Figure 1 - uploaded by Veeramani Preethish-Kumar
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(A-F). Wasting of bilateral medial forearm (left > right) with relative sparing of brachioradialis (oblique atrophy) (thick black arrows). Severe wasting of small muscles of hands (thick white arrows). Note: First dorsal interossei and hypothenar muscles are more wasted than thenar muscles.
Source publication
Context: Ventral longitudinal intraspinal fluid collection (VLISFC) presenting as hand amyotrophy has been described only in a few cases and there are no reports on associated intracranial CSF hypovolemia (ICH). We describe the clinical and imaging findings of a case with combined brachial amyotrophy and ICH secondary to VLISFC.
Findings: A 31 year...
Citations
... Nonsurgical treatments such as botulinum toxin have been used to treat patients with FTSD, although their therapeutic effects are limited [15,18,20]. This is problematic, as complete recovery is critical in ensuring that professionals can maintain their occupations. ...
Ventro-oral (Vo) thalamotomy is effective in patients with focal task-specific dystonias (FTSDs), but only in those with upper-limb symptoms. We describe a patient with drummer’s dystonia who completely recovered after Vo thalamotomy. A 37-year-old man who started playing drums at 14 began having difficulty performing fine movements with his right foot when drumming at 22. He experienced right hand cramps while drumming 3 months before visiting our hospital. He was diagnosed with FTSD. Left Vo thalamotomy was performed, which led to complete improvement of symptoms. Vo thalamotomy may be effective for FTSD patients with upper- and lower-extremity symptoms.
BACKGROUND AND OBJECTIVES
Cerebrospinal fluid (CSF) collections extending longitudinally at the anterior aspect of the spinal dura have been reported in association with various conditions and under multiple names. The aim of this study was to report cases associated with brachial amyotrophy (BA) and examine its relationship with other clinical variants.
METHODS
We conducted a retrospective cohort study including patients who presented with a motor deficit of the upper limbs and an anterior interdural CSF collection on spinal MRI. We performed a systematic review of the literature to include cases revealed by BA.
RESULTS
Seven patients presenting with BA and a confirmed dural dissection on spinal MRI were included. All patients were male with a slowly progressing history of asymmetrical and proximal motor deficit of the upper limbs. Chronic denervation affecting mostly C5 and C6 roots was found on electroneuromyography. Spinal MRI demonstrated an anterior CSF collection dissecting the interdural space and exerting a traction on cervical motor roots. Dynamic computed tomography myelogram localized the dural defect every time it was performed (4/7 cases), and surgical closure was possible for 3 patients, leading to resolution of the collection. Literature review yielded 18 other published cases of spinal dural dissections revealed by BA, including 4 in association with spontaneous intracranial hypotension and 4 others in association with superficial siderosis.
CONCLUSION
We propose a unifying diagnosis termed “spinal anterior dural dissection” (SADD) to encompass spinal dural CSF collections revealed by BA (SADD-BA), spontaneous intracranial hypotension (SADD-SIH), or superficial siderosis (SADD-SS).
Intraspinal cerebrospinal fluid (CSF) collection has been reported as a rare cause of lower motor neuron (LMN) disorder. We report a case of bibrachial diplegia associated with intraspinal CSF collection and perform a systematic literature review.
A 52-year-old man developed a bibrachial amyotrophy over 6 years, confirmed by the presence of cervical subacute neurogenic changes at electromyography (EMG). Brain magnetic resonance imaging (MRI) revealed cerebral siderosis, while spine MRI showed a ventral longitudinal intraspinal fluid collection (VLISFC) from C2 to L2. No CSF leakage was localized at myelography; a conservative treatment was chosen. We searched for all published cases until 30th April 2023 and extrapolated data of 44 patients reported in 27 publications.
We observed a male predominance, a younger disease onset compared to amyotrophic lateral sclerosis, and a quite long disease duration, highlighting a slow disease progression. LMN signs were more frequently bilateral, mostly involving C5-C6 myotomes. Around 61% of patients presented additional symptoms, but only three referred to a history of headache. Accordingly, CSF opening pressure was mostly normal. Spinal MRI revealed the presence of VLISFC and in some cases myelomalacia. EMG patterns displayed both chronic and subacute neurogenic change in the cervical region. The disease course mainly depended on the treatment choice, which was mostly represented by a surgical approach when a specific dural defect was detected by imaging.
Bibrachial diplegia due to VLISFC can be a treatable cause of focal amyotrophy and presents some clinical and radiological “red flags” which cannot be missed by a clinical neurologist.
Purpose of review:
Spontaneous intracranial hypotension is a disorder caused by spinal CSF leakage. This article reviews the clinical presentation, diagnosis, and treatment of spontaneous intracranial hypotension.
Recent findings:
The hallmark symptom of spontaneous intracranial hypotension is acute orthostatic headache; however, clinical presentations can be heterogeneous. New evidence shows that lumbar puncture is not always necessary or sufficient to establish the diagnosis. Some patients may have normal opening pressure, which suggests that insufficiency of CSF volume (hypovolemia) rather than CSF pressure might be the underlying mechanism. Several neuroimaging modalities can aid in diagnosis and localization of the CSF leakage, including brain MRI, spinal MRI, CT myelography, digital subtraction myelography, and radionuclide cisternography. Complications, such as subdural hematoma, can lead to a change in the headache pattern and potentially life-threatening consequences. Conservative treatments, such as fluid supplementation, can provide temporary relief; however, epidural blood patches, especially targeted ones, are more effective and definitive. For patients with refractory spontaneous intracranial hypotension, surgical repair of spinal CSF leakages should be considered.
Summary:
Brain and spinal MRIs are important for the diagnosis and treatment of patients with spontaneous intracranial hypotension. Early treatment with epidural blood patches may be considered to shorten the disease duration and minimize the potential risk of complications.
