar, nificant worsening of unsteadiness with
sensory and vestibular ataxia. Box 1 visual deprivation (including incoordin-
shows suggested clinical criteria for diag- ation of limbs or of station ie, Romberg’s
To cite: Chhetri SK, Gow D, nosing sensory ataxia,1 2 worth consider- sign) requires experienced and focused
Shaunak S, et al. Pract Neurol ing in any patient with ataxia who has no clinical judgement. We recommend
2014;14:242–251. nystagmus or cerebellar dysarthria. caution in presuming a cerebellar basis for

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Table 1 Clinical differences between sensory and cerebellar ataxia
Sensory ataxia Cerebellar ataxia
Nystagmus Absent* Present
Dysarthria Absent* Scanning/staccato speech
Eye movements Sometimes abnormal (eg, CANOMAD) Normal/slow
Finger–nose ataxia Present (significantly worse with eye closure) Present
Heel–shin ataxia Present (significantly worse with eye closure) Present
Deep tendon reflexes Absent/present Normal/pendular
Joint position sense Impaired Normal
Pseudoathetosis Usually present Absent
Exteroceptive sensation Reduced Normal
Romberg’s sign Positive Classically negative
Gait High stepping (stamping)/unsteady Broad-based, staggering
*Except sensory ataxia-plus syndromes.
CANOMAD—chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies.

ataxia in the absence of nystagmus and/or dysarthria. Cerebellar ataxia is more common and easier to
Additionally, some ‘sensory ataxia-plus’ syndromes may identify with certainty than sensory ataxia; thus,
have a component of cerebellar ataxia. Distinguishing sensory ataxia is often mistaken for cerebellar ataxia.
sensory from cerebellar ataxia is often more difficult To blur the differences further, a midline anterior
than table 1 might imply (see case 2, box 3). cerebellar syndrome (mostly from alcoholic cerebellar
degeneration) may present only with gait ataxia.4
Thus, the absence of dysarthria and/or nystagmus
need not indicate an underlying sensory component
Box 2 Case 1 to the ataxic syndrome. Moreover, sensory ataxia may
coexist with cerebellar ataxia (case 2, box 3) and dys-
arthria, as in certain mitochondrial cytopathies. The
A 58-year-old man with type 2 diabetes mellitus gave a
dominant feature (sensory or cerebellar) provides the
3-year history of paraesthesia in his legs, and a 4-month
clue to diagnosis. ‘Sensory ataxia-plus’ implies added
history of similar sensations in his hands. He reported
neurological multisystem features, so could include
gradually progressive unsteadiness with occasional falls.
nystagmus, cerebellar dysarthria and subordinate cere-
His local doctors had diagnosed diabetic polyneuropathy.
bellar ataxia. Incorrectly assigning the dominant syn-
He had stopped smoking 11 years before and did not
drome (cerebellar, instead of sensory ataxia) could
drink alcohol to excess. There was no weight loss, poor
lead to misdiagnosis, for example, with spinocerebel-
appetite, dry eyes/mouth, bulbar or sphincter distur-
lar ataxia (SCA)-associated with neuropathy5 (see
bances. He was taking metformin, ramipril, simvastatin
Case 2, box 3) where SCA was wrongly diagnosed by
and aspirin. On examination, his tone and power were
successive experienced neurologists over two
normal and there was no tremor or pseudoathetosis. All
decades). Boxes 2–5 show other examples of failures
reflexes were absent except the triceps jerk. He walked
to detect sensory ataxia initially.
with a high-stepping gait and Romberg’s sign was posi-
tive. Vibration sense was absent below the knees, and
DIFFERENTIAL DIAGNOSIS
pinprick was impaired to mid-shin bilaterally. Joint-
Figure 1 emphasises the classification of sensory
position sense was impaired at the toes. Nerve conduc-
ataxia according to the site of lesion. The pathology is
tion showed a sensory and motor demyelinating neur-
most likely in the proprioceptive system along its
opathy without conduction block. Routine blood tests
pathway from the peripheral sensory nerves, dorsal
and autoimmune studies were normal. However, serum
root ganglia, sensory nerve roots, dorsal column of
electrophoresis showed monoclonal kappa bands with
spinal cord, medial lemnisci of the brainstem and par-
raised IgM of 4.8 g/L (0.4–2.3). CSF protein was raised at
ietal cortices.1 The dorsal root ganglia are often the
0.84 g/L (0.15–0.45) with normal cell count and glucose.
major site of pathology, but dorsal column or large
Oligoclonal bands were negative. Urine electrophoresis
diameter sensory fibre involvement could present
was normal. Anti myelin-associated glycoprotein antibody
similarly. Table 2 shows a temporal (acute: days to
was raised at >70 000 Buhlmann titre unit (0–1000).
weeks; subacute: weeks to months; or chronic:
Bone marrow biopsy suggested a low-grade B cell lym-
months to years) and aetiological subclassification to
phoproliferative disorder. The time from onset of symp-
help to delineate these disorders further.6 It is also
toms to diagnosis was 3 years. We felt his sensory
important to explore the family history carefully, as
ataxia was secondary to an antimyelin-associated
several inherited causes can manifest with sensory
glycoprotein-associated demyelinating neuropathy.
ataxia (box 6).

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Box 3 Case 2 Box 4 Case 3

A 70-year-old man first presented in the early 1990s A 56-year-old right-handed man gave a 3-year history of
with gradually progressive unsteadiness leading to recur- a burning sensation over the left flank. There was no pre-
rent falls. A few years later, he developed ptosis and dys- ceding rash; he had been investigated for the possibility
arthria and was diagnosed as spinocerebellar ataxia. of varicella. There was also an 18-month history of erect-
There was no history of alcohol excess. He was reas- ile dysfunction, dry mouth, and a gritty sensation in both
sessed in 2007. On examination, he had a partial right eyes, and a 5-month history of unsteady gait.
ptosis with equal and reactive pupils, broken pursuit Examination was normal except for bilaterally suppressed
movements and an infranuclear vertical gaze palsy. He supinator and biceps jerks. He felt unsteady on his
was dysarthric, but also had marked pseudoathetosis and feet although his gait was not particularly broad-based.
a profoundly ataxic gait. Romberg’s sign was present. Romberg’s sign was negative. Routine blood tests, sero-
Limb strength was normal but knee and ankle jerks were logic workup, nerve conduction studies, CSF analysis
absent. Sensory examination showed a glove and stock- were normal. CT scan of the thorax and abdomen were
ing predominantly sensory polyneuropathy. Routine blood normal. A year later, he noticed constant numbness over
tests, serologic workup and CSF analysis were normal. the right thumb, electric shock-like sensations over the
Nerve conduction studies showed a sensory neuronopa- radial aspect of right forearm, and a burning sensation
thy. Muscle biopsy showed a mitochondrial DNA over the lateral aspect of right leg. Examination at this
rearrangement consistent with SANDO. POLG1 mutation stage showed pseudoathetosis in the upper limbs and
analysis was positive. The time from onset to diagnosis reduced pinprick sensation in the right median, lateral
was almost 20 years. antebrachial, both sural and superficial peroneal nerve
SANDO, Sensory ataxic neuropathy, dysarthria and territories. Vibration was reduced at the great toes.
ophthalmoparesis. Repeat nerve conduction showed normal motor studies
with patchy asymmetric non-length-dependent dimin-
ution of SNAPs. Schirmer’s test was negative.
Paraneoplastic antibody screening was positive for
ROLE OF NEUROPHYSIOLOGY anti-Hu and anti-Ri, prompting a repeat CT scan of the
Neurophysiology is important in diagnosing sensory chest. This demonstrated an irregular 2.4×2.1 cm tumour
ataxias by helping to localise the lesion and to suggest in the left upper lobe, confirmed as large cell neuroendo-
the pathological process, that is, axonal versus demye- crine tumour on histology. We diagnosed paraneoplastic
lination.37 In dorsal root ganglionopathy, sensory con- dorsal root ganglionopathy. The time from onset of
duction studies may show widespread loss or reduced symptoms to diagnosis was 4 years.
amplitude of sensory nerve action potentials
(SNAP).28 38 In some patients, the SNAPs may be
more affected in the upper than the lower limbs. This provide appropriate clinical information, raising spe-
pattern of non-length-dependent axonal degeneration cific questions. Last, just like the clinical examination,
helps to distinguish dorsal root ganglionopathy from neurophysiology can and should be repeated if the
the more common dying-back axonal neuropathies, clinical syndrome progresses or alters.
for example, diabetic neuropathy (where there is a
distal-to-proximal pattern of sensory loss). Complete Sensory ataxia secondary to peripheral neuropathies
absence of SNAPs in someone with no positive Peripheral neuropathies may present with either
sensory symptoms strongly suggests a genetic cause. chronic or, less commonly, acute sensory ataxia.
Motor nerve conduction studies in dorsal root gang-
lionopathy are often normal, but there may be sub- Acute/subacute
clinical reductions in conduction velocity and Miller Fisher syndrome is an acute form of sensory
compound muscle action potential (CMAP), particu- ataxia. There may be either antecedent infection
larly in paraneoplastic forms.28 38 Finding increased (mainly Campylobacter jejuni and Haemophilus
distal motor latencies, slowed conduction velocities, influenzae) and/or underlying autoimmune or neo-
prolonged F wave latency, motor conduction block plastic disorder.7 The syndrome is characterised by
and/or temporal dispersion suggests a demyelinating ophthalmoplegia, ataxia and areflexia. Although limb
neuropathy.39 Sensory root involvement, as in chronic weakness and superficial sensory loss are not part of
immune sensory polyradiculopathy, gives normal the classical triad, about one-third have these fea-
nerve conduction, but often abnormal tures.7 The serum anti-GQ1b IgG antibody titre is ele-
somatosensory-evoked potentials.31 Note that an elec- vated in over 80% of cases.7
trodiagnostic examination is a targeted extension of The sensory variant of Guillain–Barré syndrome,
the neurological evaluation, and one must work in which solely involves the large sensory fibres, is rare
close liaison with the neurophysiology team and but can also present as acute sensory ataxia.8 The

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sensory ataxic neuropathies, as intravenous immuno-
Box 5 Case 4 globulin treatment is often effective.41

An 80-year-old woman with no history of smoking pre-

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
sented with a 3-year history of gradually progressive
CIDP typically presents with monophasic, relapsing
unsteadiness of gait and an 18-month history of numb
or progressive symmetrical sensory and motor neur-
feet. There was no weakness, but she did have dry eyes
opathy, evolving over 8 weeks.39 The classical mani-
and mouth. She had a history of stable myelodysplasia
festations include paraesthesia, proximal weakness
and hypothyroidism. Her medications included hydro-
without wasting, areflexia and loss of vibration and/or
xyurea, thyroxine and pyridoxine. On examination, there
joint position sense. Nerve conduction studies may
was mild upper limb pseudoathetosis. Tone and power
show either conduction slowed to the demyelinating
were normal. The ankle jerks were absent with flexor
range or partial conduction block.39 The uncommon
plantars. Her gait was ataxic and Romberg’s sign was
sensory ataxic form manifests as prominent numbness
present. Vibration was absent below the knees, and pin-
in the extremities, ataxia, areflexia and impaired
prick was impaired to the mid-calves. Joint position
vibration and/or joint position sense.12 Weakness may
sense was reduced to the ankles bilaterally. Her blood
be mild or absent, by contrast with classical CIDP.
tests including glucose, folate and B12 were normal
Nerve conduction shows motor abnormalities typical
apart from haemoglobin of 103 g/L, and mean corpuscu-
of CIDP, and nerve biopsy shows demyelination and
lar volume of 103 fL (76–96) related to myelodysplasia.
remyelination.12 It often responds to corticosteroids
Immunological studies, inflammatory markers, syphilis
and/or intravenous immunoglobulins, and its manage-
serology, tumour markers, paraneoplastic antibodies and
ment is along lines similar to typical CIDP.
serum electrophoresis were normal or negative. Chest
X-ray was normal. Her serum pyridoxine level was very
high at 132 041 nmol/L (8–135 nmol/L). MR scan of cer- Paraproteinaemic neuropathy
vical spine showed spondylotic changes with no neural A range of paraproteinaemic neuropathies can cause
compression. CSF analysis was normal. Nerve conduction sensory ataxia.13 42 Antimyelin-associated glycoprotein
studies showed a large fibre, length-dependent general- neuropathy is a distinct syndrome of late onset, a
ised sensori-motor neuropathy of axonal type. She slowly progressive, demyelinating neuropathy charac-
reported no significant improvement after stopping pyri- terised by distal and symmetrical, mainly sensory neur-
doxine. A thorough screen for all causes of sensory opathy. It results from monoclonal IgM antibodies
ataxia led to a lip biopsy; this suggested Sjögren’s syn- against myelin-associated glycoprotein.13 42 There is
drome. The time from onset of symptoms to diagnosis predominant impairment of joint position and vibra-
was 3 years. tion sense. Patients may have tremor but little weak-
ness. Clinicians should consider testing for
antimyelin-associated glycoprotein antibody in all
clinical findings include sensory ataxia, impaired joint patients with IgM paraproteinaemic neuropathies, as
position and vibration sense and areflexia, but usually nearly half of them have high titres.42 43 Nerve con-
normal strength. Serum antibodies to GD1b and duction studies usually show symmetrical and predom-
GQ1b gangliosides may be positive.8 Nerve conduc- inantly distal demyelination with disproportionately
tion in both conditions may show demyelinating poly- prolonged distal motor latencies.42
radiculoneuropathy, although in Miller Fisher Waldenström’s macroglobulinaemia, a lymphoplas-
syndrome they are often either normal or show small macytoid malignancy producing IgM paraprotein, can
SNAPs.7 8 Cerebrospinal fluid commonly has a present with sensory ataxia if the IgM paraprotein
normal cell count but raised protein. shows antimyelin-associated glycoprotein activity.44
Semisynthetic penicillins may cause acute sensory Note that IgM paraproteinaemic neuropathies may
ataxia.9 cause sensory ataxia in the absence of antimyelin-
Lyme disease10 and neurosarcoidosis11 may uncom- associated glycoprotein antibodies.13 42
monly present with subacute sensory ataxia. CANOMAD (chronic ataxic neuropathy, ophthalmo-
plegia, monoclonal IgM protein, cold agglutinins and
Chronic disialosyl antibodies) is another antibody-mediated
Chronic sensory ataxia can result from chronic paraproteinaemic neuropathy.14 It is associated with
inflammatory demyelinating polyradiculoneuropathy anti-GQ1b and antidisialosyl antibodies. Note that a
(CIDP),12 paraproteinaemia,13 14 diabetic neur- history of diabetes mellitus and alcohol excess (both
opathy,15 coeliac disease,16 vitamin E deficiency17 and common causes of neuropathy) do not preclude the
mitochondrial dysfunction.40 Chronic sensory ataxia possibility of coexisting pathology (Case 1, box 2).
can uncommonly be associated with anti-GD1b IgM Serum immunoelectrophoresis and immunofixation
antibody; it is worthwhile measuring antiganglioside are, therefore, important investigations in the diagnos-
antibodies in all patients with otherwise idiopathic tic workup of ataxic neuropathy.

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Figure 1 Anatomical localisation of sensory ataxia. CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; CISP, chronic
immune sensory polyradiculopathy; GBS—Guillain–Barré syndrome; HCV, hepatitis C virus; HIV, human immunodeficiency virus;
HTLV1, human T cell lymphotrophic virus type 1; MFS, Miller Fisher syndrome.

Diabetic neuropathy joint position and pressure sensations and loss of

Diabetic neuropathy commonly presents with distal ankle reflex. In advanced stage, there may be sensory
symmetrical polyneuropathy.15 The impairment may ataxia.15
be sensory or motor and is length-dependent. Small
fibres are usually affected first, giving pain, burning, Coeliac ataxia
impaired spinothalamic sensations and autonomic dys- Coeliac disease is a chronic inflammatory enteropathy
function. Large fibre sensation is affected later, when associated with sensitivity to ingested gluten.
patients may develop paraesthesia and gait imbalance. Neurological complications occur in 6–10%; ataxia and
On examination, there is often impaired vibration, peripheral neuropathy are the common presentations.16

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Table 2 Acquired causes of sensory ataxia
Site Onset Aetiology Ancillary investigations
Peripheral nerve/nerve root Acute Miller Fisher syndrome7 Anti-GQ1b antibodies, CSF, NCS
Sensory variant of GBS8 Anti-ganglioside antibodies, CSF, NCS
Semisynthetic penicillins9 History of exposure
Subacute Lyme disease 10 Lyme serology
Neurosarcoidosis 11 CSF, NCS, ACE, chest X ray, HPE
12
Chronic CIDP NCS, CSF
Paraproteinaemia13 14 Electrophoresis, anti-MAG
Diabetes mellitus15 Plasma glucose.
16
Coeliac disease Anti-tTG antibodies
Vitamin E deficiency17 Serum vitamin E level
Dorsal root ganglion Subacute HIV18 HTLV-1,19 HCV20 Serology
Small cell lung cancer21 Anti-Hu, CRMP-5, relevant imaging
Pyridoxine intoxication 22 History of exposure; pyridoxine levels
Chemotherapeutic agents23 History of exposure
24
Thalidomide History of exposure
Organophosphate exposure25 History of exposure
Colonic carcinoma26 Relevant imaging
Neuroendocrine tumour27 Relevant imaging
Breast and ovarian cancer28 Relevant imaging
29
Chronic Sjögren’s syndrome ENA (Anti-SSA/SSB) abs, lip biopsy
Chronic active hepatitis30 RF, ANA, ASMA, hepatitis serology
Spinal root Chronic CISP31 NCS, SSEP, CSF, MRI
Dorsal column Chronic Vitamin B12 deficiency32 Serum B12, Schilling’s test*
Copper deficiency33 Serum copper and caeruloplasmin level.
Tabes dorsalis34 Syphilis serology
HIV,18 HCV35 Serology
36
Cervical myelopathy Neuroimaging
Abs, antibodies; ACE, angiotensin-converting enzyme; ANA, antinuclear antibody; ASMA, antismooth muscle antibodies; CIDP, chronic inflammatory
demyelinating polyradiculoneuropathy; CISP, chronic immune sensory polyradiculopathy; CRMP-5, collapsin response mediator protein 5; CSF, cerebrospinal
fluid analysis; ENA, extractable nuclear antigen; GBS, Guillain–Barré syndrome; HCV, hepatitis C virus; HIV, human immunodeficiency virus; HPE,
histopathology; HTLV-1, human T cell lymphotrophic virus type 1; MAG, myelin-associated glycoprotein; NCS, nerve conduction studies; RF, rheumatoid
factor; *Schilling’s test, useful but obsolete in UK; SSA, Sjögren’s syndrome A; SSB, Sjögren’s syndrome B; SSEP, somatosensory evoked potentials;
tTG, tissue transglutaminase.

The ataxia may be cerebellar or sensory. Patients may analysis shows mutations in polymerase gamma
report problems with stance and gait secondary to pro- (POLG) or TWINKLE genes.40
prioceptive deficits. Clinicians should therefore request
serum antigliadin antibodies in patients presenting with
Sensory ataxia due to dorsal root ganglionopathies
sporadic idiopathic ataxia.16 38
Pathology of sensory neurones in dorsal root ganglia
leading to degeneration of peripheral axons and
Mitochondrial neuropathy central sensory projections in the dorsal columns
Sensory ataxic neuropathy, dysarthria and ophthalmo- cause a distinct entity called dorsal root ganglionopa-
paresis (SANDO) is a rare heterogeneous systemic thy.28 38 There is a unique pattern of non-length-
entity resulting from mitochondrial dysfunction, char- dependent sensory nerve degeneration leading to
acterised by the triad of sensory ataxic neuropathy, asymmetric, patchy neuropathic symptoms of pain,
dysarthria and ophthalmoparesis.40 As the clinical fea- burning, paraesthesia and sensory loss, with predilec-
tures include dysarthria, this leads to confusion with tion for upper limbs. Clinical findings include early
cerebellar pathology (case 2, box 3). Molecular sensory ataxia, areflexia, markedly impaired proprio-
ception, pseudoathetosis, but relatively preserved
muscle strength.28 38 Electrophysiologically, there is
Box 6 Inherited causes of sensory ataxia often marked involvement of the sensory fibres, with
reduced or absent SNAPs in a non-length-dependent
fashion.28 38 This is frequently associated with para-
Friedreich’s ataxia
neoplastic disorders, dysimmune conditions like
Ataxia with vitamin E deficiency
Sjögren’s syndrome or toxic exposure to drugs, such
Abetalipoproteinaemia
as chemotherapeutic agents and pyridoxine.
Spinocerebellar ataxia with neuropathy
Mitochondrial neuropathies: for example, SANDO
Paraneoplastic dorsal root ganglionopathy
SANDO, sensory ataxic neuropathy, dysarthria and
This is most commonly associated with small cell lung
ophthalmoparesis.
cancer,21 although sometimes with other malignancies

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including colonic carcinoma,26 Hodgkin’s lymphoma, HIV infection, but a few patients may develop a sub-
neuroendocrine tumours, breast and ovarian carcin- acute dorsal root ganglionopathy.18
oma.27 28 Patients usually present with subacute and Other infections causing sensory ataxia include
rapidly progressive sensory ataxia, pain and paraesthe- hepatitis C, measles, Epstein–Barr, varicella zoster,
sia. Most patients with paraneoplastic dorsal root and human T-cell lymphotropic virus type I
ganglionopathy have one of the antionconeural anti- infections.19 20 28
bodies; anti-Hu (antineuronal nuclear autoantibodies
type 1), antiamphyphysin, and anticollapsin response- Chronic idiopathic ataxic neuropathy
mediator protein-5 (CRMP-5).45 Anti-Hu—the most A few patients with chronic sensory ataxia have no
commonly associated antibody—has a sensitivity of identifiable cause; they are usually diagnosed as
82% and specificity of 99%, implying that their chronic idiopathic ataxic neuropathy.49 Typically, they
absence does not exclude an underlying malignancy.46 have normal strength with areflexia; cerebrospinal
The syndrome may develop before the cancer fluid analysis (CSF) electromyography and motor
becomes clinically overt (case 3, box 4) and can ante- nerve conduction are often normal, but sensory
date the cancer diagnosis by 0.5–62 months.28 potentials are absent. Presumably, it is an indolent,
slowly progressive, dorsal root ganglionopathy, and is
Immune mediated dorsal root ganglionopathy often refractory to treatment.49
Sjögren’s syndrome, an autoimmune disease, causes
dorsal root ganglionopathy through lymphocytic infil- Sensory ataxia due to sensory nerve root involvement
tration in the dorsal root ganglion.29 Patients may Chronic immune sensory polyradiculopathy results
present with dry eyes and dry mouth resulting from from preferential involvement of the large myelinated
mononuclear infiltration of the glands; lip biopsy can sensory nerve roots proximal to the dorsal root gan-
show destruction of small salivary glands and inflam- glion.31 It presents with ataxia and limb paraesthesia.
matory infiltrates.47 The ganglionopathy almost On examination, there is usually sensory ataxia, are-
always predates the diagnosis of Sjögren’s syndrome.38 flexia, impaired joint position and vibration sense but
The sensory symptoms of numbness, tingling, burning normal strength. Nerve conduction studies are
pain and dysaesthesia are often asymmetric at onset, normal, but somatosensory-evoked potentials are
and predominantly involve the upper limbs; with pro- abnormal, suggesting sensory root involvement.31 MR
gression, the symptoms and signs become symmetrical scan of the lumbar spine may show enlarged and
and generalised.28 38 A negative ANA/ENA (antinuc- enhancing nerve roots. CSF may show raised protein.
lear antibody/extractable nuclear antigen) (Anti-SSA Sensory rootlet biopsies may show demyelination
(anti Sjögren’s syndrome A) (Ro))/SSB (Sjögren’s syn- similar to CIDP. The condition may respond to
drome B) (La) antibodies) does not exclude the diag- immunomodulation.31
nosis.47 Lip biopsy established the diagnosis after
prolonged delays in case 4 (box 5). Although ENA Sensory ataxia due to posterior spinal column involvement
was negative, a positive lip biopsy gave the diagno- Vitamin B12 deficiency
sis.47 The initial explanation of sensory ataxia had Classical subacute combined degeneration from B12
been pyridoxine toxicity, and it was only after long- deficiency is uncommon nowadays. It more likely
term follow up and continued disability that we sus- occurs with nitrous oxide exposure—anaesthesia and
pected Sjögren’s syndrome. dental staff or recreational abusers.32 However, gastric
Medication-induced dorsal root ganglionopathy or ileal resections with bacterial overgrowth in blind
Pyridoxine overuse can rarely cause sensory dorsal loops, anastomoses or diverticula may continue to
root ganglionopathy.22 Patients usually present with a underlie this classical neurological syndrome. Patients
pure sensory neuropathy with features of large fibre present with limb paraesthesia, subacute gait disorder
involvement. Doses as little as 200 mgs per day may with sensory ataxia and/or spasticity. Examination
be the cause.48 often shows signs of dorsal column impairment,
Anticancer drugs, particularly platinum compounds including loss of proprioception and vibration as well
like cisplatin, can also cause sensory ataxic neur- as the combination of sensory ataxia and spastic para-
opathy; the severity of neuropathy usually correlates paresis. Length-dependent peripheral neuropathy
with the cumulative drug dose.23 With platinum- often coexists, and may manifest as depressed lower
containing compounds, the sensory deficits may pro- limb reflexes with stocking sensory loss.32 MR scan of
gress for several months after stopping treatment, a spine may show increased T2 signal, most commonly
phenomenon called ‘coasting’.23 in the dorsal cervical and thoracic cord. Note that
some patients with subacute combined degeneration
Infection-associated dorsal root ganglionopathy of the cord may have normal or borderline B12 level,
HIV is the most common infection associated with and helpful tests to confirm the diagnosis include
sensory ataxia, and typically presents acutely or suba- increased serum levels of homocysteine and methyl-
cutely. Peripheral neuropathy commonly complicates malonic acid.32

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Copper deficiency although half show pathological evidence at autopsy.
Copper deficiency is an under-recognised cause of The condition manifests with a slowly progressive
neurological and haematological abnormalities.33 This painless spastic paraparesis, sensory ataxia and sphinc-
essential trace element is absorbed in the stomach and ter dysfunction.18 MR scan of the spine is usually
proximal duodenum. Risk factors for copper deficiency normal although there may be non-specific tract
include previous upper gastrointestinal surgery (eg, gas- hyperintensities. HIV-associated vacuolar myelopathy
trectomy, bariatric surgery, small bowel resections) and often occurs in the late stages of HIV infection, and
malabsorption. Zinc overload—from zinc supplementa- commonly parallels the development of AIDS demen-
tion or ingestion of denture cream—competes with tia complex.18
copper for absorption, causing copper deficiency. The Tabes dorsalis results from degeneration of the
clinical picture is often clinically and radiologically dorsal columns of the spinal cord and sensory nerve
indistinguishable from vitamin B12 deficiency.33 There roots in late-stage or tertiary neurosyphilis.34 38 The
may be anaemia (microcytic, macrocytic, or normocy- presentation is classically with lancinating or
tic), leucopenia and, rarely, thrombocytopenia. Plasma lightning-like pains, progressive sensory ataxia, pro-
copper, caeruloplasmin levels and urinary copper levels prioceptive loss and positive Romberg’s sign.
are all reduced. Although copper deficiency is rare, the Hepatitis C infection may cause transverse myelop-
neurological syndrome is incapacitating and frequently athy; sensory ataxia may be its only manifestation.35
irreversible, especially if treatment is delayed. Early
Compressive and demyelinating disorders
diagnosis and treatment with copper supplementation is
therefore essential.33 Cervical myelopathy commonly presents with painful
stiff neck, upper limb paraesthesia and gait and
Infections balance disturbance.36
HIV infection can cause vacuolar myelopathy.18 This Other compressive pathologies (eg, meningioma)
causes symptoms in about 10% of patients with AIDS, can also cause sensory ataxia if there is predominant

Figure 2 Integrated diagnostic algorithm to evaluate sensory ataxia. abs, antibodies; ACD, alcoholic cerebellar degeneration;
ANA, antinuclear antibody; CA, cerebellar ataxia; CA +, cerebellar ataxia plus; CANOMAD, chronic ataxic neuropathy,
ophthalmoplegia, monoclonal IgM protein, cold agglutinins and disialosyl antibodies; CIDP, chronic inflammatory demyelinating
polyradiculoneuropathy; CISP, chronic immune sensory polyradiculopathy; CSF, cerebrospinal fluid; DRG, dorsal root ganglionopathy;
ENA, extractable nuclear antigen; GBS, Guillain–Barré syndrome; HCV, hepatitis C virus; HTLV1, human T cell lymphotrophic virus
type 1; JPS, joint position sense; MAG, myelin-associated glycoprotein; MFS, Miller Fisher syndrome; NCS, nerve conduction studies;
SA, sensory ataxia; SA +, sensory ataxia-plus; SANDO, sensory ataxic neuropathy, dysarthria and ophthalmoparesis;
SCA, spinocerebellar ataxia; SSEP, somatosensory evoked potentials; tTG, tissue transglutaminase.

Chhetri SK, et al. Pract Neurol 2014;14:242–251. doi:10.1136/practneurol-2013-000764 249

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involvement of the posterior columns. In addition to of sensory ataxia. This is clearly crucial for appropriate
spastic weakness in limbs, patients may have impaired management of these potentially disabling conditions.
joint position and vibration sense as well as a positive Correction notice This paper has been amended since it was
Romberg sign.36 published Online First. In the earlier version there were some
minor errors regarding the box numbers. Page 2, box citations
Multiple sclerosis can cause sensory ataxia if demye- have been corrected to “see case 2, box 3” rather than “case 2,
lination involves the central sensory pathways. box 2”. Page 4, it now reads “case 1, box 2” instead of “case 1,
box 3”. Page 6, it now reads “see case 2, box 3”. Finally, on
page 5, top right hand column, the word ‘only’ has been
USING A DIAGNOSTIC ALGORITHM TO DETECT deleted because half of patients with AIDS (not only of the
AND DIAGNOSE SENSORY ATAXIAS 10%) show pathological evidence. The sentence now reads:
The illustrative cases (boxes 2–5) show the complex- “This causes symptoms in about 10% of patients with AIDS,
although half show pathological evidence at autopsy”.
ities and difficulties in characterisation of the sensory
ataxia syndrome. However, the initial error of mis- Acknowledgements We acknowledge Dr Graham Niepel’s help
labelling sensory ataxia (usually as cerebellar ataxia) is in the management, including diagnosis of Case 4. We thank
the Neurophysiology departments at North Manchester
the most important. Adherence to a strict definition of General Hospital (Dr Bangash); Salford Royal Hospital
sensory ataxia based on careful history and examin- (Drs Marshall, MacDonagh, Ramdass); Royal Preston Hospital
ation should prevent mistakes. Further pitfalls in dis- (Dr Lekwuwa) for their help in the work-up of these patients.
We also thank Matt Briggs, senior medical graphic artist,
cerning the correct cause of sensory ataxia could be Department of Medical Illustration, Royal Preston Hospital for
avoided by adopting a logical anatomical approach help with figure 1.
(figure 1). Confident detection of sensory ataxia is Contributors SKC: study design, acquisition of data, drafting/
more difficult when there is an underlying multisystem revising the manuscript for content, coordination and execution
disorder ( paraneoplasia, chronic infections, eg, HIV or of the study. DG: acquisition of data and revising the
manuscript for intellectual content. SS: acquisition of data and
hepatitis C, mitochondrial disorders, etc.; figure 2, revising the manuscript for intellectual content. AV: study
table 2). It takes a very careful examination to detect concept and design, acquisition of data, revising the manuscript
sensory ataxia in association with cerebellar ataxia; for intellectual content, execution and supervision of the study.
clinicians should also try to identify the dominant syn- Competing interests None.
drome (sensory ataxia-plus vs cerebellar ataxia-plus). Patient consent Obtained.
Figure 2 shows an algorithm to facilitate characterisa- Provenance and peer review Commissioned; externally peer
tion of sensory ataxia, including identification of the reviewed. This paper was reviewed by John Winer.
site of lesion and its possible cause. A survey of the lit-
erature failed to identify studies that formally address a
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