A ‘useless’ hand

A 30-year-old woman reported a 3-month history of an ‘odd and heavy feeling’ and loss of dexterity in her right hand, with onset over days. The hand prevented her from typing at work and seemed to ‘do its own thing’. Family members noted that she held the arm as though she had had a stroke. Her walking felt imbalanced and she had mild urinary urgency. She had been previously well.

On examination, she cradled the right arm in her left, but when distracted the hand writhed around in her lap. Cranial nerve examination was normal, including funduscopy. On arm extension, there was pseudoathetosis of the right hand and all fingers. These movements were more profound on distraction and on closing her eyes. Tone was normal, and strength was essentially normal in the right arm with eyes open, including all small hand muscles, but appeared mildly weak with eyes closed. Reflexes were pathologically brisk and asymmetric (greater on the right). Hoffman’s sign was absent; there were no pectoral jerks.

Pain appreciation with pinprick testing was relatively intact and temperature appreciation was normal. There was reduced fine-touch appreciation and reduced sharp-blunt discrimination on the right arm from fingertip to shoulder, and to a lesser extent on the left. We did not test two-point discrimination. Vibration sense was lost to the shoulder in the right arm but not the left. Joint-position sense was absent to the wrist on the right, but normal on the left. Lower limb examination was normal apart from brisk reflexes and mild instability with tandem gait. Plantars were flexor and Romberg’s test was negative. Figure 1 shows demyelinating, hyperintense and asymmetric lesion at the C2 level in the cervical cord with evidence of further demyelination elsewhere.

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Figure 1

(A) T2 sequence showing several lesions but prominent dorsal column high cervical lesion (arrowed). (B) On the right, STIR sequence highlighting the same lesion (again, arrowed). (C) MRI cervical spine cross-sectional image showing asymmetric, demyelinating lesion.

The useless hand of Oppenheim

Hermann Oppenheim (1858–1919) (figure 2) was born in Westphalia and gave his name to the clinical sign in which the great toe dorsiflexes with downward stimulus of the medial tibia, often useful if Babinski’s sign is equivocal. He wrote a prominent neurology textbook, translated into several languages and lasting over seven editions.1

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Figure 2

Hermann Oppenheim.

The ‘Useless Hand of Oppenheim’ was first described as a manifestation of multiple sclerosis in 1911, in his lecture to the 79th Annual Meeting of the British Medical Association, on clinical presentations of multiple sclerosis:

‘a sudden numbness and awkwardness of one arm in which the sense of posture is most seriously affected… A most remarkable fact is that in this cervical form of multiple sclerosis the posterior columns symptoms are the most prominent features. I may assert from my own experience that the acute ataxia brachialis is nearly always the result of multiple sclerosis, always dominated by the sensory ataxia in the arms. The progress is also characteristic. All of our observations show that after weeks and months, it comes to a considerable remission’.2

He recognised that this profound loss of proprioceptive function with relatively preserved strength was due to a high cervical cord lesion predominantly affecting the posterior columns.2 3 This may produce a bilateral syndrome (sometimes significantly asymmetrical) in which both upper limbs show loss of position and vibration sense that dominate the sensory findings.3

Clinical features and differential diagnosis

As with all classical neurology, a deafferented limb presents a neurologist with an interesting exercise in postulating lesion location. The dominant features here of proprioceptive and vibration sensation loss allow us a brief review of the mechanisms of sensory ataxias of limbs (ie, with preserved strength).

Sensory abnormalities are best considered systematically and anatomically. With preserved pain and temperature appreciation, the problem does not involve small peripheral nerve fibres. Similarly, an anterior cord syndrome is excluded with preservation of spinothalamic modalities.

Can the presentation be attributed to a large-fibre sensory neuropathy impairing joint-position and vibration sense? Primary afferent fibres innervating muscle spindles are the principal receptors for both limb position sense and kinesthesia.4 The receptors responsible for vibration sense include Merkel disk receptors and Pacinian corpuscles. These afferent fibres course through peripheral nerves into the medial aspect dorsal roots and then enter the dorsal horn of the spinal cord.

However, peripheral neuropathies affecting large sensory nerves are typically length-dependent. The lower limbs are frequently affected first as they have the longest nerves, giving Rombergism and unsteady gait (worse in low light due to loss of visual feedback). A good example of this is with chronic inflammatory demyelinating polyradiculopathy.

A sensory neuronopathy (or dorsal root ganglionopathy) can present relatively quickly and be patchy and asymmetrical, and so can predominantly involve the upper limbs. Although large nerve fibres are most affected, producing sensory ataxia and sometimes pseudoathetoid movements, all sensory nerves can be involved, including autonomic, cranial and small fibre nerves with varied clinical presentations. However, with a sensory neuropathy or dorsal root ganglionopathy, the relevant deep tendon reflexes should be lost.5 Sensory neuronopathies (ganglionopathies) are usually immune-mediated, typically Sjögren’s syndrome or paraneoplastic (especially anti-Hu), but rarely from other infective and toxic causes.6

Many of the afferents subserving well-localised touch, pressure, vibration and joint-position sense enter the dorsal columns of the spinal cord through the dorsal horn. These sensory axons ascend the cord to the dorsal aspect of the caudal medulla, synapsing in the dorsal column nuclei (nucleus gracilis and cuneatus). The afferent fibres from the lower half of the body (fasciculus gracilis) are medial to those from the upper body (fasciculus cuneatus). The sensory fibres in the dorsal columns have not synapsed and are therefore still the primary ‘first order’ fibres.

Pathology in the dorsal columns (and dorsolateral ascending tracts) can therefore produce proprioceptive problems and reduced vibration sense. Classically, micronutrient deficiencies such as vitamin B₁₂ (subacute combined degeneration of the spinal cord) present with lost joint-position/vibration sense and a progressing myelopathy, as can copper deficiency. Examination findings usually include a spastic paraparesis, absent ankle jerks and extensor plantars.7 Infective causes such as HIV and neurosyphilis (tabes dorsalis) can selectively damage posterior columns. All these pathologies typically give symmetrical deficits.

Following the neuroanatomy, second-order neurones from the nuclei gracilis and cuneatus give rise to axons within the medulla (internal arcuate fibres). These axons decussate and reach the medial lemniscus, a tract comprising axons connecting the dorsal column nuclei to the ventral posterolateral nucleus of the thalamus. The third-order neurones in the ventral posterolateral nucleus relay to the primary somatosensory cortex. The parietal lobe, immediately behind the postcentral gyrus, synthesises cortical information into a recognisable pattern. Damage to this ‘association cortex’ produces an inability to interpret sensory signals (somatosensory agnosia).

A cortical cause of sensory ataxia in the arm usually presents unilaterally; lesions of the postcentral gyrus and sensory radiations in the posterior limb of the internal capsule can cause deafferented distal upper limbs. This ‘cortical hand’ rarely has complete loss of sensation but can have a severe proprioceptive loss giving sensory ataxia and pseudoathetosis; however, vibration sense is only rarely affected, confirming that vibration and joint-position sense are dissociated.8 Astereognosis (and sensory inattention) in the presence of mild sensory disturbance is a clue; this can occur in both cortical/subcortical lesions and is less likely in dorsal column lesions.

Abrupt onset of semipurposeful movement of a limb with feelings of estrangement can occur in a stroke of the corpus callosum or parietal lobe (alien hand syndrome), but these are much rarer and have no loss of joint-position sense9 (table 1). With no higher cortical features such as neglect, cognitive dysfunction or alien limb, a cortical cause of this woman’s arm dysfunction (such as a parietal lesion or corticobasal syndrome) is unlikely. If there were other features such as neglect, ideomotor apraxia or Parkinsonism, we would have had to consider these differentials.

Clinical clues to the useless hand of Oppenheim

The sensory symptoms are relatively vague: a feeling of an odd sensory disturbance or numbness—not the frank, intrusive paraesthesia that accompanies some cervical cord demyelinating lesions—sometimes with feelings of tightness. Such symptoms do not immediately point to a significant sensory deficit. An important clue is the pseudoathetosis: slow, continuous writhing movements of the fingers or hands, much worse on eye closure, almost like ‘fingers searching in the darkness’. Examination identifies reduced joint-position and vibration appreciation—often missed by non-neurologists—which in the context of brisk reflexes (with mostly preserved spinothalamic function) points to a posterior spinal cord lesion. The asymmetry and the time course of evolution also point to an inflammatory demyelinating cause.

Strength examination can change by asking the patient to look at the affected limb during testing so the limb can appear weak when it is not. Therefore, finding better limb strength with the patient looking at, compared with looking away from, the limb can be instructive. The pseudoathetosis should also improve by looking at the limb.

By contrast, the opposite can occur in some functional disorders manifesting as odd hand movements and weakness. Thus, distracting the patient’s attention from the limb being examined can improve function, while drawing attention to it during formal examination often makes it worse.

In functional disorders the sensory disturbance is usually less clear-cut, being often either patchy or reportedly complete across all sensory modalities, does not follow any anatomical distribution, and often shows an abrupt border between abnormal and normal. Despite apparently abnormal joint-position sense, the patient may still be able to perform skilled movements and fine acts without difficulty, when distracted. This can also manifest in other functional disorders such as gait problems, which improve when using a smartphone.10 The weakness has a ‘give-way’ quality even when the patient is asked to concentrate on the affected limb.

This clinical presentation highlights the need for a sensory examination to include joint-position and vibration sense, as well as temperature and pain appreciation. Tasks such as writing or doing up a button can test loss of functional ability and dexterity (box). 

We have moved away from using eponyms in clinical medicine—recognising that many remain very valuable such as Guillain-Barré syndrome or Parkinson’s disease. However, we believe that the term ‘useless hand of Oppenheim’ is useful as the alternative—‘de-afferented hand secondary to posterior column demyelination’—is cumbersome and not nearly so memorable. The term ‘useless hand of Oppenheim’ conveys useful information about the clinical presentation and the site and type of lesion and helps in teaching and conveying historical background in clinical neurology. The term does, however, need to be explained when discussed with the patient, who can be reassured by the implied likely good prognosis.

Outcome

This patient received 5 days of oral methylprednisolone 500 mg per day. We diagnosed a clinically isolated syndrome and her scan suggested a high risk of progress to multiple sclerosis. Three months later she had improved, although left with mild impairment of right fluent finger movements. We have discussed with her disease-modifying therapies and the possibility of relapse.