Syringomyelia and Arachnoiditis

106 Journal of Neurology, Neurosurgery, and Psychiatry 1990;53:106-113 Syringomyelia and arachnoiditis L R Caplan, A B Norohna, L L Amico Abstract and weakness in his right hand. Months later, Five patients with chronic arachnoiditis he developed sensory loss and weakness of the and syringomyelia were studied. Three hands, more noticeable in the left than the patients had early life meningitis and right. Atrophy, frequent burns, and hand developed symptoms of syringomyelia tremor were reported by the patient. Four eight, 21, and 23 years after the acute years later, the lower extremities became weak, infection. One patient had a spinal dural initially on the right side. He then experienced thoracic AVM and developed a thoracic weakness in the left leg which soon became syrinx 11 years after spinal subarachnoid more severe than in the right. He complained of haemorrhage and five years after surgery a drawing, burning pain in both arms from the on the AVM. A fifth patient had tuber- hands to the radial forearms and from the hips culous meningitis with transient spinal to the toes. cord dysfunction followed by develop- In 1943 (aged 46), he was first evaluated at ment ofa lumbar syrinx seven years later. the Harvard Neurological Unit at Boston City Arachnoiditis can cause syrinx formation Hospital. There was diminished body hair and by obliterating the spinal vasculature burn scars on his fingers. Mental function and causing ischaemia. Small cystic regions cranial nerves were normal except for a slight of myelomalacia coalesce to form left lower facial droop and slighlt leftward cavities. In other patients, central cord protrusion of the tongue. Upper extremity ischaemia mimics syringomyelia but no muscles, especially the deltoids and intrinsic cavitation is present. Scar formation with hand muscles, were atrophied and occasional spinal block leads to altered dynamics of fasciculations were noted. There was bilateral cerebrospinal fluid (CSF) flow and con- lower extremity weakness with a left foot drop tributes to the formation of spinal cord and atrophy of the left tibialis anticus. An cystic cavities inconstant intermittent "piano playing" tremor was seen in the hands. Upper extremity reflexes were absent except for a weak right Symptomatic or secondary syringomyelia triceps jerk but lower extremity reflexes were refers to spinal cord cysts, often long and exaggerated with right ankle clonus and exten- tubular, that are caused by other recognised sor plantar responses. Pin and thermal sensa- disease processes. Intramedullary spinal cord tion were lost in the hands, radial forearms and tumours are sometimes associated with a syrinx entire left leg. Touch was also decreased in usually because ofsecretion offluids by tumour these parts but to a lesser degree. Vibration cells or necrotic cavitation of the neoplasm. sense was lost at both wrists, the right elbow, Scarring of the spinal meninges (usually term- and the left leg below the knee. Position sense ed "arachnoiditis" though both the pia mater was diminished below T3. Gait was unsteady and arachnoid are involved), has also been and wide-based and was characterised by over- reported as a cause of secondary syringomyelia. steppage due to a left foot drop. Lumbar The arachnoiditis may be caused by trauma,''12 puncture revealed normal fluid and dynamics. pyogenic infection,'3 14 tuberculous menin- He was placed in a chronic care unit, at the gitis,'"20 luetic meningitis,2"52' bleeding into Long Island Hospital, where he was examined the meninges'822 and post operative scarring.'3 by successive generations of Harvard Medical Department of Neurology, Michael Though arachnoiditis is known to be associated School students, residents and attending Reese Hospital with syrinx formation, the mechanism ofsyrin- physicians and considered to be an example of L R Caplan gomyelia is not well understood. We report five "classic syringomyelia". He became progres- L L Amico patients with non-traumatic arachnoiditis who sively quadriplegic and bedridden and was Department of later developed syringomyelia. Neurology, the readmitted to the Neurological Unit in 1966. University of Chicago At that time his mentation and cranial nerve Hospitals and Clinics functions were normal. There was diffuse mus- A B Norohna Case reports cle weakness; he could not lift his head from the Correspondence to: Case 1. This man was observed extensively extend his outstretched hands, sit up Dr Louis R Caplan, pillow, Department ofNeurology, during many years and studied at necropsy. In from a supine position, maintain wrist flexion New England Medical 1914 (aged 17), whilst in the army, he suffered or or move his hands. The right Center, 750 Washington extension, Street, Boston, MA, 02111, an acute infectious illness that was diagnosed lower extremity was completely immobile, but United States. by lumbar puncture as meningitis. He made a he could lift his left leg from the bed. The lower Received 28 February 1989 good recovery except for temporary strabismus and in revised form extremities were very spastic and sensory 23 August 1989. and continued to serve in the army until 1919. stimuli evoked flexor spasms in the left foot. Accepted 6 September 1989 In 1935 (aged 38), he noted loss of sensation. There were no upper extremity reflexes; knee Syringomyelia and arachnoiditis 107 and ankle reflexes were exaggerated and both was removed but there were no changes in the ankles showed sustained clonus. Both plantar neurological symptoms and signs in the post responses were extensor. Position and vibra- operative period. tion sense were impaired below C5. There was In 1981 (aged 47), she noticed increased complete sensory loss to all modalities below weakness of the left leg. Urinary retention TI on the left and a relative loss to pin prick developed and was treated with an indwelling below T6 on the right. All sensory modalities catheter. Her left hand became weak and the were lost in the C5, C6 and C7 dermatomes on inside of the hand was numb. The left half of the right. CSF protein was 260 mg per cent, the face also became numb and she experienced later 185 mg per cent. CSF dynamics were occasional vertigo. Examination revealed abnormal and myelography confirmed an ir- rotatory nystagmus greater on left gaze. The regular high cervical block. He died of pupils were small but reacted to light. Pin and pneumonia after a brief stay in another ex- thermal sensations were diminished on the left tended care unit. face while touch perception was normal. The At necropsy, all layers of the meninges were left corneal reflex was diminished. There was severely scarred and adherent to each other. wasting and weakness of the left intrinsic hand The thickened dura mater was densely ad- muscles and some weakness of the right wrist herent to the underlying bony skeleton, making extensors and intrinsic hand muscles. The it difficult to extract the spinal cord intact. The lower extremities were quite weak and spastic. meninges of the posterior fossa were also Deep tendon reflexes were exaggerated except scarred. There was no cavitation or slit within for the left biceps and brachioradial jerks which the medulla. The spinal cord was thin, atro- were absent. There was bilateral ankle clonus phic, and flattened, especially in the cervical and plantar responses were extensor. Vibra- and thoracic regions, but extending through tion, position, pain and temperature sensation lubar segments. Microscopic sections con- were diminished below T7 bilaterally. There firmed severe fibrosis and thickening of the was decreased pin and thermal sensation in the meninges. Both arteries and veins showed left arm, face and neck. Cranial CT was nor- fibrous degeneration of vessel walls. At times, mal. Myelography using metrizamide that we in the areas of necrosis, there was extensive introduced through a lateral Cl-C2 puncture connective tissue proliferation with accumula- revealed a nearly total block at T5 with some tion of dense eosinophilic staining collagen dye descending to T6-7. The cord was widened surrounding thickened vessels. There were few at T6 and in the cervical region. CT of the remaining inflammatory cells and no granu- dorsal spine during metrizamide myelography lomas. showed a cystic swelling within the cord. The cervical cord was reduced to a thin Somatosensory evoked responses could not be ribbon with extensive central necrosis and obtained with stimulation of the left peroneal, cavity formation. The cavity was collapsed, saphenous, or median nerves. Denervation was smooth and often bilobed, but there were no present in the left C8 and TI root innervated lining cells nor a well formed wall. There were muscles. Blink response recording was abnor- few remaining anterior horn cells in the cervical mal in the left supraorbital region. The electro- or upper thoracic cord. The cervical roots were physiological abnormalities were consistent thin. The dorsal and ventral roots also showed with a lesion affecting the left cervical spinal moderate to marked loss of myelin. There was cord and lower medulla. Spinal angiography some Schwann cell proliferation. was normal. The thoracic cord was also flattened. The At surgical exploration, there were dense central zones stained poorly for myelin and had dural and arachnoid adhesions at T6, but no occasional small cavities. Caps of peripheral residual AVM. The spinal cord was dilated myelin remained. The corticospinal tracts were above the cicatrix. Incision of the dilated cord decimated throughout the thoracic and lumbar led to fluid drainage from a syrinx and a cord. The spinocerebellar tracts and posterior catheter was placed within the cavity. Micros- columns were scarred especially at the C7 and copic examination of material removed at the TI region as part of the general destruction. T6 level revealed only dense fibrocollagenous The lumbar roots were also thin and enmeshed tissue. Postoperatively, her legs were slightly within adhesive arachnoidal scarring which weaker. extended to the filum terminale and cauda Two years later, she noticed increasing equina.

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