Table 1. Clinical data and lesion distribution on neuroimaging in the seven patients with dentate nucleus pathologies.
| Case | Diagnosis | Presentation | Dentate/ cerebellum | Medulla | Pons/MCP | Midbrain | Thalamus | BG | WM | DR | SWI | Other areas a | MRI resolution |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | Isoniazid toxicity | Encephalopathy and ataxia | Y | – | – | – | – | – | – | – | – | Midbrain and thalamus 8 | 4 wk |
| 2 | Ornidazole toxicity | Encephalopathy, vertigo, and ataxia | Y | – | – | – | – | – | – | – | – | Brainstem, Cc, BG, and supratentorial WM 9 | 4 wk |
| 3 | ADEM | Altered mental status and ataxia | Y, cerebellum | – | Pons/MCP | – | – | – | Corona radiata, CSO | – | – | Cerebellum, brainstem, BG, thalamus, cortex, supratentorial WM, and spinal cord 10 | 12 wk |
| 4 | CTX | Childhood diarrhea, juvenile cataracts, progressive ataxia, and impaired cognition | Y, cerebellum | Y (inferior olive) | – | – | – | – | Posterior limb of IC, parieto-occipital deep WM | – | – | Inferior olive, substantia nigra, globus pallidus, supratentorial WM, corticospinal tracts, and Cc 6 | No resolution |
| 5 | NF1 | Café-au-lait spots and neurofibromas. Screening MRI was done. | Y | – | MCP | – | – | – | – | – | – | BG, IC, brainstem, MCP, and cerebellum 11 | Data not available |
| 6 | Primary hypo-parathyroidism | Seizures | Y, cerebellum | – | – | – | Y | Y | Y | Not done | Brainstem, BG, thalamus, and IC 12 | No resolution | |
| 7 | CLIPPERS | Headache, vomiting, dysarthria, generalized spasticity, and ataxia | Y, cerebellum | Y | Pons/MCP | Y | – | – | Posterior limb of IC | – | Y | Brainstem, MCP, cerebellum, and cervical spinal cord 13 | 4 wk |
Abbreviations: ADEM, acute disseminated encephalomyelitis; BG, basal ganglia; Cc, corpus callosum; CTX, cerebrotendinous xanthomatosis; CLIPPERS, chronic lymphocytic inflammation with pontine perivascular enhancement sensitive to steroids; CSO, centrum semiovale; DR, diffusion restriction; IC, internal capsule; MCP, middle cerebellar peduncle; MRI, magnetic resonance imaging; NF1, neurofibromatosis type 1; SWI, susceptibility weighted imaging; WM, white matter; Y, involved; –, Not involved
a
Other areas: other structures likely involved in each disease.